Human Function Module (Fix)

PBL Group 8A Medical Faculty of Airlangga University
Human Function Module 2014

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HUMAN FUNCTION MODULE
PROBLEM BASED LEARNING

SCENARIO A

Composed by :
Group 8A

Medicine Faculty of Airlangga University
2
nd
Semester
2014
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SCENARIO A

TUTOR
Muhammad Saiful Ardhi, dr., Sp.S

SCENARIO COMPOSER
Irfiansyah Irwadi, dr., M.Si.

INFORMANTS
Raden Argarini, dr., M.Kes
Muhammad Saiful Ardhi, dr., Sp.S
Dr. Anggraini Dwi Sensusiati, dr., Sp.Rad(K)
Andriati, dr., Sp. KFR

EDITOR
Prof. Dr. Nancy Margarita Rehatta, dr. SpAnKIC-KNA
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COMPOSED BY
GROUP 8A

LEADER
M. Syaifullah 011311133024
VICE LEADER
Anastasha Puspagita 011311133066

SECRETARY
Husnur Rofiqoh 011311133045
Savira Amanda 011311133010

MEMBERS
Berli Arfani Rantam 011311133003
Rr. Fara Luthfita 011311133017
Listiana Rizka 011311133031
Khrisna A. Ismanda 011311133038
Faradillah Mutiani 011311133052
Meilia Dwi Cahyani 011311133059
M. Wahyu Aghdhi Pradipta 011311133073
Ayu Wandira 011311133080

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Main Competency
After undergoing this module, all second semesters students of Medical
Education Study Program, Medicine Faculty of Airlangga University can
explain the human anatomy physiology, pathophysiology and its
associated clinical problems, the necessary investigation and its
management principles every faced with clinical problem in a simulation
case.

Component Competency
1. Able to explain Eksitabel Cell and Action Potential
2. Able to explain Physiology of Nervous System
3. Able to explain Physiology of Musculoskeletal System
4. Able to explain Pathophysiology and Management of the related case
5. Able to explain Radiology principal to support the diagnosis of the
related case
6. Able to explain the role of rehabilitation to the related case

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CONTENTS
COVER .......................................................................................................... 1
CONTENTS ................................................................................................... 6
TABLE LIST ................................................................................................. 7
PICTURE LIST .............................................................................................. 8
CHAPTER ONE : FIRST TUTORIAL ......................................................... 9
1.1. SCENARIO ........................................................................................ 9
1.2. MAIN PROBLEM ............................................................................. 9
1.3. KEYWORDS ..................................................................................... 9
1.4. EARLY HYPOTHESIS ..................................................................... 9
1.5. COGNITIVE STRATEGY ................................................................ 9
1.6. EARLY CONCEPT MAPPING ........................................................ 10
1.7. ANALYSIS OF CONCEPT MAPPING ............................................ 10
1.8. LEARNING ISSUE ........................................................................... 11
1.9. ADDITIONAL INFORMATION ...................................................... 11
CHAPTER TWO : SECOND TUTORIAL ................................................... 14
2.1. ANSWERS OF LEARNING ISSUES 1 ............................................ 14
2.2. LEARNING ISSUES 2 ...................................................................... 54
2.3. ANALYSIS OF LEARNING ISSUES 1 ........................................... 54
CHAPTER THREE : THIRD TUTORIAL ................................................... 56
3.1. ANSWERS ......................................................................................... 56
3.2. ANALYSIS LEARNING ISSUES 2 ................................................. 94
3.3. SOLUTION ........................................................................................ 95
3.4. FINAL HYPOTHESIS ....................................................................... 95
3.5. OBSTACLES ..................................................................................... 95
3.6. FINAL CONCEPT MAPPING .......................................................... 95
3.7. ANALYSIS OF FINAL CONCEPT MAPPING ............................... 96
3.8. GROUP OPINION ............................................................................. 97
3.9. CONCLUSION .................................................................................. 98
REFERNCE ................................................................................................... 99

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TABLE LIST
TABLE 1 ........................................................................................................ 58
TABLE 2 ........................................................................................................ 60
TABLE 3 ........................................................................................................ 62
TABLE 4 ........................................................................................................ 70

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PICTURE LIST
PICTURE 1 .................................................................................................... 34
PICTURE 2 .................................................................................................... 39
PICTURE 3 .................................................................................................... 43
PICTURE 4 .................................................................................................... 44
PICTURE 5 .................................................................................................... 59
PICTURE 6 .................................................................................................... 60
PICTURE 7 .................................................................................................... 61
PICTURE 8 .................................................................................................... 62
PICTURE 9 .................................................................................................... 62
PICTURE 10 .................................................................................................. 64
PICTURE 11 .................................................................................................. 64
PICTURE 12 .................................................................................................. 87

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CHAPTER ONE
FIRST TUTORIAL

1.1 SCENARIO
A 50 years old male was escorted by his child to the Emergency Room of a
hospital with complaints weakness in the limbs.

1.2 MAIN PROBLEM
A 50 years old man experiencing weakness in the limbs

1.3 KEYWORDS
weakness in the limbs, degenerative disease, neurological disease,
neurotransmitter.

1.4 EARLY HYPOTHESIS
1.4.1 Mr. A suffered the nervous system disease, which can be in the central
nervous and peripheral nerve
1.4.2 Mr. A suffered locomotor system disease
1.4.3 Mr. A suffered neurotransmitter disease
1.4.4 By notice Mr A age, hes might be suffered a degenative disease.

1.5 COGNITIVE STRATEGY
1.5.1 In the discussion session with tutor, students determine the main problem,
keywords and some early hypothesis about the case. By asking questions
and giving reasons, students collect information related to the patients
condition from the tutor.
1.5.2 Asking the PBL Department through the question list for every
information needed that can not be answered by the tutor.
1.5.3 Brainstorming and sharing informations/experiences
1.5.4 Following the experts lectures
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1.5.5 Self study in the library by reading literatures, journals and collecting
additional information from the internet and textbook with due regard the
principle of evidence based learning
1.5.6 Group discussion and online meeting without the tutor to express other
free ideas
1.5.7 Ask the experts about the management of the case
1.5.8 Composing the review of the tutorial process in group

1.6 EARLY CONCEPT MAPPING

1.7 ANALYSIS OF EARLY CONCEPT MAPPING
A patient came to the emergency room with main problem of pain
and swelling in his right forearm. Deformity occur in distal right forearm
area. His condition can be examined by doing anamnesa and physical
examination. The mechanism of injury must be known. And with
supporting examination such as laboratory through complete blood count
(CBC) and radiology trough X-ray we can describe the pathological and
structural problem better. X-ray also can explain the condition of the
growth plate.
Anamnesa must be done. Anamnesa give information about the
mechanism of injury and surrounding informations about patient sickness
(example : the onset, radiation, characteristics, post injury, patient feeling,
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past and present condition) that important for the therapy that must be
given.
Physical examination is also important. Through physical
examination we can determine if the movement causing the pain, which
structure that might be injured and define if there is abnormality such as
crepitation as a symptoms of fracture. To indicate which structure bones,
muscles, tendons and ligaments, nerves, blood vessels, and articulation.
The occurance of inflammation can also be determine through physical
examination.
Supporting examination helps us to diagnose better. In this case we
need supporting examination such as laboratory imaging through complete
blood count (CBC) and radiology imaging trough X-ray. The data will be
needed for further explanation.

1.8 LEARNING ISSUE
1.8.1 What are the things that must be considered in a physical examination?
1.8.2 How the pathological and physiological reflex and its relation to the
UMN and LMN disorders?
1.8.3 What are the things that must be considered in neurological
examination?
1.8.4 What disease that causes limb weakness?
1.8.5 What is the tensilon test and how it is done to the patient?
1.8.6 What the purpose of the test muscle strength motor = 4-4, 4-4?
1.8.7 What is the musculoskeletal system?
1.8.8 Whatnis the excitable cell?

1.9 ADDITIONAL INFORMATION
Anamnesis:
1. The Man name is A. His age is 50 years old. He lives in Jl. Darmo Permai,
Surabaya. Status : married.
2. He was taken to the emergency room by his son. His son lift Mr. A body
with both hands at chest (Mr. A unable to walk).
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3. Still work as PNS/civil servants, work in office administration
4. Mr. A feel weakness at his arms and legs, weakness since 1 week ago. He
still can work, but so weak.
5. Never suffered this disease before
6. No family suffered like Mr. A.
7. Never do any treatment before, no consume drugs
8. No consume alcohol, no consume milk and other supplement
9. Less able to seen in the afternoon
10. Hard to swallow when eating a lot
11. If longer speak, his voice is lost
12. His weak sense is lost after rest
13. He eats normally, no special nutrition intake
14. Shortness of breath sometimes

Physical Examination:
1. 168 cm height, 60 kg weight, BMI = 21.26
2. Awareness: compos mentis
3. Vital Sign:
blood pressure= 120/70 mmHg
pulse rate= 92/minutes
perfusion= Normal
RR= 36/minutes
Temperature= 36,5C
4. Head and Neck:
Pupillary reflex= (+)/(+)
Icterus (-)
Anemia (-)
Strabismus (-)
Eyelids dimmed (ptosis)
Face = symmetry, no sianosis
Tongue= normal
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No gland enlargement (no neck gland enlargement, no thyroid
enlargement)
Facial sensory= normal
5. Thorax: Normal
6. Abdomen: Normal
7. Extremity: Normal

Reflex Examination (neurological reflex)
1. Extremity reflex for physiologis = (normal, but weak)
APR+1
PTR+1
BPR+1
TPR+1
2. Patological reflex (-) normal
Babinski (-)
Chaddock (-)
3. Motorik = 4-4
4-4
Supporter Examination
1. Tensilon test= + (positive)
2. Blood screening result:
Serum Kreatinin= normal
SGOT= 19 U/L
SGPT= 15 U/L
GDP= 80mg/dl
Fasting Blood glucose= 80 mg/dl, 2 hours after fasting= 120 mg/dl
3. Cholesterol= normal
4. Calcium= normal
5. Uric acid= 5gr/dl (normal)

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CHAPTER TWO
SECOND TUTORIAL

2.1 ANSWERS OF LEARNING ISSUES 1
1. What are the things that must be considered in a physical
examination?
Before we diagnose the patient, after anamnesis, we can do the
physical examination. In physical examination, we must do systematically. So,
no part will we missed. Begin from Head and Neck, chest, Abdomen, and
limb.
Based on History taking and physical examination book by
Greenberger and Hinthorn (1993), complement that must we record in
physical examination is:
Head and Neck
1. HEAD
During we do examination from head, we must observe: size, shape,
symmetry, contour, tenderness, bruits; in children, check if fontanelle is
closed
The component of Head that will be examined is:
A. Eyes
External:
conjunctivae, sclera, pupil size and reaction, ptosis, arcus
senilis, protrusion, gross visual acuity, visual fields by
confrontation, extraocular movements, reaction to light and
accommodation, nystagmus.
Funduscopic:
Red reflex,lenticular opacity, optic disc, arteries, veins,
hemorrhages, exudates, microaneurysms, and photophobia. For
visual fields by confrontation, draw diagram indicating site and
type of abnormality
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B. Ears
Normal, pinna, tophi, external canal,tympanic membrane, discharge,
cerumen, hearing, Webers test, Rinne test.
C. Nose
Normal, septum (deviation or perforation), mucosa, airway
obstruction, discharge, enlarged turbinates, polyps, sinus tenderness; if
sinus disease suspected, transilluminate sinuses
D. Mouth and Throat
Normal, odor of breath, color and appearance of lips, tongue, gums;
conditions of teeth including caries, dentures (remove to examine
palate), tonsils, uvula, soft and hard palate, larynx, epiglottis, gentle
percussion of teeth, salivary glands, rigidity or limitation of motions,
thyroid, trachea, venous distention, anterior cervical nodes,
submaxillary nodes, postero cervical nodes, carotid and jugular pulses,
and bruits.
2. NECK
Thyroid Gland
Inspection:
The patient looked up a little, swallow saliva, noted: form and symmetry
Palpation:
The patient and the examiner sitting in the back, middle finger and index
finger to the two hands placed on both isthmus, along the trachea halted
touch krokoid of bone and laterally, note: a lump; konsidstensi, shapes,
sizes.
Auscultation:
Place the bell on the thyroid gland, note: the presence of noise (normal:
absence of
noise)
Trachea
Inspection:
Examining addition to the right patient, stick the middle finger on the
bottom of the trachea, touch up and to the side, note: the location of the
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trachea, symmetry, mark oliver (when the heart rate, trachea pulled
down),
Normally: symmetrical middle.
JVP (jugular venous pressure)
Position the patient lying half sitting, specify the upper limit of the jugular
venous pulse, let the patient change position to sit and observe the venous
pulsation rate. Normally: when sitting as high as the manubrium of the
sternum.
Or
Patient lying half-sitting position, determine the zero point (highest point
of the manubrium sterni) And place it on top of a ruler, determine the
upper limit venous pulsation, venous pulse height measuring with a ruler.
Normally: not more than 4 cm.
Carotid Artery noisy
Determine the location of the carotid pulse (from the middle of the neck
sliding sideways), Put the bell side of the stethoscope in the area of the
carotid artery, record the presence of noise. Normally: no noise.

CHEST
During we do examination from head, we must observe: general
configuration, symmetry, movement with respiration.
Respiratory Inspection: labored, shallow, kussmaul, periodic, or other
breathing; use the accessory muscles in breathing
Palpation: palpate for areas of tenderness (costochondral junctions), access
respiratory excursion, symmetry, dullness, hyperresonance
Auscultation: cracles, wheezes, rubs, rales, rhonci, egobronchophony,
whispered, pectoriloquy, fremitus.
A. Breast
Size, consistency, symmetry, tenderness, palpable masses, retraction,
ulceration, asymmetry, dimpling, discharge from nipple,
gynecomastia.
B. Back
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Mobility, kyphosis, lordosis, scoliosis, tenderness on palpation or
percussion, tenderness to CVA percussion, sacral edema.
C. Heart
Inspection: precordium for abnormal pulsations, heaving, or bugling;
point of maximal impulse
Palpation: precordium for shocks, thrill, rubs
Percussion: Heart size
Auscultation: ascultate at valve areas, rate, rhythm; quality of heart
sounds; extra sounds; draw diagram indicating timing of extra
sounds, murmurs, and rubs; note areas where each sound is most
intense.
ABDOMEN
A. Inspection: scaphoid, flat, distended, obese, dilated veins, scars, striae
gravidarum; describe intrinsic movement, scars, hair distribution
- Malnutrition
- Dehydration
- Hyperpigmentation
- Scars
- Striae
- Visible pulsations
- Visible peristalsis
- Dilated veins
- Diastasis recti
B. Palpation: tenderness, rigidity, rebound, guarding, mass, fluid wave,
hernia, liver span, spleen, kidneys, referred pain
- Liver
- Spleen
- Gallbladder
- Kidneys
- Aorta
- Masses
- Stomach
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- Bowel loops
- Bladder
- Pelvic/rectal
- Hernias
- Tenderness (reproducibility)
- Rebound tenderness
- Jar tenderness
- Rigidity, guarding
- Abdominal pain
C. Percussion: tympany, shifting dullness, sizes of organs or masses
D. Auscultation: bowel sounds (pitch, absent, rushes), rubs, bruits ; note
abnormalities on appropriate diagram
- Peristaltic sounds
- Bruits
- Succussion splash
- Friction rubs
EXTREMITIES
Joint swelling, tenderness, redness, heat, deformity, and limitation of
motion; edema (if pitting is present, grade 1+ through 4+), cyanosis,
varicosities, ulceration, clubbing, rash on palms or soles, color and
temperature of legs, hair growth on legs, calf tenderness, muscle
weakness, condition of nails (e.g., pitting, lines, configuration, tinea)
2. How the pathological and physiological reflex and its relation to the
UMN and LMN disorders?
According to Larry (2003), reflexes are the bodys intrinsic stimulus response
systems for maintaining homeostasis, and are often used for diagnosing and
localizing nervous system disorders. A reflex consist of two or more neurons
through which nervous impulses are transmitted to the brain or spinal cord from a
receptor, and then to an effector. If the reflex is interrupted, effector response is
diminished or absent.
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Reflexes divided into 4 groups:
1. SUPERFICIAL REFLEXES
Superficial reflexes are usually elicited by stroking the skin or mucous
membranes. Mita (2013) says that, Superficial reflexes checked by scraping the
skin with a hard object (eg, the end of the reflex hammer or applicator) that
causes muscle contraction.
A. Skin Reflexes
1. Anal reflexAction: Stroke the perianal area or insert gloved finger into
rectum. Response: Contraction of the sphincter ani.
2. Cremasteric reflexAction: Stroke inner thigh. Response: Testicular
elevation.
3. Gluteal reflexAction: Stroke buttocks. Response: Contraction of
buttocks.
4. Interscapular reflexAction: Stroke skin of interscapular space.
Response: Scapulas draw inward.
5. Plantar reflexAction: Stroke sole of feet. Response: Plantar flexion of
toes.
6. Upper and lower abdominal reflexesAction: Medially stroke each side
of abdomen above and below the umbilicus. Response: Umbilical
deviation toward the stimulus.
B. Mucous Membrane Reflexes
1. Corneal reflexAction: Touch cornea with wisp of cotton. Response:
Blinking
2. Gag reflexAction: Irritate pharynx with tongue blade. Response:
Gagging.
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3. Sneeze reflexAction: Irritate nasal membrane. Response: Sneezing.
4. Uvular reflexAction: Phonation of Ahh or irritation of posterior third
of tongue with tongue blade. Re- sponse: Uvular elevation.
2. DEEP REFLEXES
Deep tendon reflexes can be elicited by tapping a reflex hammer quickly
and firmly on the tendon partially stretched. Deep tendon reflexes is also called
muscle stretch reflex (Mita, 2013).
1. Achilles reflexAction: Strike achilles tendon. Response: Plantar
flexion of foot.
2. Biceps reflexAction: Strike biceps tendon. Response: Elbow flexion.
3. Maxillary reflexAction: Striking middle of chin with mouth slightly
open. Response: Sudden jaw closure.
4. Patellar reflexAction: Strike patellar tendon. Response: Knee
extension.
5. Radial reflexAction: Strike radius above wrist.
6. Triceps reflexAction: Strike triceps tendon. Response: Elbow
extension.
7. Ulnar reflexAction: Strike ulna above wrist. Response: Extension and
ulnar deviation of wrist.
3. VISCERAL REFLEXES
A. Pupillary Reflexes
1. Accommodation reflexAction: Patient looks at distant object, then near
object. Response: Pupillary constriction and ocular convergence.
2. Consensual light reflexAction: Shine light into opposite eye.
Response: Pupillary constriction.
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3. Ciliospinal reflexAction: Pinch neck. Response: Pupilary dilation.
4. Light reflexAction: Shine light onto retina. Response: Pupillary
contraction.
B. Blink Reflex
Action: Unexpected, abrupt movement of object toward eyes. Response:
Blinking or eyelid closure.
C. Oculocardiac Reflex
Action: Pressure directly over closed eyes. Response: Slowing of heart
rate.
D. Carotid Sinus Reflex
Action: Pressure over carotid sinus. Response: Slowing of heart rate and
lowering of blood pressure.
E. Bulbocavernosus Reflex
Action: Stroking, pinching or pricking the dorsum glans penis. Response:
Contraction of the bulbocavernosus muscle.
F. Bladder and Rectal Reflexes
Action: Interruption of afferent fibers. Response: Dimin- ished urge to
urinate or defecate.
Action: Interruption of efferent fibers. Response: Incon- tinence.
G. Mass reflex
Action: Spinal cord interruption or emotional arousal, such as fear.
Response: Sudden emptying of bowel and bladder.
4. PATHOLOGICAL REFLEXES
The more common pathological reflexes demonstrating upper motor
neuron syndrome are anatomically grouped below:
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A. Lower Extremity
1. Ankle clonusAction: Forcibly and quickly dorsifle ing the foot while
holding up the leg under the popliteal space. Response: Continued rapid
flexion and extension of the foot.
2. Babinskis signAction: Stroking the plantar surface of the foot from
heel to great toe, starting from the lateral side and sweeping across to the
medial side at the ball of the foot. Response: Extension of the great toe.
3. Chaddocks toe signAction: Stroking the lateral mal- leolus. Response:
Extension of the great toe.
4. Gonda reflexAction: Pressing a toe down (other than the great toe) and
releasing it with a snap. Response: Extension of the great toe.
5. Gordons leg signAction: Squeezing the calf muscle. Response:
6. Hoovers signAction: a) The hemiplegic patient is supine. b) The
examiners palms are placed under the patients heels. c) Patient presses
down. Only the nonparalyzed leg will exert pressure. d) Exam- iners hand
is placed on the dorsum of the nonparalyzed leg. e) Patient raises the well
leg against the examiners resisting hand. Response: a) If true hemiplegia,
no additional pressure will be felt by the hand under the paralyzed leg. b)
If hysterical paralysis, additional pressure will be felt as the at-tempt is
made to raise the well leg.
7. Huntingtons signAction: Supine patient coughs and strains. Response:
Hip flexion, knee extension, and elevation of affected weak lower
extremity.
8. Marie-Foix retraction signAction: Forcing toes downward. Response:
Knee and hip flexion.
9. Oppenheims signAction: Caudal stroking of the tibia and tibialis
anterior muscle. Response: Extension of the great toe.
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10. Patellar clonus (trepidation sign)Action: Forcibly and quickly
depressing the patella while the leg is in extension and relaxed. Response:
Rapid up-and-down movement of the patella.
11. Rossolimos signAction: Tapping the ball of the foot. Response:
Flexion of the toes.
12. Schaefers signAction: Squeezing the Achilles tendon. Response:
B. Upper Extremity
1. Babinskis pronation signAction: Patients supinated hands are
approximated. Examiner jars the hands several times from below.
Response: Affected hand falls in pronation, while the sound hand remains
unaffected.
2. Bechterews signAction: Patient flexes and relaxes both forearms.
Response: Paralyzed forearm falls back more slowly and in a jerky
manner.
3. Chaddocks wrist signAction: Stroking ulnar side of forearm near
wrist. Response: Wrist flexion, with fanning and extension of the fingers.
4. Gordons finger signAction: Pressure applied over the pisiform bone.
Response: Extension of flexed fingers or thumb.
5. Grasping signAction: Firm, radial stroking of examiners fingers
across patients palm. Response: Grasping reaction.
6. Hoffmans signAction: Flicking the distal phalanx of the index finger.
Response: Clawing movement of the fingers and thumb.
7. Klippel-Weil thumb signAction: Examiner quickly extends flexed
fingers of patient. Response: Flexion and adduction of thumb.
8. Leris signAction: Forceful passive flexion of the wrist and fingers.
Response: Absence of normal flexion of elbow.
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9. Stru mpells pronation signAction: Flexing the forearm. Response:
Dorsum of the hand, instead of the palm, approaches the shoulder.
10. Tro mners signAction: Sharp tapping on the palmar surface or tips of
middle three fingers. Response: Finger flexion.
C. Head
1. Babinskis platysma signAction: Flexion of chin to chest or opening
the mouth against resistance. Response: Platysma will contract only on the
unaffected side.
2. Head retraction reflexAction: Patients head is slightly inclined
forward. Upper lip is sharply percussed downward. Response: Head
bending, followed by brisk head retraction.
3. McCarthys sign (glabella reflex)Action: Percussion of the supraorbital
ridge. Response: Reflex contraction of orbicularis oculi muscle.
4. Snout reflexAction: Sharp tapping on middle upper lip. Response:
Exaggerated reflex contraction of lips.
UMN or Upper Motor Neurons derive from cerebral cortex and panhandle
to below, one part (kortikobulbaris tract) ended in the brain stem, while the other
part (corticospinal tract) cross section the lower part of medulla oblongata and
down to the spinal cord. The cranial nerve nuclei is the tip of corticobulbaris tract.
The corticospinal tract end in the cervical cornu anterior spinal cord area until
sacral. Spinal fibers which cross medulla oblongata pyramid form pyramidal tract.
LMN include motoric cells cranial nerve nuclei and its axon and anterior horn
cells of the spinal cord and its axon. Motor fibers exit through the anterior roots or
motor of the spinal cord then innerve the muscles (Mita, 2013).
UMN and LMN lesions causing characteristic changes in muscle response.
Knowledge of the differences in muscle weakness will facilitate determining the
neurological lesion. The difference between UMN and LMN lession is:

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UMN (Upper Motor Neuron) signs
The type and distribution of weakness, lesions in the brain: "pyramidal
distribution" is part of the distal muscles especially hand, arm extensor
and flexor weaker leg.
The tone of the muscle become spasticity, which is more pronounced in
the flexor and extensor leg sleeve
Only slightly disuse atrophy
There are pathological reflexs such use babinski
No fasciculation
Often happen clone
According to the University of Bristol (no date), Can be further localised
by the other symptoms that go along with the weakness. For example:
A cord lesion may also cause sphincter symptoms, a sensory level,
bilateral motor signs.
A brain stem lesion may also cause dysarthria, dysphagia, Horner's
syndrome, cerebellar signs, spinothalamic sensory loss.
A lesion of the motor cortex may be associated with frontal signs,
dysphasia, hemianopia, disturbance of higher sensory function eg
agnosias.
LMN (Lower Motor Neuron) signs
The type and distribution of weakness, lesions in the spinal cord: has
variations dependent lesion in where segmen.
Tone of the muscle become flaccid or weaker
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Atrophy can be very clearly
No pathological reflexes
There is fasciculations
Nothing clone
According to the University of Bristol (no date), Again the level of the
problem can generally be inferred from accompanying symptoms and signs,
for example:
- Back pain and sciatica suggests a root problem
- Weakness of the biceps with absence of the biceps reflex, with upper
motor neurone signs in the legs suggests cord disease (eg a disc) at
C5/6 (LMN at that level, UMN below).
- Weakness of thumb abduction, wasting of the thenar eminence and
numbness in the thumb and lateral 21/2 fingers suggests median
nerve pathology.
The relation between reflex and UMN and LMN
Based on larry (2003), the combination of diminished or absent
superficial reflexes with deep reflexes and pathological reflexes indicates
upper motor neuron (UMN) involvement. Hyperreflexia is consistent with
an UMN, conversely hyporeflexia is consistent with a LMN. Clonus or
muscle rigidity (spastic paralysis) may be present with an UMN, whereas
flaccid paralysis may be present with a LMN.
Upper Motor Neuron paralysis caused by interruption of descending
motor pathways on one side of the spinal cord segment. Immediately after
the lesion, the deep tendon reflex will be depressed for a while. This
situation is called areflexia. In addition, the paralyzed muscles will be
paralyzed (flaccid). A few weeks or months after the lesion, the deep
tendon reflexes becomes hyperactive. Superficial reflexes disappear and
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become positive Babinski reflex. Lower Motor Neuron paralysis caused by
destructive LMN paralysis caused by destructive peripheral motor nerves
and anterior horn cells. If there LMN paralysis, muscle becomes flaccid,
hipotonus, and deep tendon reflexes are lost.peripheral motor nerves and
anterior horn cells. If there LMN paralysis, muscle becomes flaccid,
hipotonus, and deep tendon reflexes are lost (Mita, 2013)

3. What are the things that must be considered in neurological examination?
Components of a Neurological Assessment:
1. Interview
2. Level of Consciousness
3. Pupillary Assessment
4. Cranial Nerve Testing
5. Vital signs
6. Motor Function
7. Sensory Function
8. Tone
9. Cerebral Function
1. INTERVIEW
The patient/family interview will allow the nurse to:
gather data: both subjective and objective about the patient's
previous/present health state
provide information to patient/family
clarify information
make appropriate referrals
develop a good working relationship with both the patient and the
family
initiate the development of a written plan of care which is patient
specific
Interview to identify presence of:
headache
28
difficulty with speech
inability to read or write
alteration in memory
altered consciousness
confusion or change in thinking
disorientation
decrease in sensation, tingling or pain
motor weakness or decreased strength
decreased sense of smell or taste
change in vision or diplopia
difficulty with swallowing
decreased hearing
difficulty with swallowing
altered gait or balance
dizziness
tremors, twitches or increased tone

2. LEVEL OF CONSCIOUSNESS
Consciousness can be defined as a state of general awareness of
oneself and the environment (Goldberg, 2008). Consciousness is
difficult to measure directly but it is estimated by observing how
patients respond to certain stimuli.

Physiologic Basis for Consciousness
1. Reticular Activating System (RAS)
Loose network of neurons and fibres in the brainstem which
receive input from spinothalamic (sensory) pathways and project to the
entire cerebral cortex. Arousal is dependent on the adequate
functioning of the RAS. Arousal is purely a function of the brain stem.
It does not have anything to do with the thinking parts of the brain.
The fact that your patient opens his/her eyes when you call their name
29
is an indication that their RAS (brainstem) functioning is intact but it
does not tell you if they are awake or aware.
2. Cortex
Modulates incoming information via connections to the RAS.
Therefore, the cortex requires functioning of the RAS to function
itself. Awareness, means that the cerebral cortex is working and that
the patient can interact with and interpret his environment.
We can evaluate awareness in many ways but tend to focus on four
areas of cortical functioning: orientation, attention span, language, and
memory. Consciousness will be disturbed if a lesion of the RAS is
present or if there is diffuse damage to the cortex (both hemispheres).
Some mechanisms by which consciousness is disturbed:

Diffuse cortical dysfunction:
decreased cerebral metabolism:
hypoxia, hypoglycemia, acidosis/alkalosis, hyponatremia
drugs:
alcohol, barbiturates, phenytoin, phenothiazines, benzodiazepines,
methanol, ethylene glycol, paraldehyde
hypotension:
decreased cerebral blood flow
structural lesions:
infarctions, hemorrhages, tumours
Lesions of the RAS
Occasionally a lesion occurs directly in the upper brainstem (e.g.
bleed, infarction, tumour) and causes coma by destruction of RAS.
More often, a large destruction cortical lesion causes secondary
damage to the RAS via: herniation or direct extension of the lesion into
the midbrain or diencephalon.
Assessment of Level of Consciousness
A. Stimulate with progressively stronger stimuli:
30
i) normal voice
ii) shout
iii) light touch
iv) pain

The Glasgow Coma Scale (GCS) helps us to decrease the
subjectivity of our responses. The GCS is not intended to identify focal
findings; it is a rating score to grade the best possible central (brain)
response. Remember to give each score the BEST possible rating. If
the patient can only move one eyebrow to command, they are still
given a 6 for motor score.
3. PUPILLARY ASSESSMENT
When assessing pupils (eyes) it is important to assess the following:
size
shape
reactivity to light
comparison of one pupil to the other
4. CRANIAL NERVE ASSESSMENT
5. VITAL SIGNS
Changes in vital signs are not consistent early warning signals. Vitals
are more useful in detecting progression to late symptoms. Both
respiratory and cardiac centres are located in the brainstem. Therefore,
compression of the brainstem will cause changes in vital signs. This is
usually a late sign and impending herniation/death will occur if the
problem is not resolved. The respiratory centres in the brainstem
control rate, rhythm, inspiration/expiration. The cardiac centres also
play a part in cardiac acceleration/inhibition e.g. controlling heart rate
and rhythm as well as hemodynamic stability/instability.
31
Respiratory
The role of the Nurse is to:
1. Ensure patent airway is maintained
2. Assess rate, rhythm, and characteristics of inspiration/expiration
3. Assess gas exchange, tissue perfusion, airway clearance, and risk for
aspiration
4. Assess for causes of respiratory disturbances or secondary
conditions that can cause respiratory complications
5. Assess for actual respiratory complication/insufficiency and
intervene appropriately
6. MOTOR FUNCTION
When assessing motor function, from a neurological perspective, the
assessment should focus on arm and leg movement. You should
consider the following:
1. muscle size
2. muscle tone
3. muscle strength
4. involuntary movements
5. posture, gait
Symmetry is the most important consideration when identifying focal
findings. Compare one side of the body to the other when performing
your assessment.

32
7. SENSORY FUNCTION
When assessing sensory function remember that there are three main pathways for
sensation and they should be compared bilaterally:
1. pain and temperature sensation
2. position sense (proprioception)
3. light touch
8. TONE
Upper motor neuron problems (brain and spinal cord) are associated with
increased tone. Lower motor neuron problems are associated with decreased tone.
Look at the muscles on each side of the body in pairs. Assess for symmetry of
bulk.
Evaluation of the stretch reflexes assesses the intactness of the spinal
reflex arc at various spinal cord levels. The limb should be relaxed while applying
a short and snappy blow with a reflex hammer. Hold the hammer loosely in a
relaxed manner, making a wrist action. Allow the hammer to bounce.
Reflex responses:
0 no response
1+ diminished, low normal
2+ average, normal
3+ brisker than normal
4+ very brisk, hyperactive
Lower motor neuron disease is associated with 0 or 1+, upper motor
neuron disease is associated with 3+ or 4+.

33
Biceps Reflex (C5 C6 )
Triceps Reflex (C7-C8)
Brachioradialis Reflex (C5-C6):
Quadriceps Reflex (Knee jerk) L2 L4
Achilles Reflex (ankle jerks) L5 S2
Heel Lift
Babinski Response
9. CEREBELLAR FUNCTION
The cerebellum is responsible for muscle coordination and balance on
the same side. To test cerebellar function use the following tests:
1. Finger to finger test: have the patient touch their index finger to your
index finger (repeat several times).
2. Finger to nose test: perform with eyes open and then eyes closed.
3. Tandem walking: heel to toe on a straight line
4. Romberg test: stand with feet together and arms at their sides. Have
patient close his/her eyes and maintain this position for 10 seconds. If
the patient begins to sway, have them open their eyes. If swaying
continues, the test is positive or suggestive of cerebellum problems.
Dizziness that occurs in response to position changes is usually blood
pressure initiated. If the patient sways during a Romberg test, but stops
when the eyes are opened, the problem is probably visual or CN VIII
(vestibular).

34
Intensity of Muscle motoric system
Muscle Tonus
Patient were asked to relax the limb to be examined. Then move to the
position of flexion and extention at the knee and elbow joint . In normal
individuals theis is a reasonable resistency.
Flaccid: their is no resistence (in a LMN paralysed)
Hypotoni: minus resistence
Spastic: strong resistence at the beginning of the movement (in UMN
paralysed)
Rigid: strong resistence and continous every movement (e.g: parkinson)
4. what disease that causes limb weakness?
1. Subdural Hematome (SDH)
Definition and Cause
Subdural Hematome is collective blood between
duramater and arachnoidea which cause by rupture of
veins or artery. Subdural Hematom or known as SDH
usually attack on alcoholic or elderly. It is usually
caused by a seriously head injury, or very minor injury
but happened many times. An elderly who often had
small head injury but more than once can cause SDH.
The other factor that cause SDH are:
Anticoagulant medication (blood thinners,
including aspirin)
Long-term abuse of alcohol
Recurrent falls
Repeated head injury
Very young or very old age
Symptoms and Signs
Confused speech
Difficulty with balance or walking
Picture 1 : Subdural Hematome CT Scan
Source :
http://www.nhs.uk/Conditions/Subdural-
haematoma/Pages/Introduction.aspx
35
Headache
Lethargy or confusion
Loss of consciousness
Nausea and vomiting
Numbness
Seizures
Slurred speech
Visual disturbances
Weakness extremity
In infants:
Bulging fontanelles (the "soft spots" of the baby's skull)
Feeding difficulties
Focal seizures
Generalized tonic-clonic seizure
High-pitched cry
Increased head circumference
Increased sleepiness or lethargy
Irritability
Persistent vomiting
Separated sutures (the areas where growing skull bones join)
Types
A subdural haematoma can be:
Acute the haematoma forms immediately after the initial
injury
Subacute- the haematoma forms up to a week after the initial
injury
Chronic the haematoma forms over a period of two to three
weeks after the initial injury

2. Amyothropic Lateral Scleriosis (ALS)
36
Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's
disease, is a rapidly progressive, invariably fatal neurological disease that
attacks the nerve cells (neurons) responsible for controlling voluntary
muscles (muscle action we are able to control, such as those in the arms,
legs, and face). The disease belongs to a group of disorders known as
motor neuron diseases, which are characterized by the gradual
degeneration and death of motor neurons.
The cause of ALS is not known, and scientists do not yet know why ALS
strikes some people and not others. An important step toward answering
this question was made in 1993 when scientists supported by the National
Institute of Neurological Disorders and Stroke (NINDS) discovered that
mutations in the gene that produces the SOD1 enzyme were associated
with some cases of familial ALS. Although it is still not clear how
mutations in the SOD1 gene lead to motor neuron degeneration, there is
increasing evidence that mutant SOD1 protein can become toxic.
In searching for the cause of ALS, researchers are also studying the role of
environmental factors such as exposure to toxic or infectious agents, as
well as physical trauma or behavioral and occupational factors. For
example, studies of populations of military personnel who were deployed
to the Gulf region during the 1991 war show that those veterans were more
likely to develop ALS compared to military personnel who were not in the
region.
Symptoms and Signs
At the onset of ALS the symptoms may be so slight that they are
frequently overlooked. With regard to the appearance of symptoms and the
progression of the illness, the course of the disease may include the
following:
muscle weakness in one or more of the following: hands, arms,
legs or the muscles of speech,
swallowing or breathing
twitching (fasciculation) and cramping of muscles, especially those
in the hands and feet
37
impairment of the use of the arms and legs
"thick speech" and difficulty in projecting the voice
in more advanced stages, shortness of breath, difficulty in
breathing and swallowing

3. Guillain-Barr Syndrome
Guillain-Barr Syndrome is a disorder affecting pheriperal nervous
system. It is attacked by imunosystem of body. Guillain-Barr syndrome
can affect anybody. It can strike at any age and both sexes are equally
prone to the disorder. The syndrome is rare, however, afflicting only about
one person in 100,000. Usually Guillain-Barr occurs a few days or weeks
after the patient has had symptoms of a respiratory or gastrointestinal viral
infection. Occasionally surgery will trigger the syndrome. In rare instances
vaccinations may increase the risk of GBS.
No one yet knows why Guillain-Barr which is not contagious
strikes some people and not others. Nor does anyone know exactly what
sets the disease in motion.
Symptoms and Signs
The symptoms can spread quickly. It happened within few hours. It is
uasually started at hands before spreading to others.
pain, tingling and numbness
progressive muscle weakness
co-ordination problems and unsteadiness (you may be unable to
walk unaided)
In mild cases of Guillain-Barr syndrome, your muscles may only be
slightly weakened. However, in more severe cases, the muscle weakness
can progress to:
temporary paralysis of the legs, arms and face
temporary paralysis of the respiratory muscles
blurred or double vision
difficulty speaking
38
difficulty chewing or swallowing (dysphagia), resulting in the need
to be fed through a tube
difficulty with digestion or bladder control
fluctuations in heart rate or blood pressure
Complications
This may happen on patient with GBS
respiratory failure where your lungs are unable to provide
enough oxygen for the rest of your body
infections particularly respiratory infections in people who are
on a ventilator (a machine that assists with breathing)
heart rhythm disorders including cardiac arrest
bowel obstruction
Diagnostic
Beside the symptoms that may occure, a GBS person can diagnostic by:
Electromyography (EMG)
Lumbar puncture
physical examination
muscle strength tests
muscle activity tests
reflex tests, such as the knee-jerk reaction
nerve conduction velocity tests
spinal tap, to check for higher than expected levels of protein in the
cerebrospinal fluid.

4. Myasthenia Gravis
Myathenia gravis is chronic autoimun disease from neuromuscular
transmission that caused weakness of muscle. Based on medical
dictionary, autoimun disease is a kind of disease where antibody attacked
the tissues.
39
Myasthenia gravis is caused by a defect in the transmission of nerve
impulses to muscles. Antibody blocked, changed, ruined the accepting
acethylcolin (ACTH) so blocked muscle work.
Myasthenia gravis is caused by a defect in the transmission of nerve
impulses to muscles. It occurs when normal communication between the
nerve and muscle is interrupted at the neuromuscular junctionthe place
where nerve cells connect with the muscles they control. Normally when
impulses travel down the nerve, the nerve endings release a
neurotransmitter substance called acetylcholine. Acetylcholine travels
from the neuromuscular junction and binds to acetylcholine receptors
which are activated and generate a muscle contraction.
In myasthenia gravis, antibodies block, alter, or destroy the receptors for
acetylcholine at the neuromuscular junction, which prevents the muscle
contraction from occurring. These antibodies are produced by the body's
own immune system. Myasthenia gravis is an autoimmune disease because
the immune systemwhich normally protects the body from foreign
organismsmistakenly attacks itself.
Symptoms and Signs
Picture 2 Myasthenia Gravis
Source: http://medical.cdn.patient.co.uk/images/060.gif
40
The muscles around the eyes are most commonly affected first.
This causes drooping of the eyelid (ptosis), and double vision.
Difficulty in swallowing and slurred speech may be the first signs
of myasthenia gravis.
Weakness in the arms, hands, fingers, legs and neck may develop.
Weakness in the chest muscles sometimes occurs. If this is severe,
a myasthenic crisis may result (see below).
Diplopia
Disturbed breathing
All the symptoms may occure when the patients feel tired and
doing activity for a long time. And it will be better after having a
rest.
Complication
Complications of myasthenia gravis are treatable, but some can be
life-threatening.
Myasthenic crisis
Myasthenic crisis is a life-threatening condition that occurs when
the muscles that control breathing become too weak to do their
jobs. Emergency treatment is needed to provide mechanical
assistance with breathing. Medications and blood-filtering
therapies help people to again breathe on their own.
Thymus tumors
About 15 percent of people with myasthenia gravis have a tumor in
their thymus, a gland under the breastbone that is involved with the
immune system. Most of these tumors, called thymomas, aren't
cancerous (malignant).
People with myasthenia gravis are more likely to have the
following conditions:
Underactive or overactive thyroid. The thyroid gland, which is in
the neck, secretes hormones that regulate your metabolism. If your
thyroid is underactive, you may have difficulties dealing with cold,
41
weight gain and other issues. An overactive thyroid can cause
difficulties dealing with heat, weight loss and other issues.
Autoimmune conditions. People with myasthenia gravis may be
more likely to have autoimmune conditions, such as rheumatoid
arthritis or lupus.
Diagnostic
To diagnose your condition, your doctor will review your symptoms
and your medical history and conduct a physical examination. Your
doctor may conduct several tests, including:
Neurological examination - Your doctor may check your
neurological health by testing your:
Reflexes
Muscle strength
Muscle tone
Senses of touch and sight
Coordination
Balance
The key sign that points to the possibility of myasthenia gravis
is muscle weakness that improves with rest. Tests to help
confirm the diagnosis may include:
Edrophonium test - Injection of the chemical edrophonium
chloride (Tensilon) may result in a sudden, although temporary,
improvement in your muscle strength. This is an indication that
you may have myasthenia gravis. Edrophonium chloride blocks
an enzyme that breaks down acetylcholine, the chemical that
transmits signals from your nerve endings to your muscle
receptor sites.
Ice pack test - If you have a droopy eyelid, your doctor may
conduct an ice pack test. In this test, a doctor places a bag filled
with ice on your eyelid. After two minutes, your doctor
removes the bag and analyzes your droopy eyelid for signs of
42
improvement. Doctors may conduct this test instead of the
edrophonium test.
Blood analysis - A blood test may reveal the presence of
abnormal antibodies that disrupt the receptor sites where nerve
impulses signal your muscles to move.

Repetitive nerve stimulation - In this nerve conduction study,
doctors attach electrodes to your skin over the muscles to be
tested. Doctors send small pulses of electricity through the
electrodes to measure the nerve's ability to send a signal to your
muscle. To diagnose myasthenia gravis, doctors will test the
nerve many times to see if its ability to send signals worsens
with fatigue.
Single-fiber electromyography (EMG) - Electromyography
(EMG) measures the electrical activity traveling between your
brain and your muscle. It involves inserting a fine wire
electrode through your skin and into a muscle. In a single-fiber
EMG, doctors test a single muscle fiber. Most people find this
test to be uncomfortable.
Imaging scans - a CT scan or an MRI to check if there's a
tumor or other abnormality in your thymus.
Pulmonary function tests - to evaluate whether your
condition is affecting your breathing.

5. What is the tensilon test and how it is done to the patient?
The Tensilon test is a method to help diagnose myasthenia gravis
(MG). The tensilon test or "edrophonium test" is infrequently performed to
identify MG. Its application is limited to those situations in which other
investigations have failed to yield a conclusive diagnosis. This test
requires the intravenous (IV) administration of edrophonium chloride
(Tensilon, Reversol) or neostigmine (Prostigmin), drugs that block the
breakdown of acetylcholine by cholinesterase (acetylcholinesterase
43
inhibitors) and temporarily increases the levels of acetylcholine at
the neuromuscular junction. In people with myasthenia gravis involving
the eye muscles, edrophonium chloride will briefly relieve weakness. The
test may be repeated and you may have other Tensilon tests to help tell the
difference between myasthenia gravis and other conditions.

Picture 3. Ptosis in Myasthenia Gravis patientSource:
http://en.wikipedia.org/wiki/File:Myasthenia_gravis_ptosis_reversal.jpg
Description : Photograph of the patient showing right partial ptosis. The
left lid shows compensatory pseudo lid retraction because of equal
innervation of the levator palpabrae superioris ( Herringss law ). b. Post
tensilon test: Note the improvement in ptosis
There are two special clinical tests that the doctor might perform
looking for MG. These are the ice test and the Tensilon test. In the ice test
the doctor will examine for improvement in eyelid drooping after covering
the eye for a minute or two with an ice pack or a cold pack. In the Tensilon
test the doctor will examine for improvement in any weak area of the body
after administration of the medication Tensilon. A medicine called
Tensilon (also called edrophonium) or a dummy medicine (inactive
placebo) is given during this test. This medication only works when
injected into the vein, and only has a short duration of effect. A medicine
called atropine may be given before receiving Tensilon so that the patient
do not know they are getting the medicine. Variations on this test include
the injection of neostigmine, and taking a tablet of Mestinon, again see if
there is any improvement of weakness. Obviously both the ice test and the
Tensilon test require something abnormal to be present at the time of the
test, in other words the tests cannot be done if there are no abnormal
findings on examination at the time.
44
The abnormal results mean that many patients with myasthenia
gravis, the muscles will improve right after the Tensilon.
The improvement lasts only a few minutes. For some types of myasthenia,
Tensilon can make the weakness worse. When the disease gets worse
enough to need treatment (myasthenic crisis), there is a brief improvement
in muscle strength. When there is an overdose of anticholinesterase
(cholinergic crisis), Tensilon will make the person even weaker.
The medicine used during the test may cause side effects, including
fainting or breathing failure. This is why the test is done by a health care
provider in a medical setting.
Edrophonium is a readily reversible acetylcholinesterase inhibitor.
It prevents breakdown of the neurotransmitter acetylcholine and acts by
competitively inhibiting the enzyme acetylcholinesterase, mainly at
the neuromuscular junction. It is sold under the trade
names Tensilon and Enlon (according to FDA Orange Book).
Edrophonium, ethyl-(3-hydroxyphenyl) dimethylammonium chloride, is
made by reacting 3-dimethylaminophenol with ethyl bromide, which
forms ethyl (3-hydroxyphenyl) dimethylammonium bromide, the bromine
atom of which is replaced with a chlorine atom by reacting it with silver
chloride, giving edrophonium.
.

Picture 4. Chemical structure of Edrophonium
Source : .A. Aeschlimann, A. Stempel, U.S. Patent 2,647,924 (1953).

Myasthenia Gravis (MG) is an autoimmun disorder where the number of
acethylcholin receptor, one kind of the neurotransmitter, in postjunctional
membrane decrease and antibody in the receptor block the transmission of nerve
impuls.
45
Neuromuscular transmission depend on releasing the ACh package in
nerve terminal, then it followed by the reaction of AChR in postsynaptic
membrane. Reaction of this ion make the gate depolarize and the end-plate
potential increase. After depolarization reach the maximum number, potential
action will spread around muscle membrane and it activated the contractile
apparatus. In physiology abnormality, the decreasing of end-plate potential
amplitudo will fail the releasing of potential action.
The symptoms of Myasthenia Gravis such as fluctuative weakness of
muscle, that is usually involves extraoculer muscle and muscle of the eyelids,
ptosis, caused by the weakness of m. levator palpebrae superior, it also involves
muscle for chew, masseter muscles, and it makes hard to chew. The weakness of
facial muscles if hard will makes myasthenia fasiaes has weak smile and facial
expression that looks sad. The weakness of pallatum muscles makes the voice of
patient gone after a long talks. The weakness of neck extensor muscles make the
patient feel pain around neck and shoulder. The weakness of upper extrimity
makes the patient difficult to do work with hand, include writing, but at first it
involves difficulty of open the door and using hand tool. The weakness of lower
extrimity sometimes make the patient difficult to walk, the weakness of m.
gastrocnemius make it hard to start walking, and cant stand with their feet. The
weakness of this muscles will be better after a rest. Theres no sensoric disruption.
Myasthenia Gravis most common on woman, but it also can happen on a
man. It can be involves a thymus tumor, it reported that on 75% case there is
abnormality of thymus, and 10% case occur tumor. Lupus and rheumatoid
arthritis also can be happen.
Myasthenia Gravis patient has been classified by some factor, such as
weakness distribution, range time of di sorder and activity relative of disorder.
Osserman Classification (1958)
MG I Just oculer weakness
MG IIa Common MG with light weakness
MG IIb Common MG with severe weakness
MG III Acute MG
46
MG IV Late-Severe MG
MG V Beginning of atrophy muscle

Modification of Osserman classification system that is used in Virginia University (1976)
I Oculer
II
Common, light weakness, usually include weakness of
oculer muscles
III
Specially include orofaring, involve common light
weakness
IV Common moderate weakness
V Common severe weakness

Classification by Simpson (1978)
Degree I "Active level"
The increase of clinical symptoms fluctuated usually on
5-10 years. Very unstable period
Degree II "Non-Active Level"
The clinical symptoms less fluctuated and unstable on 5-
10 years of beginning
Degree III " Final Level"
Occur on 14-20 years after relative clinical symptoms,
non-fluctuated, and there is permanent weakness,
"Myopati Myasthenic"

The most severe symptoms of Myasthenia Gravis is the weakness of
respiratory muscles that can be happen because
1. There is chronic obstructive lung ilness
2. There is development of pneumonitis, that makes blood oxygenated failure
3. Disfunction of thorax and diaphragm wall

Myasthenic crisis can be happen when the treatment and therapy isnt
functional and it become fast because of infection. The symptoms is difficult to
breath, stop breathing, fast pulse, increase of blood pressure, diaphoresis, unable
to cough, increase of secretion, disphagia, and common weakness.
47
When medicinal treatment of Myasthenia Gravis is too much, such as
giving too much of Anticholinesterase, Cholinergic crisis can be happen. The
symptoms is such as abdomen muscle cramps, diarrhea, nausea, vomitus, too
mush secretion, myosis, sign of Myasthenia crisis, and diapheresis.
Laboratorium Evaluation of Myasthenia Gravis
1. Respond to Anticholinesterase
Most of Myasthenia Gravis patient will show a better respond of the group
of muscles when receive anticholinesterase treatment, from oral or
parentral
Anticholinesterase treatment that is usually used is Edrophonium
(Tensilon) in 10 mg (1,0 cc) doze, and prepare Atropin sulphate 1 mg.
Usually evaluation used doze for test 2 mg (0,2 cc), wait for 60 second,
check hipersensivity and not tolerantion, need Atropin sulphate 0,5 mg.
Other treatment is Neostigmin (Prostigmin) in doze for 0,5 mg until 1,5
mg IV or IM and Atropin sulphate 0,5 mg IM
2. Curare Test
This test is based on relative hipersensivity Myasthenia Gravis patient of
curare so a small fraction of normal doze will make increasing weakness.
Giving 1/10 of normal doze with IV
3. Electrophysiologic Test
Using Electromyograph for decide diagnose and Myasthenia Gravis
patients characteristic is assessment of electromyograph on muscle
respond to continous stimulation and analyzing of single fiber
4. Stapedius Reflex Test
Activity of stapidius muscle on respond to auditory stimulation is reflected
by acoustic impedantion change so a pattern of stapedius muscles
weakness can be demonstrated by Myasthenia Gravis patient
5. Immunologic Test
48
Antibodi Receptor Acetycholin (AchRA) can be found on 80-90%
Myasthenia Gravis patient serum. Reported that theres some of False
Positive test ( amyotrophic lateral sclerosis, thymoma, and polymyositis).
This test is used for decide diagnose.
Antibody of anti-sceletal muscle can be found on 90% Thymoma patient
and 30% on Myasthenia Gravis patient
6. Test for related disorder
On Thymoma patient, its needed to identify thorax picture that is sensitive
to look at anterior of mediastinal, and CT Scan examination with contrast
increase to most of the tumor
6. What the mean of the patient result of test muscle strength motor = 4-4, 4-4?
Manual muscle testing (MMT) is a physical examination of volunteer
muscle which is shown by grade 0-5 (trace-normal). This grade is used to score a
malfunction that connected with muscle contraction, myoneural junction, and
lower motor neurons(LMN). This examination obviously cant be used for the
Upper motor neurons malfunction. Basically, procedure of this test uses make
and break principle.
Evaluation of physical examination is based on two things:
Capability of contracted muscle to hold the pressure
Normal/5: able to hold whole pressure
Good/4: able to hold a half pressure
Fair/3: able to against the gravitation
Poor/2: disable to against the gravitation
Positive or negative contraction of the muscle
Trace/1: a bit of contraction, without any movements
Zero/0: negative for the contraction of the muscle
49
The data of the patient:
4(upper
right)
4(upper left)
4(lower
right)
4(lower left)

From the date above, we can know that this patient has a normal/good for his
physical examination.
MMT 4/Good
When the muscle strength on the opposite side has confirmed the normal, Then
the muscle strength can be compared about the two sides.
7. What is the musculoskeletal system?
Locomotor system of body is roled by muscles, bones, and joint. Main
function locomotor system is movement. Movement played by muscles, joint, and
bone working together, and brain, as the control of movement. Muscle, roled as
active movement tool, while bone roled as passive movement tool.
The component of skeletal muscle is such as Sarcolemma, the cell
membrane of the muscle fiber, Sarcoplasma, as the sitoplasma, Sarcosome, as the
mitochondria, and Myofibrils that is composed by actin (thin filament) and
myosin (thick filament) filaments, that are responsible to contraction and
relaxation of the muscle. Actin and myosin also make the myofibril has dark and
light band. The light band called I band only has actin filament. The dark band has
both actin and myosin filament, are called A band.
Muscle has 2 characteristic, as contractile tissue and excitable tissue. As
contractile tissue, muscle has contractile protein, actin, and myosin. As contractile
tissue, muscle respond when theres a load, the respond is with contraction.
Muscle doing contraction is for surviving. Theres 3 types of contraction:
50
1. Isomethric, contraction that has respond the length of the muscle is not
changing
2. Excentric, contraction that has respond the length of the muscle is
increase
3. Concentric, contraction that has respond the length of the muscle is
decrease
Actin complex composed of troponin and tropomyosin as the gate of the actin.
Subunit of troponin is troponin I that has a strong affinity for actin, another
(troponin T) for tropomyosin, and a third (troponin C) for calcium ions. When
relaxation, actin should be locked. But when theres contraction, active site actin
should be opened. In the resting state, the tropomyosin molecules lie on the top of
the active sites of the actin strands, so that attraction cannot occur between the
actin and myosin filaments to cause contraction. Mechanism of opening troponin
is, when calcium stick at troponin, it will make troponin stay away from active
site actin, so the gate is opened. Theres a condition of troponin C that is ready to
receive calcium, its when troponin I is reducted. When troponin I oxidated, the
connection between troponin C and T very distant so contraction cant be happen.
Antioxidan is needed to keep muscle doing contraction well.
Muscle as excitable tissue means, all the movement of the muscle is
caused by stimulation of nerve. The relationship of muscle and nerve is connected
by neuromuscular junction (NMJ). If theres more NMJ active, contraction of
muscle will be bigger. To connect it, neurotransmitter is needed. The example of
neurotransmitter is acethylcholin, which is the releasing of acethylcholin will be
catched by receptor in the muscle membrane. If nerve isnt doing their function
normally, neurotransmitter fail to release its product to give stimulus to muscle,
releasing of ion will not happen, and troponin C will be closed.

8. What is the excitable cell?
Excitable cell is when membrane of a cell can generate potential membran
in response to depolarization and transmit an impulse along the membrane
51
(Dorland's Illustrated Medical Dictionary, 32 Edition). As we known the most
excitable cell are muscle or nerve cell although there are other kinds of cells.

NERVE CELL
According to Chudler (2013), Nerve cell or usually call neuron have a similar
characteristic whit other cell such us neurons are surrounded by a cell membrane,
neurons have a nucleus that contains genes, neurons contain cytoplasm,
mitochondria and other organelles, Neurons carry out basic cellular processes
such as protein synthesis and energy production.
Neurons are the oldest and longest cells in the body. when other cells die they will
replaced, many neurons are never replaced when they die. In fact, when human
become older they will have less neuron than when they are young. On the other
hand, data published in November 1998 show that in one area of the brain, that is
the hippocampus (Chulder 2013).
The special characteristic of the neuron is:
1. Neurons have specialize cell parts called dendrites and axons. Dendrites
bring electrical signals to the cell body and axons take information away
from the cell body.
2. Neurons communicate with each other through an electrochemical process.
3. Neurons contain some specialized structures (for example, synapses) and
chemicals (for example, neurotransmitters).
One way to classify neurons is by the number of extensions that extend from the
neuron's cell body (soma).
Bipolar neurons have two processes extending from the cell body
(examples: retinal cells, olfactory epithelium cells).
Pseudounipolar cells (example: dorsal root ganglion cells). Actually,
these cells have 2 axons rather than an axon and dendrite. One axon
52
extends centrally toward the spinal cord, the other axon extends toward the
skin or muscle.
Multipolar neurons have many processes that extend from the cell body.
However, each neuron has only one axon (examples: spinal motor
neurons, pyramidal neurons, Purkinje cells).
Neurons can also be classified by the direction that they send information.
Sensory (or afferent) neurons: send information from sensory receptors
(e.g., in skin, eyes, nose, tongue, ears)TOWARD the central nervous
system.
Motor (or efferent) neurons: send information AWAY from the central
nervous system to muscles or glands.
Interneurons: send information between sensory neurons and motor
neurons. Most interneurons are located in the central nervous system.
MUSCLE CELL
There are three kind of muscle, Smooth muscles are involuntary (i.e., they
cannot be controlled voluntarily). Their cells have a variable length but are in the
order of 0.1 mm. Smooth muscles exist, for example, in the digestive tract, in the
wall of the trachea, uterus, and bladder. The contraction of smooth muscle is
controlled from the brain through the autonomic nervous system.
Striated muscles, are also called skeletal muscles because of their
anatomical location, are formed from a large number of muscle fibers, that range
in length from 1 to 40 mm and in diameter from 0.01 to 0.1 mm. Each fiber forms
a (muscle) cell and is distinguished by the presence of alternating dark and light
bands. This is the origin of the description "striated," as an alternate terminology
of skeletal muscle (see Figure 2.6).
The striated muscle fiber corresponds to an (unmyelinated) nerve fiber but
is distinguished electrophysiologically from nerve by the presence of a periodic
transverse tubular system (TTS), a complex structure that, in effect, continues the
surface membrane into the interior of the muscle. Propagation of the impulse over
the surface membrane continues radially into the fiber via the TTS, and forms the
53
trigger of myofibrillar contraction. The presence of the TTS affects conduction of
the muscle fiber so that it differs (although only slightly) from propagation on an
(unmyelinated) nerve fiber. Striated muscles are connected to the bones via
tendons. Such muscles are voluntary and form an essential part of the organ of
support and motion.
Cardiac muscle is also striated, but differs in other ways from skeletal
muscle: Not only is it involuntary, but also when excited, it generates a much
longer electric impulse than does skeletal muscle, lasting about 300 ms.
Correspondingly, the mechanical contraction also lasts longer. Furthermore,
cardiac muscle has a special property: The electric activity of one muscle cell
spreads to all other surrounding muscle cells, owing to an elaborate system
of intercellular junctions.
Unlike cardiac muscle, skeletal muscle has no intrinsic spontaneous
activity because it lacks the ion channels responsible for spontaneous membrane
depolarization. Therefore, the stimulus for physiological skeletal muscle activity
is always derived from a nerve impulse. The great majority of skeletal muscle
fibres receive their nerve inputs at single central swellings of the fibres known as
motor endplates (Hopkins, 2006).
THE SYNAPSE
The movement of a signal through the neuron and its axon is all about ions. An
ion is a charged particle, such as Na+, the sodium ion. It has a positive charge,
because it is missing one electron. Other ions, of course, are negatively charged.
Cells have membranes that are made of lipid molecules (fats), and they prevent
most things from entering or leaving the cell. But all over a cell membrane are
proteins that stick out on both sides of the cell membrane. Some of these are ion
channels.
Accoding to Boeree (2004), Some channels are called gates. They can, depending
on their environment, open or close. For some, it's a matter of what chemicals
attach themselves to a part of the gate. For others, it's a change in the positive-
negative balance that causes them to open or close. In the neuron, there are many
54
such gates, including sodium gates and potassium gates. Both of these respond to
positive-negative balance changes.
The events already described occur when a muscle fiber is excited to fire
an action potential. An action potential is triggered in a muscle fiber when it is
depolarized due to excitation at its synapse, the neuromuscular junction. Each
muscle fiber has one neuromuscular junction, receiving input from just
one somatic efferent neuron. An action potential in a somatic efferent neuron
causes it to release the neurotransmitter acetylcholine (ACh). ACh binds
to nicotinic receptors in a specialized region of the muscle fiber known as
themotor endplate. ACh binding allows Na
+
ions to enter the cell, causing a
depolarizing excitatory postsynaptic potential (EPSP) that is above threshold and
triggers an action potential.
The neuromuscular junction differs from typical synapses in the CNS in one
critical way: the EPSP is always well above threshold. This means that under
normal circumstances, an action potential in a somatic efferent
neuron always elicits an action potential in the muscle fiber (Washington Course,
ND).

2.2 LEARNING ISSUES 2
1. What is NMJ (Neuromuscular Junction) ?
2. What characteristic features of LMN (Lower Motor Neuron)? Are there is
a relationship between the LMN and NMJ?
3. How the treatment of myasthenia gravis? (pharmacology and physiology)
4. How the management myasthenia gravis?

2.3 ANALYSIS OF FIRST LEARNING ISSUE
An Old man (Mr.A), 50 years old come to emergency room with his
son. His body He was taken to the emergency room by his son. His son lift
his body with both hands at chest (Mr. A unable to walk). With doing
anamnesis, we got some clue for diagnose patient, which are: his suffered
weakness in upper and lower limbs (both side), feeling since 1 week ago. He
55
cant see clearly at afternoon, hard to swallow food if eating a lot. If he
speaking a lot, his sound is lost. The weakness sometime gone after breaking.
From this anamnesis, we can know that Mr.A have lesion in cranial nerve,
include LMN.
After we doing anamnesis, we will got some clue by physical examination.
We got something wrong with this man, that is: eyelids (right and left side) is
ptosis. and for the other physical examination parts, is normal. Response for
pathological reflex is normal too. But for neurological reflex, shows APR+1,
BPR+1, TPR+1, and PTR+1 (normal, but weakness). And from the result of
physical examination, we can know that Mr.A is not get lesion in UMN,
because he is havent pathological reflex. After we got some clue from
anamnesis and physical examination, we suspicious that Mr.A is suffering
Myasthenia Gravis. So, we doing supporting examination, Tensilon test. From
the tensilon test, shows that the result is positive.
From the tensilon test result, we more and more suspicious that Mr.A
suffering Myasthenia Gravis. But, we must think a lot and diagnose again for
the certainty. So, we do compare patient data between Myasthenia Gravis and
other weakness in limb disorder. Which are: Subdural Hematome (SDH),
ALS (Amyothropic Lateral Scleriosis), and Guillain-Barr Syndrome. And
from comparing data, the sign and symptoms of patient is near on Myasthenia
Gravis. Hes not SDH, because Hes not difficulty with balance or walking,
not Headache and nausea. and hes not Guillain-Barr Syndrome because hes
not suffering infections/particularly respiratory infections, heart rhythm
disorders and bowel obstruction. And for ALS, hes not twitching
(fasciculation) and cramping of muscles. From this all possible, We must
checking again.
56
CHAPTER THREE
THIRD TUTORIAL
ANALYTICAL REVIEW

3.1 ANSWERS
1. What is NMJ (Neuromuscular Junction) ?
Based on Neuromuscular Junction Disorder book by Freeman, et
al. (2004), Neuromuscular Disorder is the disorders which hinder the
production, release, or uptake of acetylcholine. A low safety factor causes
the amplitude of the end plate potentials to fall below the threshold needed
to generate a muscle fiber action potential. This occurs due to an alteration
of quantal response or content. Myasthenia gravis is a disorder resulting in
a decreased quantal response due to loss of acetylcholine receptors. This
leads to reduced miniature end plate potential amplitudes, but their
frequency remains normal. Lambert-Eaton Syndrome (Myasthenic
Syndrome) is a disorder resulting in decreased quantal content leaving
normal miniature end plate potential amplitudes but with decreased
frequency.
Neuromusclar Junction Disorders
Myasthenia Gravis Lambert-Eaton
Syndrome (LEMS)
Botulism
Locatio
n
Postsynaptic Presynaptic Presynaptic
Etiolog
y
A disorder of
neuromuscular
transmission
due to an
autoimmune
response
against ACh
receptors on the
postsynaptic
membrane
Associated with
thymic disorder
or thymic
tumor
A disorder
of
neuromuscu
lar
transmission
due to an
autoimmune
response
against the
active sites
on the
presynaptic
membrane
This
decreases
A disorder of
neuromuscular
transmission
caused by
Clostridium
Botulinum
toxins blocking
exocytosis of
ACh from the
nerve terminal
Associated with
ingestion of
contaminated
raw meat, fish,
canned
57
Ca
++
entry
into the cell,
causing a
decreased
released of
ACh into
the synaptic
cleft
Associated
with small
cell (oat
cell)
carcinoma
of the lung
vegetables,
honey
Onset Female > Male Male < Female
(>40 yrs)
Begins 27 days after
ingestion
Clinical
Present
ation
Proximal
fatigue and
weakness
Exacerbated
with exercise,
heat, or time of
day (evening)
Normal MSR
Facial or bulbar
symptoms:
o Ptosis
o Diplopi
a
o Dyspha
gia
o Dysarth
ria
Improved with
rest
Edrophonium
(Tensilon) Test:
2 mg dose
followed by a 8
mg dose,
improvement
begins in 1
minute
Proximal
fatigue and
weakness
Mainly
affects the
lower limbs
(quadriceps)
Abnormal
MSR
Exacerbated
with rest
Improved
with
exercise
Viselike
grip
Rarely
involves the
neck, facial,
or bulbar
muscles
Bulbar
symptoms are
noted first:
o Ptosis
o Dysphag
ia,
o Dysarthr
ia
GI Symptoms:
diarrhea, N/V
Widespread
paralysis or
flaccidity
Abnormal MSR
Respiratory and
cardiac
dysfunction
Labs Muscle Biopsy:
Simplification of the
postsynaptic membrane
Muscle Biopsy:
Overdevelopment
of neuromuscular
Stool: Toxins noted.
Blood: toxins noted
58
with loss of junctional
folds and receptors.
Blood: Anti ACh
receptor antibodies
junction. Decreased
active zones are
noted
EDX
Finding
s
NCS
SNAP: Normal
CMAP: Normal
EMG
Unstable
MUAP, dropoff
occurs with sustained
contraction
See special studies
NCS
SNAP:
Normal
CMAP: Low
amplitude
EMG
Unsta ble
MUAP, dropoff
occurs with
sustained
contraction
See special studies
NCS
SNAP: Normal
CMAP: Abnormal
amplitude
EMG
Unstable MUAP
See special studies
Treatm
ent
Thymectomy
Anticholinester
ase drugs:
Mestinon 30
mg q 46 hours
Corticosteroids
Immunosuppres
sive agents
Plasmapheresis
One-third
improve
spontaneously
Treat
malignancy
Corticostero
ids
Immunosup
pressive
agents
Plasmapher
esis
Guanidine
increases
ACh quanta
Side effects:
GI, bone
marrow
suppression,
renal
tubular
necrosis
Treat with
trivalent ABE
antitoxin
Recovery
occurs from
collateral spro
Table 1. The difference between 3 type of Neuromuscular Junction Disorders
Source: http://www.ncbi.nlm.nih.gov/books/NBK27244/
Repetitive Nerve Stimulation (Rns)
These are studies in which a repeated supramaximal stimulation of a motor nerve
is performed. A run of CMAPs are recorded for pathologic amplitude changes.
Muscles should be evaluated in a proximal progression if an abnormality is
suspected, but not demonstrated. Proper setup is essential to obtain the appropriate
responses.
59

Picture 5. Example of Repetitive Nerve Stimulation: Normal Response.
Set-up
Immobilize the electrode
Immobilize the limb
Stimulate at a supramaximal level
Control limb temperature (~30 C)
Minimize electrode gel
Stop anticholinesterase inhibitors
Abnormality:
A greater than 10% decrease in amplitude from the first to fifth waveform is
significant for pathology.
Low Rate Repetitive Stimulation (LRRS)
Description: This repetitive stimulation test is performed at a rate of 23 Hz. Each
stimulus causes the endplate potential (EPP) amplitude to drop. If the safety factor
is decreased the potential will fall below the threshold necessary for activation.
This results in a decrease of the MUAP amplitude

60

Picture 6. Example of LRRS Decremental Response.

LRRS Amplitude Changes

Table 2. LRRS Amplitude Changes.

Post Activation Facilitation (PAF)
After a decrement is noted with LRRS, a 10-second isometric contraction or
tetany producing stimulation (50 Hz) should be performed. PAF demonstrates a
repair in the CMAP's amplitude with an immediate follow-up LRRS because of an
improvement in neuromuscular transmission.
Post Activation Exhaustion (PAE)
This response is seen as a CMAP amplitude decreases. It occurs with a LRRS
performed every minute for 5 minutes after an initial 3-second isometric
contraction. The greatest dropoff is between 24 minutes. This test should be used
if a decrement does not present with the initial LRRS, but a diagnosis of a
neuromuscular junction disorder is suspected.

Disorder Amplitude Change
Myasthenia Gravis Greater than 10% decrement
Eaton-Lambert Syndrome Greater than 10% decrement
Botulism Greater then 10% decrement
61

Picture 7. Postactivation Facilitation and Postactivation Exhaustion. Repetitive nerve
stimulation studies in a normal subject (N) and patients with myasthenia gravis (MG) and
Lambert-Eaton Myasthenic Syndrome (LEMS). The results illustrate facilitation and
postactivation depression (exhaustion)
Source: http://www.ncbi.nlm.nih.gov/books/NBK27244/figure/A8105/?report=objectonly

High Rate Repetitive Stimulation (HRRS)
Description: This repetitive stimulation test is performed at a rate of 1050 Hz. It
causes an accumulation of calcium in the cell, which assists ACh release and
repairs the waveforms. HRRS is uncomfortable and a maximal isometric
contraction can serve as a substitute.

62

Picture 8. High Rate Repetitive Stimulation. I: Increment with 50 Hz stimulation.
II: Increment with voluntary contraction (50Hz simulation/train of 50,
Femoral/Rectus Femoris, 500% facilitation.

Table 3. HRRS Amplitude Changes
Source: http://www.ncbi.nlm.nih.gov/books/NBK27244/table/A8108/?report=objectonly
Pseudofacilitation
Description: This is a normal reaction and demonstrates a progressive increase in
CMAP amplitude with HRRS or voluntary muscle contraction. It represents a
decrease in temporal dispersion and increased synchronicity of muscle fiber
contraction. The waveforms produced maintain a constant area under the curve
though the amplitude appears increased.

Picture 9. Pseudofacilitation. Repetitive Nerve Stimulation Study in a
Normal Subject
Disorder Amplitude Change
Myasthenia Gravis Decrement demonstrated
Lambert-Eaton Syndrome 200%300% Increment
Botulism Mild increment
63
Single Fiber EMG (SFEMG)
This is a study that monitors the parameters of single muscle fiber action
potentials. It is useful if repetitive stimulation of at least three muscles is normal
and an abnormal diagnosis is still suspected. Abnormalities can be associated with
neuromuscular junction disorders, motor neuron disorders, and peripheral
neuropathies.
Parameters
Fiber Density (FD)
Description: This represents the number of single fibers belonging to the
same motor unit within the recording radius of the electrode. The fiber
density is determined by dividing the number of single muscle fibers
action potentials at 20 sites by 20. A fiber density of 1.5 is normal. Higher
than this represents a denervation and reinnervation process.
Jitter
Description: During voluntary contraction a small variation exists between
the inter-potential discharges of two muscle fibers belonging to the same
motor unit. This variation is normally 1060 . It is typically considered
abnormal if higher than this amount.
Blocking
Description: This is an abnormality that occurs when a single muscle fiber
action potential fails to appear. It occurs if the jitter becomes greater than
100 . It typically resolves in approximately 13 months, after
reinnervation is completed.

64
Picture 10. Single Fiber EMG Recordings. Top: Superimposed view.
Bottom: Rastered view. A: Normal. B: Increased Jitter. C: Increased
Jitter with blocking.

Picture 11. Increased Fiber Density. The dots represent single muscle
fibers of one motor unit with the recording radius. A. Normal muscle
(Action Potentials from 12 fibers recorded) B. Reinnervation (Action
Potentials from many fibers recorded)

2. What characteristic features of LMN (Lower Motor Neuron)? Are there is
a relationship between the LMN and NMJ?
Lower Motor Neuron or usually call LMN are neurons which innervate
skeletal muscle directly. The cell bodies of these neurons are located within
the ventral (anterior) horns of the spinal cord and within brain stem motor
nuclei or motor neuron. According to Stephen (2010), there are two types of
lower motor neurons, first type is alpha motor neurons (skeletomotor neurons).
65
Alpha motor neurons innervate regular skeletal muscle fibers, i.e., all the
muscle fibers other than those within the muscle spindles. And the second type
gama motor neuron(fusimotor neuron), smaller than alpha. The gamma motor
neurons, not visible among the larger alpha motor neurons in the ventral horn
seen here) innervate intrafusal muscle fibers in the muscle spindle. Gamma
activation adjusts (increases) the sensitivity of the muscle spindle to muscle
stretch. Abnormal gamma activity can thus cause increased muscle tone
(hypertonia, increased resistance of a limb to passive manipulation).
Numerous descending pathways regulate the gamma motor neurons. Typical
signs of lower motor neuron disease are hypotonicity/flaccidity, atrophy and
fasciculations
The corticospinal fibers terminate in the ventral horn on lower motor
neurons, either directly or via local interneurons. Of course, these motor
neurons receive input from several other sources. In fact, their dendrites and
cell bodies are literally outlined by synaptic buttons. The axons of these motor
neurons leave the cord via the ventral roots. The axons of the large ventral
horn neurons (alpha motor neurons) terminate as motor endplates on skeletal
muscle. Alpha motor neurons are also designated as "final common path
neurons" (Suzanne, 2007). Glutamate released from the upper motor neurons
triggers depolarization in the lower motor neurons in the ventral horn, which
in turn causes an action potential to propagate the length of the axon to the
neuromuscular junction where acetylcholine is released to carry the signal
across the synaptic cleft to the postsynaptic receptors of the muscle cell
membrane, signaling the muscle to contract.
Clinical characteristics of LMN lesions are loss of voluntary control,
loss or decreased muscle tone (since tone is in part dependent on the
monosynaptic reflex arc that links the muscle spindles to the lower motor
neurons), muscle flaccid paralysis (loss of movement) or paresis (weakness)
of the affected muscles, muscular atrophy (due to denervation and disuse),
loss or decreased reflexes (areflexia, due to interruption of the motor limb of
the sensory motor reflex arcs), and may have sensory disturbances. The
66
muscles involved may also exhibit fibrillations and fasciculations, which are
spontaneous twitches characteristic of single denervated muscle fibers or
motor units, respectively. These phenomena arise from changes in the
excitability of denervated muscle fibers in the case of fibrillation, and from
abnormal activity of injured motor neurons in the case of fasciculations.
Neuromuscular junction
A muscle fiber is activated via a nerve impulse generated by an
anterior horn cell. The impulse is conducted along the nerve fiber via saltatory
conduction; that is an action potential is generated at one node of Ranvier and
then jumps to the next node of Ranvier where another action potential is
generated. Once the impulse reaches the neuromuscular junction, voltage
sensitive Ca
2+
channels are opened which allow for the influx of Ca
2+
into the
nerve terminal. Ca
2+
entry into the nerve terminal initiates the fusion of
acetylcholine containing vesicles with the presynaptic membrane and the
subsequent release of acetylcholine into the synaptic cleft. Acetylcholine
binds to post-synaptic acetylcholine receptors on the muscle membrane. This
induces an end plate potential which subsequently results in the generation of
an action potential in the muscle fiber membrane. The end result of this
reaction is muscle fiber contraction. The diagnosis of a specific lower motor
neuron syndrome can be accomplished by a combination of the following
investigations:
1. History and clinical examination

2. Histological examination of muscle or nerve biopsy specimens

3. Electromyographic (EMG) examination

4. Biochemical studies

5. Genetic studies
67
And based on the data, we got Myasthenia Gravis as the most related
disease. This disease is characterized by abnormal fatigue with exercise. This
disease has a predilection for ocular, facial, masticator and proximal upper
extremity muscles. Typically the patients recover to some degree after
rest. Thus they feel much better in the morning, but become weaker as the day
progresses. When the extraocular eye muscles are affected, diplopia (double
vision) and ptosis (drooping of upper eyelid) are common and bothersome
signs. This is an autoimmune disease with antibodies destroying the
acetylcholine receptors (a postsynaptic defect).
EMG findings:
Normal nerve conduction velocities, CMAP and SNAP amplitudes.
Decremental response on repetitive nerve stimulation.
Needle examination: Relatively normal MUPs.
Biochemistry:
Acetylcholine receptor antibodies are present in blood.
Histology: Usually normal.
3. How the treatment of myasthenia gravis? (pharmacology and physiology)
Based on Yayasan Myasthenia Gravis Indonesia, Myasthenia Gravis is
an autoimun disease.What is Myasthenia Gravis person need? How is the
treatment?They also said that no wellness treatments are exist. But now,
Myasthenia Gravis can be controled with several teraphy that suggested to help
Myasthenia Gravis patient. Here are some medications teraphy and medication
acts.
Medicine
Pyridostigmine is an analogue of neostigmine with one quarter of its
potency. It is similar to neostigmine in that it binds to acetylcholinesterase via a
covalent bond and is lipid insoluble. Pyridostigmine is not used for antagonism
of neuromuscular block owing to its slow onset time (>16 min). The former
drug has fewer muscarinic side effects and is therefore more widely used. At
bedtime and as syrup for children and patients requiring nasogastric feeding,
this medication is also available in 180mg time span. Atropine can be used to
treat muscarinic side effects if occurs with 0.4-0.6 mg orally two or three times
68
daily. Postoperatively or in critically ill patients, intramuscularly injectable
pyridostigmine bromide (the dose in one thirtieth of the oral dose) and
neostigmine methylsulfate (the dose is one fifteenth of the oral dose) can be
used.
Progressive weakness despite increasing amount of anticholinesterases
signals the onset of a cholinergic or myasthenic crisis. Cholinergic crises are
associated with muscarinic effects, such as abdominal cramps, nausea,
vomiting, diarrhea, miosis, lacrimation, increase in bronchial secretions,
diaphoresis, and bradycardia. In a myasthenic crisis, the muscarinic effects are
not conspicuous, and 2mg edrophonium given intravenously improves rather
than worsens the weakness. In practice, however, the two types of crises often
are difficult to distinguish, and overmedication of a myasthenic crisis can
convert it into a cholinergic crisis.
Therefore, drug withdrawal, tracheal intubation or tracheostomy,
support with respirator, and intravenous feeding are best treated for patients
who have increasing difficulty with respiration, feeding, or handling of
secretions and who are not responding to relatively high doses of
anticholinesterases. After a few days refractoriness to drug therapy usually
disappears.
Other forms of therapy must be employed in patients whose generalized
disease does not respond adequately to modest doses of anticholinesterases.
Alternate-day prednisone treatment induces remission or significantly
improves the disease in more than half the patients. The treatment is relatively
safe if the usual precautions for corticosteroid therapy are allowed. With an
average dose of 70mg on alternate days, the average time for significant
improvement is 5 months. The dose must be lowered gradually over several
months to establish the minimum maintenance dose after the improvement
reaches a plateau.
Azathioprine in doses of 2 to 3 mg/kg/day also induces remissions or
provides measurable improvement in more than 50% of treated patients. The
earliest time for improvement is 3 months, and responses are often delayed for
12 months or longer. Surveillance to detect side effects (pancytopenia,
69
leucopenia, serious infection,a nd hepatocellular injury) must be maintained
during therapy. Azathioprine as an adjunct to alternate-day prednisone reduces
the dose of prednisone and is associated with fewer treatment failures, longer
remissions, and fewer side effects than either drug alone, but the full beneficial
effects appear only after 1 or 2 years of combined therapy. Cyclosporine or
mycophenolate mofetil can be used in patients who are refractory to prednisone
and azathioprine.
Group Name Note
Anticholinesterases
Showing nicotinic effect on skeleton
because acethylcoline buried at
connecting neuromusculuar. This may
cause musculoskeletal at inflame
condition continuous so there are tremor,
convulsions.
Prostigmine(Neostigmine) neostigmine bromide (15-mg tablets)
acts for 2 to 3 hours
It has direct effect on musculoskeletal.
It is the most effective for human
digestive system. Its decreasing
intsetinum peristaltic, gastric
contraction, and gastric acid secretion.
So its making better for digestive
problem on myasthenia gravis.
Pyridostigmine
(Mestinon)
Pyridostigmine bromide (Mestinon) (60-
mg tablets) acts for 3 to 4 hours.
Pyridostigmine bromide is given 4 hours
in the daytime in doses of one-half to
four tablets.
It has direct effect on musculoskeletal
like prostigmin. Pyridostigmine
(Mestinon) enhance communication
between nerves and muscles. These
70
medications don't cure the underlying
condition, but they may improve muscle
contraction and muscle strength.
Possible side effects may include
gastrointestinal upset, nausea, and
excessive salivation and sweating.
Pyridostigmine is an analogue of
neostigmine with one quarter of its
potency. It is similar to neostigmine in
that it binds to acetylcholinesterase via a
covalent bond and is lipid insoluble.
Pyridostigmine is not used for
antagonism of neuromuscular block
owing to its slow onset time (>16 min).
Fisostigmin (Eserine) This medicine give a good effect for eyes.
It is a natural alkaloid derived from the
Calabar bean. It has a carbamate but no
quaternary ammonium group and crosses
the bloodbrain barrier. It is used to
antagonize the central anticholinergic
toxicity caused by anticholinergic drug
overdose. Physostigmine is metabolized
by plasma esterases; elimination does not
depend on renal excretion, unlike the
other anticholinesterases.
Immunosuppressants Azathriopine It used to pressed abnormal reaction from
immune in myasthenia gravis.
Corticosteroids Prednisone It has permisive effect, is corticosteroid is
needed to have effect other hormon.
Corticosteroid works by affecting protein
synthesis speed with pasive difuse.
Table 4. Type of drugs for Myesthenia gravis
71

Other Therapy
1. Plasmapheresis. This procedure uses a filtering process similar to
dialysis. Your blood is routed through a machine that removes the
antibodies that block transmission of signals from your nerve endings to
your muscles' receptor sites. However, the beneficial effects usually last
only a few weeks.After repeated treatments, it may be difficult for doctors
to gain access to your vein. They may need to implant a long, flexible tube
(catheter) into your chest to conduct the procedure.Other risks associated
with plasmapheresis include a drop in blood pressure, bleeding, heart
rhythm problems or muscle cramps. Some people may also develop an
allergic reaction to the solutions used to replace the plasma.
Plasmapheresis is indicated in severe generalized or fulminating
myasthenia gravis that is refractory to other forms of treatment. Daily
exchanges of 2L of plasma result in objective improvement and lower the
AChR antibody titer in a few days. However, plasmapheresis is expensive
and does not confer greater long-term protection than immunosuppressants
alone.

2. Intravenous immunoglobulin (IVIg). This therapy provides your body
with normal antibodies, which alters your immune system response.IVIg
has a lower risk of side effects than do plasmapheresis and immune-
suppressing therapy. Intravenous immunoglobulin therapy at dose of
400mg/kg for 5 consecutive days, or 1g/kg on 2 consecutive days, may
improve severe myasthenia gravis within 2 to 3 weeks of the start of
therapy. The mean duration of the response is 9 weeks in patients also
treated with corticosteroids and 5 weeks in those who are not. The benefits
usually last no more than three to six weeks.Side effects, which usually are
mild, may include chills, dizziness, headaches and fluid retention.

72
Surgery
Thymectomy
Thymectomy is other solution for Myasthenia Gravis. Thymus Gland located
behind costa and this is the most immportant part of immune system. Most of 10-
15 % Myasthenia Gravis had Thymus tumor. Doctor will remove this because it
has dangerous effect. Thymectomy often decrease the powerful weakness of
Myasthenia Gravis after several months.
A thymectomy may be performed as an open surgery or as a minimally invasive
surgery. In an open surgery, your surgeon splits the central breast bone (sternum)
to open your chest and remove your thymus gland. Surgeons may perform
minimally invasive surgery to remove the thymus gland, which uses smaller
incisions. Minimally invasive thymectomy may include:
Video-assisted thymectomy. In one form of this surgery, surgeons make a
small incision in your neck and use a long thin camera (video endoscope)
and small instruments to visualize and remove the thymus gland through
your neck. Alternatively, surgeons may make a few small incisions in the
side of your chest. Doctors use a video scope and small instruments to
conduct the procedure and remove the thymus gland through these
incisions.
Robot-assisted thymectomy. In a robot-assisted thymectomy, surgeons
make several small incisions in the side of your chest. Surgeons conduct
the procedure to remove the thymus gland using a robotic system, which
includes a camera arm and mechanical arms.
The benefits of these procedures may include less blood loss, less pain,
lower mortality rates and shorter hospital stays compared with open
surgery.

4. How the management treatment myasthenia gravis?
Myasthenia Gravis (MG) is no longer considered a fatal disease. Most
myasthenics, with the help of either drugs and/or surgery, lead near-normal
lives. However, there is no standard therapy for all myasthenics and there is
still much to learn about MG - how it is diagnosed and how it is treated. As is
73
usual with all things myasthenic, the success of one treatment over another is
really dependant on the patient. What works for one person, may not work at
all for another. And what works for one body part in a patient, may not work
for another body part. A doctor may experiment with various therapies and
prescription levels to achieve relief from myasthenic symptoms for the patient.
There is no one recipe for all situations, so the choice of treatment for
an individual patient requires judgment and experience. Patients and their
doctors are often required to make decisions even when the evidence is
inconclusive. Patients need all the support which doctors, family and friends
can offer. Normally a neurologist is engaged for diagnosis and management of
the disease. It would be helpful if the neurologist has extensive knowledge and
experience in the management of MG (with the incidence of MG in only 1
person for at least every 10,000 people, it is highly likely that a number of
neurologmists have had little experience with this condition).
Current therapies for MG include:
1. Anticholinesterase Therapy - an attempt to strengthen neuromuscular
transmission with the use of drugs such as pyridostigmine bromide
(Mestinon, Mestinon extended-release) and neostigmine (Prostigmin).
2. Immunosuppressant Therapy - Prednisone; azathioprine (Imuran);
cyclophosphamide (Cytoxan); cyclosporine (Sandimmune);
mycophenolate mofetil (CellCept).
3. Plasma Exchange Plasmapheresis
4. Intravenous Immune Globin
5. Thymus & Thymectomy
6. Other Therapies - atropine; pro-banthine; ephedrine
Hopefully, the neurologist will explain the various forms of
treatment to the patient, and suggest the best way forward. There are
advantages and disadvantages to each type of therapy, and the impact of the
therapy could affect one's ability to cope with the condition and such things
as self image. Some factors to consider are:
74
1. the patient's current condition - for example, therapy for somebody in a
myasthenic crisis such as breathing difficulties would be different to that
for droopy eye lids.
2. the patient's reaction to the therapy - some medications may cause
undesirable side effects such as nausea, dizziness and stomach pains and so
could not be tolerated over long periods.
3. the effect of the therapy on the patient's ability to carry on with daily
activities eg plasmapheresis requires hospital visits; thymectomy requires
several weeks rest after surgery which could have an impact on income and
ability to work.
4. cosmetic implications - some therapies not only deal with the myasthenia,
they will have other physical impacts - a thymectomy will leave the body
scarred, some medications may cause bloating, or affect appetite.
Normally, the first treatment step is medication. There is no cure for MG,
and so the drugs used to combat MG merely provide temporary assistance to help
the body function normally. Additionally, the danger of using any drug is that the
body may become tolerant of it over time, and so the drug becomes less effective.
The choice is then to move to a stronger drug or to a different form of therapy,
such as a thymectomy, which offers long term management of the condition. In
under 20% of myasthenics, MG goes into spontaneous remission which lasts
longer than a year. No one knows why MG fluctuates or why natural remissions
occur.
What is Anticholinesterase Therapy?
Anticholinesterase Therapy (Mestinon, Prostigmin, Mytelase) is one of
current therapies for Myasthenia Gravis. Anticholinesterase drugs include
pyridostigmine (brand-name "Mestinon"), neostigmine (brand-name
"Prostigmin") and ambenonium chloride (brand-name "Mytelase")
The functioning of the muscle in the section What Causes Myasthenia
Gravis might recall that for a muscle to contract, the nerve sends a message to the
muscle through a neurotransmitter substance called acetylcholine (ACh). The
75
nerve end releases a large amount of ACh, which travels through the
neuromuscular junction (NMJ) (the gap between the nerve and the muscle) and
binds to a receptor on the muscle membrane. In MG, the attacking antibodies bind
to the muscle's membrane and initiate a series of events that destroy the
membrane and prevent ACh from binding. If ACh cannot bind to the muscle, the
muscle does not receive the message from the nerve and will not contract.
Anticholinesterase drugs boost the body's ACh by blocking the enzyme
which usually breaks down ACh. This allows the build up of ACh to concentrate
at the at the muscle receptor, which is where it is needed to transmit messages
from the nerves to the muscles, and its effect is prolonged.
The most commonly used anticholinesterase is "Mestinon". This comes in
60 milligram (mg) or 10 mg tablets and is released immediately. Mestinon
TimeSpan" is a 180 mg tablet in which 60 milligrams is released immediately and
the remaining 120 milligrams are released over several hours. TimeSpan is
usually prescribed for patients who require medication throughout the night (this
allows for comfortable, uninterrupted sleep and reasonable strength in the
morning). Timespan's uneven release provides less predictable results than with
ordinary Mestinon and is usually not recommended for day time use, but some
myasthenics prefer taking it. Liquid "Mestinon" syrup is for children and for
adults who have trouble swallowing pills.
There are no fixed dose or time schedules for anticholinesterases as muscle
involvement and severity vary so much among myasthenics. An increase in
muscle strength is usually noticeable within 20 to 40 minutes after taking the
medication, and they produce their maximal effects about one to two hours after
ingestion (although muscle strength rarely returns to normal). The effects start
wearing off after three or four hours. The medication must be taken at regular
intervals so that muscle strength is maintained throughout the day. For those
myasthenics who have trouble chewing or swallowing, it is best to take
medication at a time that will produce optimal strength during meals.
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The need for anticholinesterases varies from day-to-day, and during the
same day in response to infection, menstruation, emotional stress, and hot
weather. Different muscles respond differently; with any dose, certain muscles get
stronger, others do not change, and still others become weaker. A "brittle
myasthenic" may be adequately medicated during one 3 to 4 hour period and
over-dosed with the same dose in the next 3 to 4 hour period. Medication dosage
may require frequent adjustment according to one's response to the drug and to
hourly/daily activity level.
Anticholinesterases are the gentlest of the drugs available to treat MG.
However, some people do experience side effects, including: stomach cramps,
queasiness and nausea, gut hyperactivity and diarrhea, vomiting, increased
perspiration, increased salivation, muscle twitching and muscle cramps,
palpitations and also increased urinary frequency. The muscle controlling the
pupil of the eye is also affected, and there may be difficulty in focusing. There are
Acetylcholine receptors in the heart and so Mestinon may cause a very slow heart
beat, which can, in turn, cause dizziness.
The presence of these symptoms may be a sign of taking too much
medication, in which case the medication should be taken at longer intervals or in
lesser amount. Other symptoms of overdose could include the worsening of
generalized weakness, swallowing difficulties and respiratory failure (in which
case a doctor should be contacted immediately). The myasthenic who is
prescribed large daily dosage of anticholinesterase and suffering severe side
effects or a worsening of their condition should question their doctor about their
therapy.
To lessen the side effects, the drug can be taken with bland foods such as
crackers and milk. If the unwanted side effects are particularly intense, they can
be prevented or reduced by simultaneously taking other drugs - either
anticholinergics (atropine) and hydrozyzine (Vistaril). It is also important to
emphasise that all the unwanted effects of Pyridostigmine are short-lasting, and
that it does not cause any permanent or long-term problems.
77
What is Immunosuppressant Therapy?
As the name suggests, immunosuppressant drug therapy attempts to
suppress the body's immune system, although no one really knows how they work
in MG. They are commonly called steroids (those used for MG are properly
known as corticosteriods, not the anabolic steroids used by sports people).
Steroids and other immunosuppressive drugs are often very effective in producing
remission of symptoms.
The immune system produces antibodies to fights against foreign bacteria
and viruses. In illnesses such as MG, the antibodies become overactive and start
attacking the body's tissues. Immunosuppressive drugs suppresses the production
of antibodies to stabilize an overactive immune system. In MG, it is the ACh
receptor antibodies that are suppressed, therefore allowing the receptors to
regenerate and function in neuromuscular transmission. Hence, there is a return of
muscle strength.
In the use of immunosuppressants, the best responses occur in patients
with recent onset of symptoms, but patients with chronic disease may also
respond. The severity of disease does not predict the ultimate improvement. In
decreasing order of their frequency of use in MG, they are prednisone,
azathioprine ("Imuran"), cyclophosphamide ("Cytoxan") and cyclosporine
("Sandimmune"). Except for prednisone, none of these drugs is endorsed by its
manufacturer specifically for treatment of MG.
Unfortunately these drugs can make it harder for the body to fight
infection, and can also have serious effects, especially after prolonged use.
Prednisone
Prednisone is a synthetic drug which resembles natural hormones
produced by the cortex of human adrenal glands, and is used to treat many
illnesses. The body depends upon these hormones, called corticosteroids or
"steroids," during stress. Unique to MG is the possibility of increasing weakness
78
during the first two weeks of prednisone therapy, which requires close medical
supervision when first administered. Some doctors try to avoid this initial
weakening by starting with a low dose of prednisone and gradually working up to
a recommended amount of 50 to 60 milligrams every day for several months.
Onset of improvement in muscle strength usually occurs within 2 weeks but may
take as long as 2 months. Marked improvement or complete relief of symptoms
occurs in more than 75% of patients treated with prednisone.
When prednisone is taken in doses higher than 20 milligrams daily for
longer than a week, the body's natural production of adrenal hormones begins to
decrease. This is called "adrenal suppression," and is an undesirable but inevitable
effect of taking high doses of a synthetic steroid. Once this occurs, prednisone
cannot be stopped all at once but must be slowly tapered down over several
months to give the adrenal glands a chance to "wake up" and begin producing
natural adrenal hormones again.
Prednisone has a great many potential undesirable effects, usually related
to dose and duration of drug use. In order to lessen the chance of undesirable
effects, a gradual transition to alternate-day therapy is made after about two
months of daily therapy, so that eventually twice the usual dose is given every
other day for several more months. As soon as is feasible (3 to 12 months), the
drug is very slowly tapered over many months to a long-term maintenance dosage
(around 5 to 10 milligrams every other day) sufficient enough to keep myasthenic
symptoms at bay. The choice of prednisone therapy is thus a long-term
commitment lasting several years. 30% of myasthenics on high-dose prednisone
therapy experience a drug-dependent symptom-free remission, and another 50%
obtain marked improvement. However, 25% of patients also experience serious
complications from this drug.
The usage of Prednisons have side effects. The side effects of using
Prednisone (and any other steroid) are risk of developing osteoporosis - this is
probably the most serious long-term effect. It results in thinning and consequently
in weakening of bones and particularly those of the spine and the pelvis. The
79
effect is worse in those who are already at risk of thinning of their bones such as
the elderly or women past the age of childbearing. Exercise, particularly weight-
bearing exercise such as walking and aerobics, is highly recommended (although
some myasthenics may find this difficult). Diet should be high in calcium, protein
and Vitamin D. There are now also available drugs (such as etidronate) which are
being used successfully to treat bone thinning. Increased susceptibility to diabetes
- steroids make the body less capable of dealing with glucose and other sugars,
which affects diabetic patients. It may also induce mild diabetes in patients who
didn't previously have it. Strict dietary control may be necessary to counter this
side effect. Thinning of skin and wasting of muscles - steroids tend to break down
body tissues, and deplete them of protein. This can be counteracted by strenuous
exercises, which again may be difficult for people with MG. Thinning of the skin
leads to it being easily cut or broken, delayed healing of wounds, and also to
increased susceptibility to the affects of sunlight. Increased risk in developing
glaucoma and lens, irritation of stomach lining and gullet causing indigestion,
increased acid in the stomach and possibly ulcers. Sodium and water retention,
causing some puffiness or swelling. Other side effects include mood changes and
insomnia increased appetite and weight gain, decreased resistance to infection,
high blood pressure, increased sweating, especially at night, increased hair
growth, acne on the face, back, and chest thrush (Candida) growth in the mouth.
Patients on prednisone should watch their weight, keep as active as possible, eat a
balanced diet (high in protein, calcium and potassium but low in salt, free sugar
and fat), stay out of crowds in enclosed areas (to avoid people with infections) and
the most important thing is see their doctors regularly.
Azathioprine (brand-name Imuran)
Imuran also suppresses the production of ACh receptor antibodies. It is
used an alternative to prednisone where the patient either cannot tolerate
prednisone or does not respond to it. Others respond better to treatment with both
drugs than to either alone. In some patients, imuran is used as an aid to decreasing
the dosage of prednisone.
80
The beneficial effects of Imuran seem to take months to occur, and often
emerge so slowly and subtly that they are apparent only in retrospect. Because the
response to Imuran is delayed, both prednisone and imuran may be started
simultaneously with the intent of rapidly tapering prednisone when Imuran
becomes effective. If Imuran is working, after about 3 to 12 months (or even
longer in some patients), the myasthenic should notice a gradual improvement in
how they feel. This improvement can be measured clinically by decrease in the
number or severity of symptoms, need for less prednisone or Mestinon, need for
less frequent plasmapheresis treatments and lower AChR antibody titer
Remissions which occur on Imuran are drug-dependent. Imuran is a long
term treatment and a patient may have to stay on this medication indefinitely.
Once improvement begins, it is maintained for as long as the drug is given, but
MG symptoms recur 2 to 3 months after the drug is discontinued or the dose is
reduced below therapeutic levels. As with any immunosuppressive drug, it is
important that the patient take Imuran exactly as prescribed by their doctor.
The undesirable effects of Imuran are less varied than those of prednisone but they
can be very serious:
- Some people have a hypersensitivity or allergy to Imuran and develop fever,
chills, joint and muscle pains, vomiting and dizziness. This usually occurs soon
after commencement of treatment. When this unwanted reaction occurs, treatment
must be stopped and cannot usually be restarted.
- Women who may want to have children should avoid this drug, because it has a
known potential for producing fetal deformities.
- Imuran depresses the formation of new blood cells, just as it depresses antibody
forming cells. It is therefore necessary to have complete blood counts of red and
white cells frequently at the start of treatment, and three to four times a year once
treatment is established. If the count drops significantly, it may be necessary to
stop treatment temporarily.
81
- Liver function may be upset by Imuran. This can be monitored by blood tests.
The effects are reversible on stopping treatment or reducing the dose.
- Susceptibility to infection - as Imuran depresses immunity, it increases
susceptibility to infections. Special care must be taken to avoid contact with
shingles and chicken pox which are much more severe in patients with lowered
immunity.
- There is still much to learn about Imuran, including answers to such worrisome
questions as whether it may increase the risk for cancer many years later. This is
the most controversial, unwanted effect of Imuran. There are reports of an
increased frequency of tumours mainly of the lymph glands in patients with
rheumatoid arthritis taking Imuran. The sort of tumours that occurred in these
patients were those that respond well to treatment once Imuran is stopped.
However, a more recent report in 755 patients taking Imuran for bowel disease
and who were followed for up to 29 years found no increase in the number of
tumours of any sort. There are no comparable figures for MG Patients, but, on the
basis of current knowledge, there is little cause for alarm.
Approximately one-third of patients have mild dose-dependent side effects that
may require dose reductions but do not require stopping treatment.
Imuran is generally tolerated without serious adverse effects. The patient should
check with the doctor immediately if any of the following occur nausea and
vomiting, fever or chills, loss of appetite, upset stomach, skin rash, diarrhea,
cough or shortness of breath, cold sores in the mouth or on the lips, blood in the
urine or stool, unusual bruising, fatigue, missed menstrual period, yellowing of
the eyes and skin, hair loss, muscle or joint pain, darkening of the skin and
fingernails

82
Cyclophosphamide (brand-name Cytoxan)
Cytoxan is considered only for the most severe cases of MG when other
therapies have failed. It is used either intravenously and orally for the treatment of
MG. Whilst experience from the Philippines reports good results with complete
drug-free remission for a year and a half in three out of four MG patients who
were treated with Cytoxan, the side effects are common and can be quite serious.
They include hair loss (almost universal occurrence on the drug) and risk of
bladder hemorrhage and bladder cancer. These effects may be reduced by having
the drug taken intravenously or orally once a week instead of daily. This drug
usually requires the assistance of a rheumatologist or oncologist who is more
familiar with it than are most neurologists.
Cyclosporine (brand-name Sandimmune)
Sandimmue is an immunosuppresion used during organ transplantation,
and could be of benefit to myasthenics in reducing the dose of prednisone. A
preliminary study without prednisone suggested that such a dose of cyclosporine
could produce significant improvement in some MG patients if they could tolerate
the side effects of this medication, the most prominent of which are elevated
blood pressure, headaches, and increased body hair.
Most patients with MG improve 1 to 2 months after starting cyclosporine
and improvement is maintained as long as therapeutic doses are given. Maximum
improvement is achieved 6 months or longer after starting treatment. After
achieving the maximal response, the dose is gradually reduced to the minimum
that maintains improvement.
Adverse effects of this medication include arenal toxicity, hypertension, and many
drugs interfere with Sandimmune metabolism should be avoided or used with
caution.

83

Mycophenolate Mofetil (CellCept)
CellCept is a relatively new immunosuppressive drug that was originally
developed to prevent immune rejection of transplanted organs. Whilst its
treatment of MG is still being investigated, it appears that it may be of assistance
to myasthenics who have found prednisone and other immunosuppressive drugs
ineffective.
In a pilot trial conducted by Dr Donald Sanders (director of the MDA
clinic at Duke University in Durham, N.C.), 8 out of 12 patients on CellCept for
several months gained strength or were able to reduce their need for prednisone.
In another study, 92 patients in Chicago were prescribed the drug for 3 to 45
months. Improvement was seen in 67 of these patients, including 5 who went into
complete remission.
The advantage of CellCept over other immunosuppressant drugs is
reported to have more rapid onset and fewer side effects. The drug has been
reported to cause birth defects in animals, and so women of child bearing age
should avoid getting pregnant whilst on the drug as the effect on unborn babies is
unknown).

Plasma Exchange (plasmapheresis)
Plasmapheresis is a blood purification procedure used to treat several
autoimmune diseases. It involves the exchange of the blood plasma which
contains antibodies and other substances that interfere with the transmission of
nerve impulses and replacement with fresh or artificial plasma. The need for
plasma exchange, and its frequency of use is determined by the response in the
individual patient. The procedure is expensive, time consuming and not totally
risk free, and is not meant for long-term treatment. The reasons why is it used are
:
84
1. To stabilise the condition of patients in myasthenic crisis where the condition is
life threatening.
2. To reduce moderate to severe muscle weakness before thymectomy.
3. Some myasthenics do not respond sufficiently to more traditional forms of
treatments, and so plasmapheresis offers their only relief from near paralysis and
life-threatening respiratory problems.
The course of the exchange normally involves six to ten exchange
treatments over two to ten weeks. To access blood, one tube is inserted into a
large vein (possibly in the crook of the arm), and another placed in the opposite
arm or foot. Around 3 to 4 litres of blood (depending on one's body weight) is
removed from the first tube. The blood passes through a blood separator, which
works in one of two ways - either by spinning the blood at high speed to separate
the cells from the fluid; or by passing the blood through a membrane with pores
so small that only the fluid part of the blood can pass through. The plasma, which
contains the antibodies, is discarded. The red blood cells are returned in artificial
plasma (albumin and saline solution) via the second tube. Anticoagulant is added
to the blood to keep it from clotting, but most of the anti-clotting agent is removed
prior to returning the blood to the patient. Treatment, which can be uncomfortable,
but is not painful, can take 2 to 5 hours. The patient lies down only during
treatment but is able to walk around before and after treatment.
Maximum improvement may be reached as early as after the first exchange or as
late as the fourteenth, but exchanges do not have a cumulative benefit.
The following difficulties may arise during the procedure:
1. Sometimes it is not possible to achieve adequate blood flow from veins in the
arms. Therefore, alternative blood access, which might involve some minor
surgery, will be necessary.
2. Although the latest technology blood cell separators remove only a small
portion of blood from the patient at any one time, the changes in blood volume or
85
the type of replacement fluid utilized my make some patients feel dizzy or
lightheaded. Patients should immediately tell the medical staff if they begin to feel
uncomfortable or if they suffer numbness, tingling associated with the mouth,
eyes, fingers or toes and leg cramps, dizziness and mental confusion (all of which
may indicate a low blood calcium or potassium level). Tiredness usually occurs
after treatment.
3. The anticoagulant used to keep the blood from clotting and certain types of
replacement fluids might cause a patient to notice a sour taste in the mouth,
tingling around the lips, or sharp pains, like pins being stuck in the fingers or toes.
Patients should immediately tell the medical staff if they have any of these
symptoms.
4. The procedure isn't selective about which antibodies it removes, and so it may
result in excessive suppression of the immune system. The body can replenish anti
bodies in time, but day patients may have to take special precautions against
infection.
5. Patients with clotting disorders may not be suitable for this sort of procedure.
Plasmapheresis does not cure MG - it only temporarily reduces the level of
circuiting antibodies that attack the neuromuscular junction. It does not prevent
the production of more antibodies, and usually the patient is given medication to
help combat the condition.

What is Intravenous Immune Globin?
Intravenous immune globin (IVIG) is the opposite of plasmapheresis -
instead of drawing off the offending antibodies, IVIG swamps the body with
pooled gamma globulin antibodies from many donors. The process does not
require special equipment, and the usual dose is small (eg 400 mg per kilogram
per day infused for five successive days). Whilst the mechanism of action remains
unknown, IVIG is thought to have a nonspecific suppressive effect upon the
86
production of antibody by the immune system. It produces rapid improvement to
help patient through a difficult period of myasthenic weakness. In patients who
respond, improvement begins within four or five days, and may be sustained for
weeks to months.
The process is quite expensive, and like plasmapheresis, the treatment is
short term. As with plasmapheresis, its use is really limited to critical patients or
those who are not responding to traditional treatments. It should be noted that
there is a theoretical risk of transmission of blood born infections, although this
has not happened now for some years.
Adverse reaction occur in fewer than 10 percent of patients. Symptoms
include headache, fluid overload, and in rare cases, renal failure. Plenty of fluids
should accompany the treatments to minimize the severe headache which can
occur.
Thymus & Thymectomy
It is accepted that there is a connection between the thymus and
Myasthenia Gravis (MG) but the reason for the connection is not fully understood.
The thymus gland is an organ involved in the development of the immune system.
It is located in the upper chest under the breastbone and is composed of many
small lobes. During the formation of the fetus, the thymus migrates from the neck
into the chest, and in adults it lies beneath the breastbone (sternum). Like tonsils
and adenoids, the thymus is large in infants and gets smaller, to be replaced by fat,
as we get older, making it hardly functional.
87

Picture 12. The location of thymus in human body
The myasthenic adult has thymic abnormalities. The thymus could be
enlarged (as seen by CT scan) or it can contain more cells than normal (as seen
under the microscope) - this is called hyperplasia and is often seen in myasthenics
of many years standing.
Around 10% to 15% of myasthenics have tumours, called thymomas,
which are usually relatively benign, but may become malignant. Thymomas are
normally removed as soon as possible to prevent local spread (although, only
around 30 to 50% of people with thymomas also have MG - and in some, MG
develops after they have their thymectomy!). The risk of development of
thymoma has led to thymectomy as well for MG patients without thymoma. (For
more information on THYMOMAS, go to the Myasthenia Gravis Association UK
website [http://www.mgauk.org/]. There are two excellent papers by Dr Nick
Willcox of the Institute of Molecular Medicine , University of Oxford on The
Mysteries of Thymoma and MG.)
If most of the thymus gland is removed at surgery, myasthenic symptoms
usually lessen and in some individuals go away completely. Although the relation
of the thymus glad to myasthenia gravis is not totally understood, it appears that
88
the thymus gland is linked to the production of acetylcholine receptor antibodies
or other substances that interfere with neuromuscular transmission.
A thymectomy is the removal of the thymus gland by surgery. The goal of
thymectomy as a treatment for MG is to induce remission, or at least
improvement, permitting a reduction in immunosuppressive medication.
Remission is the complete elimination of symptoms without medication.
In 1937, Dr. Alfred Blalock removed the thymus of a myasthenic patient
when the thymus was found to have a tumour. He discovered that the symptoms
of MG in the patient showed improvement. Since that time, thymectomy has been
used as a normal course of treatment for myasthenics around the world, although
the precise reason why it is beneficial is still not known.
In the past, it was thought that thymectomy would not benefit older
patients (over 45 years old), or people who have had the disease for over 5 years.
Yet some older patients and some long-standing MG patients have benefited from
thymectomy, so now the recommendation for this procedure has to be considered
on an individual basis. Although, there is uncertainty about the persistence of
thymic tissue in such patients after the age of 60.
After a thymectomy, remission or marked improvement occurs in more
than half of cases. Those who had thymomas do not usually improve as much as
in young myasthenics without a thymoma. Access to the thymus can be gained
either by transsternal thymectomy - the incision is made length-wise on the chest
and the breastbone (sternum) is split open; or transcervical thymectomy where
access is gained through the neck.
There are advantages and disadvantages to each approach. The splitting of
the sternum is more traumatic than surgery through the neck, and the recovery
time is longer, but it is more common because it more thorough allowing easier
identification and removal of all thymic tissue (some doctors believe that leaving
any remnant of thymic tissue in the body will increase the likelihood of the
procedure failing).
89
About 30% of MG patients without a thymoma who undergo thymectomy
eventually go into complete drug-free remission, and another 50% experience
marked improvement. This improvement usually does not occur immediately after
surgery, but may take up to several months or years to reach its peak effect. You
cannot predict beforehand who will benefit from thymectomy, and even after
benefit occurs there is still a small possibility of subsequent relapse. However,
thymectomy itself rarely worsens the long-term course of MG.
Even invasive thymomas are not always detected with imaging tests and
have been discovered during thymectomy surgery. Such experiences would argue
in favor of eventual thymectomy over immunosuppressive drug therapy in
otherwise healthy young or middle-aged MG patients, once the patient is up to the
surgery. The possibility of an eventually complete symptom-free remission after
thymectomy without the need to take any drugs, compared to a remission
dependent upon the continued treatment with immunosuppressive drugs, is
another significant advantage of thymectomy.
It is normally preferred that children wait until puberty before undertaking
thymectomy because of the established role of the thymus in development of the
immune system.
The process for a thymectomy is as follows:
1. An x-ray is taken of the patient's chest to determine the location of the thymus
and to determine the existence of a thymoma.
2. An anesthetist will consult the patient about the anesthesia plan - how the
patient will be put to sleep, the use of any special drugs that will be needed to help
the patient relax before surgery, and how the patient will generally feel after
surgery. It is important that the patient tell the anesthetist about any allergies or
reactions to any foods or medicines.
3. Food and fluids will be withheld after midnight, or on the day of surgery.
Routine medication for Myasthenia may or may not be given.
90
4. On the morning of surgery, a pre-operative medicine may be given by injection.
This medication can cause relaxation, drowsiness, and dryness of the mouth.
5. After surgery, a one to three hour stay in the recovery room is required. The
patient will then be transferred to Intensive Care Unit for 12 to 24 hours to ensure
that everything is proceeding normally. In this phase of the recovery, fluids and
medication will be given by means of a needle in the vein called an intravenous,
or I.V. They will then be transferred back to their ward. Very occasionally if the
patient has some breathing difficulties he or she may temporarily be connected to
a ventilator so as to ease this problem.
6. Once fluids are tolerated by the mouth, the intravenous fluids will be stopped.
Solid foods will be started slowly, and the patient's medication will once more be
given by mouth. If progress is maintained, the patient will probably be allowed
home in 7 days (although length of stay in the hospital varies for each patient).
7. Most patients complain of some chest soreness and pain after surgery. This can
be lessened with pain medication. At intervals, the patient will be asked to do
coughing and deep breathing exercises to clear the lungs of mucus. This does
cause discomfort which can be lessened by hugging a pillow and supporting the
chest while coughing. Pain medication can be taken prior to any activity or
exercise to alleviate after-surgery discomfort.
8. The recovery period away from work varies. Most patients take 4 - 6 weeks off
work for convalescence. Those with jobs which require heavy lifting or any strain
on the chest may need a longer recovery time so that the chest is more fully healed
before starting work. The patient should discuss this with the surgeon prior to the
surgery in order to plan the recovery time.
After surgery there may be an increase in muscle weakness in some patients.
However, treatment will be adjusted to meet individual needs.
Thymectomy may lessen the severity of the myasthenic symptoms; however, the
degree to which the symptoms are lessened differs in each patient. The
91
mechanism by which thymectomy produces benefits in MG is still uncertain. In
general, ACh antibody levels fall after thymectomy, although there are conflicting
reports. A study published in 1983 study findings found as high as 85% of
patients who underwent thymectomy showed clinical improvement, and the
maximal favorable response generally occurs 2 to 5 years after surgery (but some
people may experience better health 7 to 10 years after surgery). The best
responses to thymectomy are in young people early in the course of their disease,
but improvement can occur even after 30 years of symptoms. Patients with
thymomas do not respond as well to thymectomy as do patients without thymoma.
The worst response rate to the surgery was from those who had surgery when they
were over 60 years of age.
There is no cure for myasthenia gravis, but treatments are available to help
control the symptoms.
In many cases, treatment for myasthenia gravis can significantly improve
muscle weakness and a person with the condition is able to lead a relatively
normal life.
If your symptoms are mild, you may find that getting plenty of rest helps
improve your symptoms without the need for additional treatment.
How is myasthenia gravis diagnosed?
The diagnosis of myasthenia gravis is made after the sudden or gradual
onset of specific symptoms and after diagnostic testing. During the physical
examination, the doctor obtains a complete medical history, and may also ask if
there is a family history of any medical problems.
A primary characteristic of MG results in the response of an affected
person to certain medications. When given an anticholinesterase medication, such
as neostigmine (Prostigmin) or edrophonium (Tensilon), muscle weakness often
dramatically improves for a brief time. This provides strong support for the
diagnosis of MG.
92
Other diagnostic tests that may be performed to help confirm the diagnosis
of myasthenia gravis include:
Blood tests. These tests look for antibodies that may be present in people
with myasthenia gravis: anti-acetylcholine receptor antibodies are present
in the blood of over 85 percent of affected persons; anti-MuSK
antibodies have been found in about 30 to 40 percent of people with MG
who do not have acetylcholine receptor antibodies.
Genetic tests. Diagnostic tests that evaluate for conditions that have a
tendency to run in families.
Electromyogram (EMG). A test that measures the electrical activity of a
muscle or a group of muscles. An EMG can detect abnormal electrical
muscle activity due to diseases and neuromuscular conditions.
Medication
Medications such as pyridostigmine, and less commonlyneostigmine, can
be prescribed for myasthenia gravis. They prevent the breakdown of
acetylcholine, an important chemical that helps the muscles contract (tighten).
These medicines tend to work best in cases of mild myasthenia gravis. They can
improve muscle contractions and strength in the affected muscles. They are often
used as the first line of treatment for myasthenia gravis if the initial symptoms are
not too severe.
However, they can sometimes cause side effects, such as stomach cramps,
muscle twitching, diarrhoea and nausea. Your doctor will be able to prescribe
additional medication for you if you experience these.
Steroid tablets, such as prednisolone, are used if the symptoms of myasthenia
gravis worsen despite using pyridostigmine. Initially, these steroid tablets are
usually given in hospital and the dose is quickly increased to a high level.
At the same time, doctors often prescribe additional medication that
suppresses the immune system, such as azathioprine, methotrexate or
mycophenolate. It is hoped that these tablets will act alongside the steroids to
93
reduce the level of harmful antibodies. Doctors may allow the steroid dose to
be reduced over time, but probably not within the first four to six months.
Regular blood monitoring is required for all people taking these
immunosuppressants. All of these medications have associated side effects that
your doctors will discuss with you.
Some people respond well and are able to stop using all steroids in
remission, while continuing to take secondary immunosuppressant medication.
In time (usually years), it may be possible to stop all immunosuppressant
medication if you remain well.
Thymectomy
In some cases of myasthenia gravis, surgery to remove the thymus gland
(a thymectomy) may be recommended.
Thymectomy can improve the symptoms of people who do not have tumours
(thymomas) on their thymus gland. It is thought that the immune system
rebalances itself after the thymus gland is removed.
However, the improvement may take some time to occur. It is usually seen
within the first year, although in some cases it can take up to three years.
Thymectomies are usually only recommended for people under 60 years of
age, although research is ongoing to establish exactly which non-thymoma
myasthenia gravis patients benefit from this type of surgery.
Plasmapheresis and immunoglobulin therapy
Plasmapheresis or intravenous immunoglobulin therapy may be needed in
very severe cases of myasthenia gravis, where a person has such severe muscle
weakness that it is causing life-threatening breathing or swallowing problems.
These treatments are given in hospital and involve:
plasmapheresis, where your blood is circulated through a machine
that removes the plasma containing the harmful antibodies
intravenous immunoglobulin therapy, where you are injected
with normal antibodies from donated blood that
temporarily change the way your immune system operates
94
Both treatments can produce a rapid improvement in thesymptoms of
myasthenia gravis, but the benefits usually only last a few weeks. They are
therefore not suitable as long-term treatments for myasthenia gravis, and are
usually only used to treat people who are seriously ill.

3.2 ANALYSIS OF SECOND LEARNING ISSUE
Clinical characteristics of LMN lesions are loss of voluntary control, loss
or decreased muscle tone (since tone is in part dependent on the monosynaptic
reflex arc that links the muscle spindles to the lower motor neurons), muscle
flaccid paralysis (loss of movement) or paresis (weakness) of the affected
muscles, muscular atrophy (due to denervation and disuse), loss or decreased
reflexes (areflexia, due to interruption of the motor limb of the sensory motor
reflex arcs), and may have sensory disturbances. This patient is not LMN
lesions, because hes not atrophy and normal the physiological reflex.
Based on Neuromuscular Junction Disorder book by Freeman, et al.
(2004), Neuromuscular Disorder is the disorders which hinder the production,
release, or uptake of acetylcholine. The NMJ which related for this case is
Myasthenia Gravis, Lambert-Eaton Syndrome/ LEMS (Myasthenic Syndrome),
and Botulism. Only myasthenia Gravis, have location in postsynaps. The MG is
more related to this case, from the clinical presentation characteristics. E.g: the
LEMS is improved by exercise, but this patient is improved by rest (match with
characteristics of Myasthenia Gravis) and this patient is not botulism because hes
havent toxin that indicates botulism.
And then, Current therapies for MG include:
Anticholinesterase Therapy - an attempt to strengthen
neuromuscular transmission with the use of drugs such as
pyridostigmine bromide (Mestinon, Mestinon extended-release) and
neostigmine (Prostigmin).
Immunosuppressant Therapy - Prednisone; azathioprine (Imuran);
cyclophosphamide (Cytoxan); cyclosporine (Sandimmune);
mycophenolate mofetil (CellCept).
95
Plasma Exchange Plasmapheresis
Intravenous Immune Globin
Thymus & Thymectomy
Other Therapies - atropine; pro-banthine; ephedrine
The tymus of this patient is normal, so the tymectomy is not required. The
anticholinesterase, immunosuppressant, and intravenous Immune globin is
required for Mr. A (patient).

3.3 SOLUTION
H needs a right repotition and fixation for his right forearm to reduce the
damage. The repotition must be done correctly because if it is not, it will harm the
other structures which are located in the region of forearm, near the bone segment.
Fixation is needed to faster the treatment of bone remodelling. By doing it right it
will minimize his movement so the process will happening with minimum
interruption. Indeed he must be given extra nutrition by consuming high calcium
supplement or milk.

3.4 FINAL HYPOTHESIS
3.4.1
3.4.2

3.5 OBSTACLES
1. The difficulty of finding literature that is evidence based.
2. The confusion of finding histological process and basic pathological of the
sickness.
3. The difficulty to find a time to gather and discuss the scenario
4. There are some situation that the personel of the group is too afraid to
argue others statement.

3.6 FINAL CONCEPT MAPPING
96
(Attatch in the following page)

3.7 ANALYSIS OF FINAL CONCEPT MAPPPING
A patient that categorized as children is taken to the emergency room with
main problems: pain and swelling in the right forearm. That condition deliver the
physician having differential diagnosis that must be supported with a series of
examination. The series of examination contains anamnesis, physical examination,
and supporting examination.
Based on anamnesis we got the identity of the patient: the boy name is H. His
age is 10 years old. He lives in Jl. Benowo, Surabaya, the mechanism of injury:
when H play, H fell down on to right side with his right hand supporting his body
after he was pushed by his friend while he is running. He had not receive any first
aid so he is in an emergency state. His family and his friend did not have a same
sickness at that time. Those reasons eliminates all diagnosis of genetic disease,
respond to allergy, infection, cancer. And the patients nutritional history : he did
not consume any ASI because his mother is a working-lady. His eating frequency
is normal, the content is standart, nothing special so the bone elasticity is
decreased. So his bone is more susceptible to get injured.
From the physical examination: Crepitation (+), Oedem (+), Deformity (+)
shown in the picture, located on the 1/3 distal part of the right forearm. Fork
shapped deformity.
Hematome (-) which is means that internal bleeding is not seen from the
topography, External bleeding (-); his weight is 60 kg, height : 155 cm. he
included to obese, so when he was falling. He fall with high tension. Vital sign
increased. It expressed his pain. There is no open wound but H is screaming and
97
crying when there is a movement around his right forearm. It means that the pain
is increasing there is a movement around his right forearm. He experienced
tingling sensation and thickness in dorsolateral side of his hand.
From the radiology imaging (X-ray) : He got fracture in the 1/3 part from
distal Radius. Based on the segment, his case is not a comminuted fracture. Based
on the type of fracture line it is oblique fracture. He sustained displacement and
also shortening of the Radius. The proximal segment move to the posterior and
the distal segment move to the anterior. Some of the structure in anterior and
posterior distal right forearm might injured. The growth plate is not injured
Blood Check show that the hemoglobin rate is in normal range.he experienced
very low blood loss which is mean that his condition can not be categorized as
anemia. The leukocyte is also in normal range, it means that he is not in the
process of infection.
Based on all those informations, group can decide which diagnosis can be
eliminated : osteomyelitis, cancer, infection, open fracture, allergy and
dislocation. Group suspect that there is fracture in his forearm. Between :
Chaffeur, Smith, Galleazi, and Colles, the characteristics that the patient have
leaning to the case Colles Fracture.
The fracture itself is not the end. The fracture can cause other structure injury.
In this case group suspect that the superficial branch of radial nerve and cutaneous
antebrachii lateral nerve is injured because of the tingling sensation and thick
sensation in the dorso lateral part of the hand. The segment of the fracture might
also injured the tendo-insertion of flexor pollicis longus muscle and radial artery.

3.8 GROUP OPINION
H is suffering colles fracture, close fracture in 1/3 distal part of the forearm,
the type of the fracture is incomminuted, it is segmented into two segments.
Displacement and shortening is occur. The distal part move to proximal anterior
and the proximal part move to the distal posterior. Those segment harm other
structure that is located around the fracture : Cutaneus Lateral Antebrachii Nerve,
Superficial Branch of Radial Nerve, Anterior Interossea Antebrachii Artery,
Radial Artery, and Tendo insertion of Flexor Pollicis Longus Muscle.
98

3.9 CONCLUSION
H is suffering colles fracture, close fracture in 1/3 distal part of the forearm,
the type of the fracture is incomminuted, it is segmented into two segment.
Displacement and shortening is occur. The distal part move to proximal anterior
and the proximal part move to the distal posterior. Those segment harm other
structure that is located around the fracture, in this case the Cutaneus Lateral
Antebrachii Nerve, Superficial Branch of Radial Nerve, Anterior Interossea
Antebrachii Artery, Radial Artery, and Tendo insertion of Flexor Pollicis Longus
Muscle.
So, H needs further treatment a right repotition and fixation for his right
forearm to reduce the damage. The repotition must be done correctly because if it
is not, it will harm the other structures which are located in the region of forearm,
near the bone segment. Fixation is needed to faster the treatment of bone
remodelling. By doing it right it will minimize his movement so the process will
happening with minimum interruption. Indeed he must be given extra nutrition by
consuming high calcium supplement or milk. He must be under the doctor control
to maintain his getting his ability to work with his right hand.

99
REFERENCES

Agasthian, et al., 2010. Clinical Outcome of Video-Assisted Thymectomy for
Myasthenia Gravis and Thymoma. Asian Cardiovascular and Thoracic Annals.
18(3) pp.234-239. [online] Available at:
<www.aan.sagepub.com/content/18/3/234> [Accessed June 1, 2014]

Better Health Channel, 2014. Neuromuscular System. [pdf] State of Victoria:
Better Health Channel. Available at:
<http://www.betterhealth.vic.gov.au/bhcv2/bhcarticles.nsf/pages/Neuromuscular_
system?open> [Accessed May 26, 2014]
Boeree, C., 2004. The Action Potential. [online] Available at:
<http://webspace.ship.edu/cgboer/actionpot.html> [Accessed May 27, 2014]
Chulder, E., 2013. Type of Neuron. Neuroscience For Kids. [online] Available at:
<https://faculty.washington.edu/chudler/cells.html> [Accessed May 27, 2014]
Dailymed Team, 2010. Physostigmine Salycilate. [Online] Available at:
<http://dailymed.nlm.nih.gov/dailymed/lookup.cfm?setid=a7d04b2c-fe34-480d-
8f7c-7cbcf447a70b> [Accessed May 28, 2014]

Engel, Andrew G., 2012. Myasthenia Gravis and Myasthenic Disorders. 2nd ed.
[e-book] New York: Oxford University Press. Available at:
<http://www.google.co.id/books?hl=id&lr=&id=zSBpAgAAQBAJ&oi=fnd&pg=
PA130&dq=physostigmine+mechanism+for+myasthenia+gravis&ots=26Fiy67IY
c&sig=MdwhcaDjAFolh0PpTgPFh33gWHg&redir_esc=y#v=onepage&q=physos
tigmine%20mechanism%20for%20myasthenia%20gravis&f=false> [Accesed
May 28, 2014]

Fakultas Kedokteran Wijaya Kusuma., 2012. Cara Pemeriksaan Neurologi.
Available at: <http://elib.fk.uwks.ac.id/arsip/download/5232> [Accessed May 28,
2014]

100
Freeman, et al., 2004. Physical Medicine and Rehabilitation Board Review. USA:
Demos Medical Publishing, Inc. [online] Available at:
<http://www.ncbi.nlm.nih.gov/books/NBK27244/>
Goldberg, C. (n.d.). Neuro Exam Elements of The Neuro Exam.
Goldman, L., and Ausiello, D. ed., 2004. Textbook of Medicine. 22nd ed.
Philadelphia: WB Saunders.

Greenberger, NJ and Hinthorn, DR., 1993. History Taking and Physical
Examination: Essentials and Clinical Correlates. USA: Mostby-Year Book, Inc.
Greenly, L., 2003. An Overview of Normal and Pathological Reflexes. Journal of
Chiropractice Medicine. [online] 2 (4), 165-168. Available at:
<http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2646980/pdf/main.pdf>
[Accessed May 27,

2014]
Gunawan, S., Setiabudy, R., Nafriadi., et al, Departement of Pharmacology and
Terapheutic Fakultas Kedokteran Universitas Indonesia. 2007. Farmakologi dan
Terapi. 5th ed. Jakarta: Balai Penerbit Fakultas Kedokteran Universitas Indonesia.

Guyton, Arthur C., and John E. Hall. 2006. Textbook of Medical Physiology, 11th
ed. Philadelphia: Elsevier Inc.

Hadinoto, Soedomo, etc. 1993. Penyakit Neuromuskuler & Muskuloskeletal.
Semarang: Badan Penerbit Universitas Diponegoro
Higuchi, Osamu, et al. 2011. Autoantibodies to lowdensity lipoprotein receptor
related protein 4 in myasthenia gravis. Annals of neurology. 69(2) pp. 418-422.

Huff JS. 2009. Special neurologic tests and procedures. In: Roberts JR, Hedges
JR, eds. Clinical Procedures in Emergency Medicine. 5th ed. Philadelphia, PA:
Elsevier Saunders.
101
Lee MR., 2004. The miracle of St.Alfeges: seventy years on. JLL Bulletin:
Commentaries on the history of treatment evaluation. [Online] Available at:
<http://www.jameslindlibrary.org/illustrating/articles/the-miracle-of-st-alfeges-
seventy-years-on> [Accessed May 28, 2014]
Lezak, M., Howieson, D., & Loring, D,. 1995. Neurological assessment. Oxford
Univ. Press, New York, 113. Available at:
<http://scholar.google.com/scholar?hl=en&btnG=Search&q=intitle:Neurological+
assessment#7> [Accessed May 27, 2014]
Massachusetts General Hospital, 2014. Conditions&Treatments Myasthenia
Gravis [Online] Available at:
<http://www.massgeneral.org/conditions/condition.aspx?id=315> [Accessed May
27, 2014]

Mayo Clinic Staff. 2013. Myasthenia Gravis. [Online] Available at:
<http://www.mayoclinic.org/diseases-conditions/myasthenia-
gravis/basics/definition/con-20027124> [Accesed May 28, 2014]

Meriggioli MN, Sanders DB. 2012. Disorders of neuromuscular transmission. In:
Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradleys Neurology in
Clinical Practice. 6th ed. Philadelphia, PA: Elsevier Saunders.
MGNSW. 2014. Diagnosis - how is Myasthenia Gravis confirmed?. [Online]
Available at: <http://www.myasthenia.org.au/html/diagnosis.htm> [Accessed May
28, 2014]
Myasthenia Gravis Association. 2012. Myasthenia Gravis. [Online leaflet]
Available at: <http://www.patient.co.uk/health/myasthenia-gravis-leaflet>
[Accesed May 28, 2014]

National Institute of Neurological Disorder and Stroke. 2010. Myasthenia Gravis
Fact Sheet. [Online] Available at:
102
<http://www.ninds.nih.gov/disorders/myasthenia_gravis/detail_myasthenia_gravis
.htm#261043153> [Accesed May 28, 2014]

NHS Choices, 2013. Myasthenia Gravis-Treatment [Online] Available at:
<http://www.nhs.uk/Conditions/Myasthenia-gravis/Pages/Treatment.aspx>
[Accessed May 27, 2014]

N. P. Hirsch., 2007. Neuromuscular Junction in Health and Disease. [pdf] United
Kingdom: British Journal of Anaesthesia. Available at:
<http://bja.oxfordjournals.org/content/99/1/132.full.pdf+html> [Accessed May
26, 2014]
Prijambodo, B. et al., 2014. Pedoman Keterampilan Medik 1 : Pemeriksaan
Kekuatan Otot. Surabaya: Fakultas Kedokteran Universitas Airlangga
Purves D, Augustine GJ, Fitzpatrick D, et al, 2001. Neuroscience 2
nd
Edition. [e-
book] Sunderland (MA): Sinauer Associates. Available at:
<http://www.ncbi.nlm.nih.gov/books/NBK11019/> [Accessed May 26, 2014]
Rudolf, M., & Csc, . (n.d.). Neurological Examination Functional principle of
NE few possibilities are given for the physical.
Rujito, Lantip., 2012. Pemeriksaan Reflek Patologis. [pdf] Purwokerto:
Universitas Jendral Soedirman. Available at:
<kedokteran.unsoed.ac.id/Files/Kuliah/modul%20/Modul%20B3%20-
%20Pemeriksaan%20Reflek%20Patologis.pdf modul b3 pemeriksaan reflek
patologis> [Accessed May 26, 2014]
Stensaas, S., 2007. Lower and Upper Motor Neurons and the Internal Capsule. A
Resource for Learning Anatomy. [online] Available at:
<http://library.med.utah.edu/kw/hyperbrain/syllabus/syllabus10.html> [Accessed
May 29,

2014]
University of Bristol, ND. Upper Versus Lower Motor Neurone Signs. Presenting
Complaints - Assessment and Diagnosis. [online] Available at:
103
<https://www.ole.bris.ac.uk/bbcswebdav/institution/Faculty%20of%20Medicine%
20and%20Dentistry/MB%20ChB/Hippocrates%20Year%203%20Medicine%20a
nd%20Surgery/Neurology%20-%20Presenting%20complaints/page_29.htm>
[Accessed May 28,

2014]
Wati, M., 2013. Reflek Neurologis dan Lesi UMN LMN. Problem Base Learning.
[online] Available at: <http://mitaunair-fk12.web.unair.ac.id/artikel_detail-81090-
Problem%20Base%20Learning-
REFLEK%20NEUROLOGIS%20DAN%20LESI%20UMN%20LMN.html>
[Accessed May 29, 2014]
Wilson, Frank C., 1983. The Musculoskeletal System, Basic Processes and
Disorders. 2nd edition. Philadelphia: J.B. Lippincott Company.
Yayasan Myasthenia Gravis Indonesia. 2012. Myasthenia Gravis. [Online]
Available at: <http://www.mgindonesia.org/myasthenia-gravis/definisi-
myasthenia-gravis.html> [Accesed May 28, 2014]

Yayasan Myasthenia Gravis Indonesia, 2012. Pengobatan Myasthenia Gravis.
[Online] Available at: <http://www.mgindonesia.org/myasthenia-
gravis/pengobatan-myasthenia-gravis.html> [Accessed May 28, 2014]
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105

JOURNAL
APPRAISAL
106
SCIENTIFIC PAPER APPRAISAL

Group : 8A
Title : Clinical Outcome of Video-Assisted Thymectomy for
Myasthenia Gravis and Thymoma

1. COMPREHENSIVE STUDY OF THE RESEARCH

Study Item Found / Not Found (Show the page)
Title Available (Page 234)
Abstract and or Summary Available (Page 234)
Keywords Available (Page 234)
Introduction, background Available (Page 234)
Method Available (Page 234)
Result Available (Page 235)
Discussion Available (Page 238)
Acknowledgement Available (Page 239)
Reference Available (Page 239)

Conclusion : Complete/ Not Complete

107
2. RESEARCH VALIDITY

Objective : to find the clinical outcome of video-assisted thoracoscopic
thymectomy for myasthenia gravis and thymoma.

Research Method
Study Item Found (Show the page)
Design Randomized Controlled Trial (page
234)
Hierarchy of evidence 2 (page 234)
Sample 119 patients, aged 12-83 who wwre
treated between 1998 and 2007 (page
234)
Sample Size 119 patients (page 234)
Eligibility criteria 1. Myasthenia gravis symptom
severity was graded
preoperatively by the attending
neurologists, using the
Osserman classification: grade
0=asymptomatic; grade
I=ocular myasthenia gravis;
grade IIA=slow onset,
frequently ocular; grade
III=rapid onset of severe
bulbar and skeletal muscle
weakness, with respiratory
dysfunction gradually
108
spreading to skeletal and
bulbar muscles; grade
IIB=gradual onset of bulbar
muscle involvement; and grade
IV=severe myasthenia gravis
developing at least 2 years after
the onset of grade I or II
symptoms.
2. All patients were optimally
prepared for surgery medically
by the neurologist: 54.7% were
on steroids, 78.7% on
anticholinesterases, and 16%
on
immunosuppressives(azathiopri
ne or mycophenolate).(page
234-235)
Sampling frame Patients who treated with VATS (page
234)
Data collecting method The records of 119 patients undergoing
VATS thymectomy between 1998 and
2007 were reviewed
etrospectively.(page 234)
Measurement and or assessment All data, including contact telephone
numbers, were kept in a master SPSS
data spreadsheet (SPSS, Inc., Chicago,
IL, USA) and updated every 6 months
to ensure that no patient was lost to
follow-up. (page 234)
109
Instrument Line Diagram (page 237)
Randomization 119 Patients, aged 1283 years, who
were treated between 1998 and 2007
(page 234)
Intervention Video-Assisted Thymectomy (page 234-
239)
Analysis method Using multivariate regression analysis,
there were no statistical differences in
median pre- and postoperative
Osserman grades with regards to age,
sex, duration of symptoms, and
presence of thymoma. Videoassisted
thoracoscopic thymectomy for
myasthenia gravis and selected
thymomas can achieve long-term
clinical outcomes comparable to those
of standard approaches. (page 234)

Compatibility between the design and the objective :
compatible / incompatible
Compatibility between the measurement and the instrument :
compatible / incompatible
Conclusion : valid / invalid (BASED ON THE 2 CRITERIAS ABOVE)

110
3. IMPORTANCE OF THE RESEARCH

Comment for the suitable value which is used : match / unmatched

PR/OR/RR value = no
Confidence Interval value for PR/OR/RR = no
P value = <0.001
Meanings of the score = if this research is
repeated 100 times,
0,1 and 1 else wont
get the same
conclusion as this
research

Conclusion This journal research is important / unimportant
Reason Because the Confidence Interval isnt mentioned for PR/OR/RR
value

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Human Function Module 2014

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