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Deafness
12. Weakness / inability to use limbs
Both upper limbs (UL), One half of the body (hemiplegia) Both LL (Paraplegia) All 4 limbs (Quadriplegia) Ask for Onset Sudden / within few hours / days / weeks / months (Gradual) Progression
Triggering factors Sleep, Exercise, Posture, Reading, Cough, Eating, Sound, Smell, Heat & Cold
with overflow
22. Defecation
ETIOLOGICAL HISTORY Ask for history of 1. Trauma / fall (head injury) 2. Cough with Expectoration 3. Hemoptysis 4. Chest pain & Breathlessness 5. Ear discharge 6. Fever associated exanthema 7. Drug intake- Oral Contraceptives, Phenothiazines, INH, Ethambutol, Vincristine 8. Joint Pain 9. Bleeding Diathesis 10. HT, DM 11. Vaccination, Dog bite (for paraplegia) PAST HISTORY: 1. Head/ Spinal injury 2. Infections TB, Encephalitis, Sepsis 3. Rheumatic fever (joint pain) 4. h/o STD Exposure, Discharge from Urethra, penile ulcer
5. 6. 7. 8. 9. 10. 11.
h/o Recurrent Abortions/ Still births (STD) Similar Episodes before any Surgery / Drug therapy/ Vaccination Ear discharge Natal history- full term/ premature, normal/ instrumental Postnatal history- Jaundice, Infection, Convulsions, Seizures, Infancy- injury
FAMILY HISTORY: DM, HT, TB, Seizures & similar illness PERSONAL HISTORY: Veg / non-veg, Smoker, Alcohol, Ganja, LSD addiction, Tobacco chewing, Marital status OCCUPATIONAL HISTORY: Exposure to Toxic chemical neuropathy, Encephalopathy Prolonged visual work under artificial light tension headache & irritation Overuse of certain joints carpal tunnel syndrome
PHYSICAL EXAMINATION
A. GENERAL EXAMINATION: 1. Consciousness
2.
3.
4.
5.
Comfortable / not Built, nutrition, stature, short neck Fever Skull- Shape & size, any Depression/ Bulge/ Scar, Bulging Fontanelle Hair- low hair line, alopecia, texture (soft/ coarse/ normal) Eyes- color of conjunctiva (blue), Bitots spot, Phlycten, subconjunctival Hemorrhage, KF ring, Icterus, Exopthalmos/ Enopthalmos
6. 7.
8.
Eye lids- ptosis (complete/ partial), Absence of closure of eye lid Anemia, Polycythemia, Cyanosis, Clubbing, LN Oral cavity angular stomatitis (Avitaminosis), Bleeding gums
9.
10.
11. 12.
Hematological signs- Petecheae, Purpura, Ecchymosis, Erythema Lower limb Calf muscle hypertrophy, edema of feet, foot deformity (Equinovarus/ club foot) Neurocutaneous markers
i.
13.
Haemangioma, Facial angiofibroma, Ash leaf shaped hypopigmented patch (tuberous sclerosis)
ii. Neurofibromas, Lisch Nodules, Caf au Lait (light coffee) Spots (neurofibromatosis)
iii. Oculocutaneous telangectasia (blood shot conjunctiva)
Ataxia telangectasia
14. 15. 16.
Thickened nerves, Trophic ulcers, Joint swelling Spine Kyphoscoliosis, Gibbus, Meningocele, Pilodinal sinus Examination of breast
B.
NEUROLOGICAL EXAMNATION 1. Higher functions a. Consciousness & perception: find if patient is in Coma / Stupor / Delirium; find out any perception disorder
Consciousness disorders Coma state of unconsciousness in which patient does not respond to any type of external stimuli / inner needs Deep coma = coma + absence of corneal & conjunctival reflexes Semicoma = arousable with preserved reflexes Stupor state of disturbed consciousness where patient shows some response to vigourous external stimuli like pain Disorientatio conscious but muddled in time, place & person n Delirium state of confusion with excitement & hyperactivity Akinetic patient is awake but lacks impulse to speak / action mutism Dementia Acquired global/ multifocal impairment of Cognitive function in presence of normal Consciousness Locked-in Global paralysis of limbs & cranial musculature, patient is syndrome receptive but unresponsive
1. Acute cerebral dysfunction (Cerebral hypoperfusion, metabolic disease, Encephalitis) 2. Focal lesions of thalamus esp. if medial & bilat. cause Delirium 3. Lesions of Brain stem Reticular activating System 1. 2. Perception disorders Delusion false beliefs [eg: he feels that he has cancer] Common in schizophrenia, GPI, depression Hallucinations false perception of sensations [ringing ears when no sound, seeing something which doesnt exist] Hallucinations before the attack of migraine Grandiose delusion in GPI 3. Illusion- misinterpretation of stimuli 4. Obsession recurrent & persistent thoughts that intrude in pts mind despite best efforts to get rid of them b. Appearance & Behaviour (noticed as the patient walks in)
Appearance- way of dressing, personal hygiene Behaviour disturbed / apathic/ Agitated / Confused c. Emotional state Elated, Euphoric, Excited / Depressed Emotional incontinence Pseudobulbar palsy, organic dementia, multiple Sclerosis, Cerebral atherosclerosis d. Cognitive functions 1. Speech & language: communication problem are obvious while talking to patient. Find out the type of problem aphasia / Dysarthria / Dysphonia
Disorder name Defect Dysarthria Speech pronunciation problem Causes i. Bulbar palsy difficulty in consonants (p, t, k) ii. Cerebellar Disease scanning & Robotic speech. Syllables pronounced individuallly (ask pt. to say Eyeay) iii. Pseudo bulbar palsy strangled & spastic speech, Difficulty with tongue twisters (British constitution as brizh conshishushon) Laryngeal disease / innervation defect Quiet voice => poor ventilatory capacity Fatiguing voice => in Myasthenia gravis, Parkinsonism (slow monotonous speech) (Refer below for causes)
Dysphonia
Loss of voice
Aphasia
Aphasia: Type Motor aphasia / Brocas aphasia (failure of motor aspects of speech & writing) Sensory aphasia / wernickes aphasia
Lesion Lesions at inferior frontal convolution of left hemisphere [brocas area] Large lesion involves Cortical & sub Cortical structures of Frontal & Superior Sylvian fissure including Insula [upper division of it mca territory] posterior parieto-temporal region
Characteristics Poorly articulated & Nonfluent speech with Reduced no. of words & errors of syntax & grammar (telegraphic speech) Impaired comprehension of spoken & written language Fluent speech devoid of meaning (jargon aphasia) Unawareness of speech deficit Inability to repeat phrases /words spoken by examiner with normal fluency & comprehension Same as brocas & wernickes respectively Marked elements of both brocas & wernickes aphasia
Perisylvian area with damage to fibres of arcuate fasciculus Motor antrsuprr to Brocas area Sensory postrinfrr to Wernickes area Large lesion of middle cerebral arterys area / l tint. Carotid artery/ trauma
Methods of testing Assess output of speech & fluency Naming of shown objects [eg: pen, comb] Ask to carry out commands like pick up the pencil Repetition of spoken words Writing find if error inform, grammer, syntax 2. Memory past & present Memory How to test Remote marriage date, mother or fathers birth date / memory job / schools name, / school mates name Recent memory Ask about the day & what breakfast he had? Tell a short story & ask to recall after 3-5 mins Short term Ask to repeat the numbers / names & in reverse memory order. Show pictures & ask to recall after few mins 3. Orientation: Time ask the patient to tell year, date, month, day, morning / evening Place hospital / house, city
4. Intelligence
This is tested based on the following criteria i. Abstract thinking ask pt to explain meaning of common proverb
ii. Reasoning- ask pt to compare objects/ differentiate between a lie &
Constructional apraxia / visuospatial agnosia results in difficulty in drawing lines needed in correct spatial correlation
Apraxia Defect in ability to carry out known motor acts in absence of motor weakness/ sensory loss / ataxia Seen in damage of left parietal cortes / parietal white fibres of lt or both hemispheres tested by asking pt to use objects / imitate certain movements Types Limb kinetic apraxia Ideomotor Ideational apraxia Buccofacial apraxia Motor disability of onelimb, in absence of gross wkness / ataxia Inability to do the command despite comprehending the command & adqt motor & sensory fntns i.e., defect in execution Loss of ideas behind skiled movements Pt name & describe object but not know how to manipulate it Pt cant perform learned skiled movementsof mouth, lips, tongue in absence of motor paralysis of concerened muscles
Agnosia Failure to recognize known objects in presenc of intact sensory, auditory & visual pathway Type Tactile agnosia Description Pt not recog objects though Lesion Rt & lt parietal operculum
sensory, motor & coordination normal Not recog object seen with eyes though visual pathway intact Objs color, size are described Inability to identify familiar face Lack of awareness to recog paralysed limb
Postr. Insula Bilat / left occipitotemporal Parieto occipital lesion Rt parietal lobe lesion
Handedness rt / lt g. Sleep duration, intermittent awakening, day time sleeping (narcolepsy), sleep walking & other motor activities (somnambulism), sleep aneuresis
f. Disorders Dyssomnia Intrinsic Extrinsic Parasomnia Arousal disorders Asso. With REM slp Disorders asso with medical/ psychiatric disorder Somnambulism Sleep eneuresis
Psychophysiologic, idiopathic, narcolepsy Adjustment sleep disorder, altitude insomnia,food allergy insomnia Confusional arousal, sleep walking Nightmares, sleep paralysis, sleep related painful erection Mental disorder- schizophrenia, anxiety, depression Neurological disorder parkinsomnism, sleep related epilepsy Medical- sleeping sickness, COPD, chronic renal failure, drugs Common in children & adolescents Primary fsilure to attain continence since birth Secondary emotional disturbance, UT Infection &malformations, Epilepsy, Cauda equina lesion
Frontal lobe Lesions Unilateral frontal Right/ left lobe Contra lateral spastic Hemiplegia Anosmia [orbital part] Impaired memory
Prefrontal
Bilat frontal
Dominant left lobe Parietal lobe lesions 1. Unilat. Lobe Lt / Rt 2. Dominant Lt lobe
Presence of primitive reflexes [grasping & sucking] Elevation of mood, Talkativeness Loss of initiative Bilateral Hemiplegia, Pseudobulbar palsy Prefrontal lesion-abulia / akinetic mutism, lack of attention & problem solving ability, labile mood, Primitive reflexes Agraphia, Apraxia of lips & tongue, Loss of Verbal Fluency
Cortical sensory loss, mild hemiparesis, homonymous inferior quadrantanopia, neglect of one half of body In addition to defects in (1) above Disorders of Language[alexia], Gertsman syndrome [defect in writing, calculaton, finger agnosia, Right & left disorientation], Tactile Agnosia Bilateral Ideomotor & Ideational Apraxia In addition to (1) Dressing apraxia, Anosognosia, Construction Apraxia, Visuospatial disorders Visuospatial imperception, topographic memory loss, Anosognosia, Construction apraxia, Spatial disorientation
Temporal lobe lesions 1. unilat Rt / Lt 2. dominant Lt. lobe 3. non dominant Rt. Lobe 4. bilateral Hallucinations auditory, visual, olfactory, gustatory Emotional & behavioural changes, Dreamy state with Uncinate seizures, Homonymous superior Quadrantinopia In addition to (1) Alexia, Color anomia (Splenium of corpus callosum) In addition to (1) Inability to judge spatial relationships in some cases Impairment of non verbal memory Agnosia fo sounds & some qualities of music a. Korsakoff amnesic defect b. Apathy & Placidity c. Increased sexual activity d. Sham rage b + c + d = Kluver Bucy syndrome
Occipital lobe lesions 1. unilat (Rt / Contralat. Homonymous hemianopia central/ peripheral Lt) Elementary Hallucinations in irritativee lesion 2. dominant left In addition to (1)
Alexia & Color anomia (Splenium of cor. Callosum), Object agnosia in addition to(1) Loss of visual orientation & topographic memory, Contralateral homonymous hemianopia, hallucinations Cortical blindness (pupils reactive) Loss of color perception, Inability to identify familiar faces (Proposognosia)
2.
Cranial nerves
Pure motor cranial nerves Pure sensory cranial nerves Both motor & sensory cranial nerves 3,4,6,11,12 1,2 & 8 5,7,9 & 10
Olfactory nerve
o rule out local lesion o Sense of smell in each nostril separately o Items used Tea, Coffee, Astafoedia
Lesions Anosmia: loss of smell sensation Causes - nasal diseases, head injury, tumours of anterior cranial fossa, chronic basal meningitis (TB, Syphilis, Neoplasm), Kallmans syndrome (anosmia, obesity, hypogonadism)
Optic nerve o Visual acuity each eye separately at 6 mts; At Bedside Snellens charts o Visual field by confrontation method
o Colour vision Red, Green, Blue & Yellow
o Fundus examination
Lesions 1. decreased visual acuity Primary ocular disorders, Refractory errors, Papillitis, Retrobulbar neuritis 2. unilateral loss of vision Ocular lesions, Carotid hemiplegia 3. defects of visual field (scotoma = characteristic field defects) Types of scotoma Name Definition Central Loss confined to Scotoma Central region of
Bilateral (biltl) vit B12 deficiency Disease of Choroid / Retina near the macula Unilat. vascular disease like retinal embolism, retinal artery occlusion Bilat toxic causes like Alcoholism, B12 diseases Arcuate (comma shaped) Glaucoma damaging nerve bundle in retina / optic nerve Long standing papilloedema, bilat. lesions of Striate (visual) Cortex, Retinitis pigmentosa, Hysteria Homonymous nasal field of one eye & temporal of other Heteronymous loss of nasal / temporal of both eyes Bitemporal lesion of optic chiasm (pituitary tumor), compression in mid-line Binasal lateral compression of chiasma Lesions of optic radiation & calcarine cortex Superior - temporal lobe Inferior parietal lobe
Concentric constriction of visual field Loss of vision in one half of visual field
Quadrantanopia
4. 5. 6. 7.
papilloedema all causes of raised intra cranial tension (tumors, abscess), central vein occlusion, HT, Polycythemia, Toxins (vit. A intoxication. Hypoparathyroidism) primary optic atrophy Neuro- syphilis, Sellar / Para sellar tumor, Fredreichs ataxia, Lebers optic atrophy, Multiple sclerosis color blindness hereditary & bilat. Visual cortex lesions pupilary reflex Afferent lesions (optic merve) Efferent lesions (3rd nerve) Direct reflex lost, Consensual reflex preserved Pupil fixed & dilated direct reflex lost, Accomodation also lost Response is seen in contra lat. Pupil
Elevators Depressors Abductors Adductors Internal rotators (intorsion ) External rotators (extorsion)
Sup rectus, Inf oblique Inf rectus, Sup oblique Lat rectus, Inf oblique Med rectus, Inf oblique Sup rectus, Sup oblique Inf rectus, Inf oblique
Lesions Diplopia (double vision) Types Uni & Binocular; Crossed & Uncrossed Uniocular Due to ocular conditions Binocular Weakness of Muscles; Occurs when Eyes are open Crossed Paralysis of ADDUCTOR Muscle - Medial, Superior & Inferior recti palsy Uncrossed Paralysis of ABDUCTOR Muscle Lat rectus, Sup & Inf. Oblique palsy 2. Pupil position, size, shape, equal / unequal Pupil size (normal 3-5 mm) Miotic Mydriatri c <3 mm >5mm Old age, Horners syndrome, Pontine hemorrhage, Drugs like morphine, Neostigmine, Organo phosphate poisoning 3rd nerve palsy, Optic atrophy, Infants Drugs like Atrophine, Pethidine Seen in diseases Neurosyphilis (tabes dorsalis), DM, encephalitis, Disseminated Sclerosis
Special types of pupil Definition Argyl Small, irregular, unequal. Robertson Light reflex lost Accommodation pupil reflex preserved, Poor response to mydriatric Horners syndrome (Sympathetic lesion) Hippus Miosis, Anhydrosis, Enopthalmos, Ciliospinal reflex absent
Vasculitis, encephalitis, Syringomyleia, Pan coasts tumor, Cervical rib, Brain stem lesion, pontine glioma Multiple sclerosis, Syphilis, Neoplasm
Types & cause of Jerky Nystagmus: 1. Horizontal To & fro movement of eye ball in horizontal plane 2. Vertical Up & down movement of eye ball in vertical plane 3. Rotatory Osscilatory movement of eye ball whch is rotatory in character Rare forms of Nystagmus:
Lesions of Vestibular nerve& nuclei, Medial longitudinal bundle, Cerebellum [Nystagmus to the side of lesion] Seen in conditions involving Brain stem Vascular accident, Encephalitis, Multiple sclerosis, Syringobulbia, druds [ anticonvulsants, BZD, Barbiturates], Wernickes encephalopathy Seen in Labyrinthine disorders
See saw nystagmus Convergence retraction nystagmus Upbeat nystagmus Downbeat nystagmus Rebound nystamus
Unilatera l
involvement) Pea aneurysm, Herniation of uncus, Cavernous sinus thrombosis, DM, HT, Collagen disease, Horners syndrome, Trauma 3rd nerve palsy with papillary sparing
Trigeminal nerve
Motor temporalis, masseter, Pterygoids Sensory sensations over the face Corneal & conjunctival reflex Lesions Name Nuclear lesion Preganglionic nerve lesion Affected area Diseases affecting Pons, Medulla, Upper cervical cord(C2) Preganglionic Assiociated lesions of other cranial nerves esp VI, VII, VIII (Characterised by severe facial pain) Diseases Tumor, Demylination, Vascular lesions, Syringomylia / Syringobulbia Tumors (Meningoma, Nasopharyngeal carcinoma, Cerebello-pontine angle tumor), Meningeal irritation (Acute / chronic meningitis, Carcinomatus meningitis) Tumors, Abscess, Herpes-zoster opthalmicus a. Cavernous sinus lesion b. Gradenigos syndrome c. Superior orbital fissure syndrome
Facial nerve
Motor: eye- Frowning, eye closure, Raising of Eye brows, Browing, Mouth & cheek whistle, showing teeth, nasolabial fold, platysma Sensory: taste in antr 2/3 of tongue Lesions Types unilat. & bilat.; each being futher divided as upper motor neuron type (UMN) & lower motor neuron type(LMN) Lesions Causes Unilateral UMN Usually vascular, Cerebral tumor, Multiple Sclerosis LMN Bell s palsy, Parotid tumor, Head injury, Skull base tumors, DM, HT Bilateral UMN Vascular, Motor neuron disease LMN Guilian barre Syndrome (GBS), Sarcoidosis Uveoparotid Fever, Leprosy, Leukemia, Lymphoma
Differences between UMN Bells phenomenon (movement of eye ball upwards & inwards on closing eyelids- well seen) Emotional fibres spared Associated with Long Tract signs Exaggerated Jawjerk Corneal reflex present
&
LMN type of facial palsy Bells phenomenon absent Emotional fibres involoved Absent Normal Jaw Jerk Absent
Vestibulocochlear nerve Acuity of hearing (eg- rubbing of paper) in both ears separately Rinnes test: using tuning fork ( 256 or 512hz) over mastoid process & then lateral to ear & find out when it is louder. Positive Air > Normal ear Bone Nerve deafness(sensorineural deafness) Negativ Bone > Middle ear deafness (conductive e Air Deafness) Webers test: place vibrating tuning fork over middle of fore head, Point of Application (middle of forehead) (No lateralization) Better in Normal Ear (lateralized to normal side) Better heard in Affected Ear (lateralized to affected side) Normal Nerve deafness on Opposite side Conductive deafness on Same side
Lesions of VIII nerve Vertigo = hallucinations of movement of either the body / the surroundings causes peripheral labyrinthine disorders, Cerebellar lesions, VBI Deafness neural Peripheral labyrinthine disorders Acoustic neuroma, Meniere s disease, Vestibular neuronitis
IX & X neves [glossopharyngeal & vagus]: Gag reflex & say ah & look for deviation in uvula Sensations in posterior 1/3rd of tongue & pharynx
Lesions of IX, X, XI nerves Palate remains immobile on both sides Dysphagia, Nasal regurgitation, Pharyngeal reflex absent Unilateral Palatal arch on that side is at lower level than on healthy side Bilateral GBS, Polyneuritis, Myasthenia gravis, Motor neuron Disease PICA, Tumor, Demylination, Syringobulbia, Secondaries, meningioma, Stroke
Accessory nerve Trapezius & sternomastoid shrugging of shoulder with & without resistance, turning head on both sides separately with & without resistance Hypoglossal nerve Appearance of tongue, wasting / contracted/ Fasciculations Movement of tongue & deviation of tongue
Lesions of XII nerve Causes infarction, tumour, MND, VB aneurysm, trauma, meningioma, metastasis Types LMN & UMN Differences between Wasting Fibrillation Tongue Tone Deviation Protrusion Jaw jerk 3. LMN & Present Present Flaccid Towards affected side Possible Normal UMN of XII nerve Absent Absent Small & spastic Difficult Exaggerated
Motor system: a. Nutrition (bulk of muscle) i. comparing with Opposite side, palpating the muscles ii. Note any wasting / hypertrophy & its distributionpredominantly Proximal / Distal / Both Feel for muscles Atrophic muscle Flabby Hypertrophic Firm / muscle Rubbery iii. Measure the circumference of limbs
1. 10cm Above Olecranon 2. 10 cm Below Olecranon 1. 18cm Above patella 2. 10cm below Tibial tuberosity
Spinal muscular disease, Motor neuron disease, Syringomyelia, Compressive lesion (C5- C6), Lesion of upper brachial plexus (Br Plxs) like Erbs paralysis, late stage of muscular dystrophies, inflammatory disease (poliomyelitis, Poliomyositis) Motor neuron disease, syringomyelia, Cervical cord tumors (C8,T1), lesion of lower Br. Plxs (Klumpkes paralysis), cervical rib, cervical gland enlargement, Pancoast syndrome, traumatic lesion of Radial, Median, Ulnar nerves, peripheral neuropathy, Peroneal muscular atrophy Vertebral lesion (metastasis), spinal cord lesions (syringomyelia, tumors), antr. Horn cells (motor neuron disease, poliomyelitis), root lesions, br. Plxs (klumpkes paralysis, cervical rib), peripheral nerve lesion (hansens disease, Carpal tunnel syndrome), disuse atrophy (fracture plaster)
3. lower limbs Only Lower limbs Upper limbs in addition LL Cauda equina lesion, Peripheral neuropathy, peroneal muscular atrophy, poliomyelitis, peripheral nerve trauma (lat. Popliteal nerve), tarsal tinnel syndrome Peroneal muscular atropy, Chronic poly neuropathy, Spinal muscular atrophy, Hansens disease
Uniform resistance offered to passive Parkinsonism (post 1. movement encephalitic) 2. basal ganglia neoplasms 3. catatonia Resistance offered to passive mvmt 1. parkinsons disease interrupted by alternate contractions 2. over dose of reserpine, of agonist & antagonist muscle due to Chlorpromazine Asso. Tremor 3. carbon monoxide poisoning
Causes of hypotonia i. lesions of motor side of Reflex arc- Poliomyelitis, Polyneuritis, trauma of peph. nerve ii. lesions of sensory side of - Tabes dorsalis, herpes zoster, carcinomatous neuropathies iii. combined motor & sensory lesions syringomyelia, cord / root compression iv. cerebellar lesions, Chorea Clonus Sudden stretching of muscle produces reflex contraction. If stretch maintained during subsequent contraction further reflex contraction occurs & continues till stimulus removed Dorsiflexion of the foot after flexing the hip & knee ( ankle clonus), sharp movement of patella downwards ( knee clonus) Sustained clonus => pyramidal tract lesion (UMN) Illsustained => tense persons, after straining (defecation)
c. Power Tested in all joints (flexion, extension, abduction, adduction) Grading (Medical Research Council)
Grade 0 Grade1 Grade 2 Grade3 Grade 4 Grade 5 No visible contraction Visible musle contraction, but no movement of joints Movement with gravity eliminated Movement against gravity Movement against resistance Normal power
d. Coordination upper limb 1. 2. 3. 4. 5. 6. 4. lower limb Finger Toe test ii. Heel Knee test iii. Tandem walking
i.
Outstretched arms test Finger Nose test Nose Finger Nose test Finger to Finger test Pronation Supination test Pointing & Past pointing test
Involuntary movements
Name Chorea Definition semi-purposive, irregular, non- repetitive &brief jerky movement arising in proximal joints & appearin g to flit from one part to other randomly Increased on attempting voluntary movement & Exacerbated by emotional disturbance Slow writhing movement Best seen at wrists (flexing), fingers (writhing), foot (inverted) Proximal joints of one arm resulting in Wild, Rapid, Flinging movement of wide radius, occurring constantly with short periods of freedom Involuntary sustained muscle contractions frequently causing Twisting & repetitive movements Causes & types Lesion in caudate nucleus Genetic huntingtons, hereditary Hemichorea stroke, tumor, trauma, post thalamectomy Drug neuroleptics, phenytoin, alcohol, oral contraceptives Symptomatic chorea Encephalitis lethargica, sub dural hematoma, Cerebrovascular disease, hypoparathyroidism, hypernatremia , primary polycythemia Lesion in putamen
Athetosis
Hemiballis mus
Lesion in subthalamic nucleus Most dramatic of all involuntary movements may injure patient / bystanders Genetic primary generalized D, Focal adult onset D Heredito degenerative Ataxia telangectasia, Lipid storage diseases, Wilsons diseases, Parkinsons disease Symptomatic Athetoid cerebral palsy, cerebral anoxia, post-encephalitic dystonia Drugs neuroleptics, Mn poisoning,
Dystonia
Tremors
Rhythmical & oscillatory movement of body part caused by rhythmic contractions of agonst & antagonist muscles
Myoclonus
Rapid, brief shock like muscle jerks that are often repetitive & rhythmical
Tics
Repetitive, irregular stereotyped movements / vocalizations that can be imitated, Irregular, non rhythmic contraction of muscle fascicles Contraction of individual muscles Involuntary nodding of head
Levodopa Hemidystonia stroke, trauma, tumor, AV malformations Rest tremors parkinsons disease, post encephalitic parkinsonism, drug induced parkinsonism Postural - physiological, thyrotoxicosis, anxiety, alcohol, caffeine Intentional lesions of cerebellum & its connections in diseases like multiple sclerosis, Spinocerebellar degeneration, tumor Generalized progressive myoclonic encephalopathies, hereditary myoclonus, metabolic (Tay- sachs & Battens disease), Alzheimers disease Metabolic Uremia, hyponatremia, hypocalcemia, hepatic failure Drug induced, alcohol & drug withdsnal Focal / segmental spinal tumor/ infarct/ trauma, palatal myoclonus Simple tics transient tic of childhood, chronic simple tic Complex multiple tics chronic multiple tics Symptomatic tic encephalitis lethargica, drug induced, post traumatic Motor neuron disease, syringomyelia, cervical spondylosis, peroneal muscular atrophy, poliomyelitis (in recovery stage) Denervation hypersensitivity Easily perceived over the tongue where they are easily seen under mucus membrane Lesions of Vermis of cerebellum
f.
Gait Patient is asked to walk in a straight line for at least 9 metres, & asked to turn and walk back
Gait Circumductio n gait Seen in Hemiparesis Description Pt. throws his lower limb outwards at hip joint (circumduction), & leans towards opposite healthy side Affected arm adducted at shoulder & flexed at elbow, wrist & fingers Adductor spasm causes legs to cross each other & each foot trips the other Marked spasm of adductors of lower limbs
Lesion of UMN involving both lower limbs Marked spastic gait as in cerebral diplegia
Ataxic gait
Cerebellar lesion
Shuffling gait
Waddling gait
Primary muscular diseaseproximal weakness of lower limbs(muscular dystrophy)/ bilateral hip problem (congenital dislocation of hips)
Pt raises foot high to overcome foot drop & on keeping foot down toe hits ground first There is no ataxia Pt not know where his foot is & so on walking raises foot high up in air & brings it down forcefully (stamping gait), heel touches ground first More prominent in dark / pts eyes closed Pt is ataxic & reels in any direction including backwards & walks on a broad base Difficulty in tandem walking Series of small flat footed shuffles Parkinsonism pt. stooped posture (universal flexion) & walks in small, shuffling steps as if trying to catch up with center of gravity Automatic assoc. upper limb absent Pt walks with broad base with exaggerated lumbar lordosis
Lesions of Extra pyramidal system Sign Resting tremor Muscular rigidity Hypokinesia Chorea Hemiballismus Dystonia, Athetosis Site of lesion Substantia Nigra, Red nucleus SN, Putamen SN, Putamen, Globus pallidus Caudate nucleus Sub thalamic nucleus Putamen
Signs & symptoms of Parkinsons disease & Parkinson plus syndrome (progressive supranuclear palsy, Dementia with Lewy bodies, Multiple system atrophy) Feature Tremor Tone Movements Thought process Parkinsons disease Rest tremor (pill rolling) Rigidity -cogwheel (best seen in UL) / lead pipe(best seen in LL & trunk) Bradykinesia Bradyphrenia (slow thought) Parkinson plus syndrome Action tremor & impotence Marked axial rigidity -
Saccades & pursuit movements Facial expressions Handwriting Posture Gait ANS dysfunction 4. Sensory system:
Slight jerky saccades Pursuit eye movements Facial hypomimia (lack of facial expression) Micrographia (small hand writing) Stooped Festinant (short shuffling gait) Mild
Vertical supranuclear saccade (PSP) Broken pursuit (MSA) Falls early in disease course Ataxic Severe
i. Spinothalamic sensations a. Touch Skin is lightly touched with Cotton wool shaped to a point with eyes open & closed in dermatomal areas b. Pain Superficial pain - Sharp pin is used Deep pain squeezing of muscles (usually of calf) & tendons c. Temperature Hot & cold water
Sensory levels (spinal cord shorter; end at L1) Vertebrae Lower cervical T1-6 T7 -9 T10 T11 T12 L1 Segmental level of spinal cord +1 +2 +3 Overlies L1 & 2 L3 & 4 L5 sacral & coccygeal segments
ii.
Posterior column sensations Vibration sense 128 hz preferred over 512 hz as 128 hz fork decays later Only stem of fork touched (not the pongs) on bony prominences Joint sense Eyes closed Joint fixed & digit moved up / down Ask pt. to tell direction of movement Repeated many times avoid alternate movements
In posterior column deficit there is numbness in affected limb Position sense Pts eyes closed & ask pt to Tip of Forefinger of one hand with other Place forefinger on tip of nose & heel on knee accurately d. Rombergs sign Pt stands upright with feet together & eyes closed In proprioceptive / vestibular deficit balance is impaired & pt may fall if not caught Minimal lesions demonstrated by asking to stand on toe with eyes closed e. Lhermettes sign / barber chair sign In lesion of postr column of cervical region sudden flexion / extension of neck give rise to Electric shock like sensation that travls rapidly down trunk & even hands, feet + ve in Multiple sclerosis, Cervical spondylosis, Syringomyelia, tumor of cervical cord, Subacute combined degeneration of spinal cord
iii.
Cortical sensations 1. Tactile localization Ability to correctly localize pt. touched with head of pin / finger tip 2. Tactile discrimination Finger pulp & lips 3-5 mm well recog. Palm 2-3 cm Sole 4 cm Dorsum of foot, > 5 cm Legs, Back
Loss of Tactile Discrimination in presence of intact Posterior Column sensation => Parietal lobe lesion
3. Stereognosis Ability to identify object purely by feel of its shape & size Use familiar objects 4. Barognosis 5. Graphesthesia
Ability to recognize letters/ numbers / diagrams written on skin with blunt point Loss of graphaesthesia (agraphaesthesia) seen in lesions of parietal lobe when peripheral sensations are normal
6. Cortical inattention
plantar reflexes may be extensor & knee jerk may be loss of Vibration sense alone loss of Position & Vibration only retained if affecting lower limbs =>intrinsic cord lesions like multiple sclerosis, syringomyelia postr. Column lesion as in tabes dorsalis, subacute combined degeneration
i.
Abdominal
Cremasteric
Plantar
Clinical Significance of Babinskis sign 1. Lesions of corticospinal tract (pyramidal tract) 2. infancy ( upto 1year)
3. 4. 5. 6. 7.
deep sleep deep anesthesia narcotic overdose alcohol intoxication post traumatic state
Other methods of eliciting Plantar reflexes 1. Oppenheim reflex firm stroke with finger & thumb down either side of antr, brdr. Of tibia , more pressure on mdl side 2. Gordon reflex calf muscles squeezed 3. Chaddock reflex light stroke below lat. malleolous
ii.
deep reflexes
Spinal segment involved Biceps C5 Triceps C7 Supinator C5, 6 Hoffman C7, 8, T1 Knee L3, 4 Ankles L5, S1 Pectoral C7 Deltoid C5 All reflexes should be tested on both sides 0 1 2 3 4 Grading Absent Present as Normal jerk Brisk as a normal Knee jerk Very brisk Clonus
iii. Reflex Inverted radial reflex Inverted biceps Inverted Knee reflex
Inverted reflexes Procedure Supinator jerk => absence of elbow flexion but there is finger flexion Biceps jerk absent & triceps exaggerated Biceps reflex => no flexion of elbow but extension of elbow due to triceps contraction Knee jerk => no extension instead flexion of knee due to contraction of hamstrings Lesion Presence => C5,6 segment lesion Presence=>C5,6 segment lesion Presence=>Lesion of L2,3,4
Other allied reflexes Procedure Reflex Hoffmann reflex Wartenbergs reflex Terminal phalanx of pts mid finger flicked downwards between examiners finger & thumb Ptshand supinated, slightly flexing fingers with thumb in adduction , Examiner pronates his hand & links his hand with that of pts fingers. Both flex their fingers & pull against each others resistance Lesion In hypertonia tips of other fingers flex & thumb flexes and adducts Normally thumb extends but terminal phalanx may flex slightly In hypertonia => thumb adducts & flexes strongly Indicating pyramidal tract lesion
v.
sphincteric reflexes ask the pt. for difficulties in Swallowing, Defecation, Miicturition, Sexual function primitive reflexes (released reflexes) Deficit of frontal function release primitive motor behavior which includes following reflexes Reflex Pout reflex Grasp reflex Description Rubbing of chin causes pouting / sucking lip movements Pt tends to grasp objects esp examiners fingers when they are placed in his palm, esp in contact Palmo mental reflex between thumb & index finger Scratching the palm produces unilateral contraction of mentalis muscle Non specific sign sometimes seen in normal people also Clinical inference Polysynaptic reflex released by frontal lobe disease / diffuse degenerative brain disease -
vi.
6. Autonomic nervous system i. check pupillary response to light & accommodation ii. skin dryness => absence of sweating iii. resting tachycardia- present / not iv. pulse rate slowing with deep inspiration v. trophic changes in distal skin absence of hair growth, nail bed
Bladder Innervation Innervation Segment Part innervated
Parasymp. Emptying Symp. (also carries pain sensation ) filling Somatic Types of Bladder & Incomplete spastic / uninhibited bladder
Detrussor muscle via pelvic nerve Trigone muscle via presacral & hypogastric nerves External sphincter & perineal muscle pudendal nerve
Complete spastic / reflex / automatic / hypertonic bladder Autonomous bladder / hypotonic Sensory paralytic (afferent pathway lesion ) Atonic bladder (reflec arc lesion) Motor paralytic (efferent pathway lesion )
Levels of Lesions, clinical condition Post central loss of awareness of bladder fullness, incontinence Precentral difficulty in initiating micturition Frontal inappropriate micturition, loss of social control (like infants bladder) Lesion in spinal segments above S2,3,4 Lesion at S2,3,4 & Cauda equina Seen in DM, Syringomyelia, Tabes dorsalis Intact voluntary initiation of micturition Urinary retention- overflow incontinence Frequent urinary tract infection Seen in Tabes dorsalis, Conus & Cauda Seen in lumbar canal stenosis Lumbo-sacral meningio- myelocele Painful urinary retention
7. Cerebellar signs Part Archicerebellum Paleocerebellu m Neocerebellum Parts Connected to Vestibular nuclei Spinal cord Cerebral cortex Function Maintain equilibrium Maintain posture Center of voluntary movements
Lesions of cerebellum Clinical signs 1. Hypotonia , 2. Dysmetria (inappropriate range of movement) 3. Intention tremor 4. Adiadochokinesia 5. Rebound phenomenon
6. Nystagmus quick & towards direction of gaze, occasionally skew deviation in acute lesions 7. Jerky, explosive speech, Disturbance of articulation & phonation in lesion of vermis 8. Gait staggering towards affected side + ataxia 9. Pendular knee jerk 10. Titubation Causes 1. Fredreichs ataxia (other signs scoliosis, pes cavus, Ataxia, Dysarthria, Nystagmus, Muscle weakness, Optic atrophy, Deafness) 2. Cerebellar abscess (acute) & tumors, paraneoplastic syndrome 3. Vascular causes 4. Hypothyroidism 5. Alcohol 6. Drugs like Phenytoin, Babiturates, Aminoglycosides 7. Refsums disease
Scoliosis = lateral curvature of spine Postural, congenital, unequal limb in length, acute disc collapse, Extensive fibrosis of lungs, inflammatory disorders Kyphoscoliosis = lateral + posterior bending of spine (kypho = backward bending) Congenital, poliomyelitis, neuromuscular disorders Tenderness Height- neck ratio Auscultation over skull b. Signs of meningeal irritation Usually meningitis, sub-arachnoid hemorrhage
In Meningeal irritation Flexion causes Pain in posterior part of neck & Movement is resisted by spasm of extensor muscles Pain & Spasm of hamstrings in Meningeal irritation of lower part of spinal sub-arachnoid space
Pt supine on bed Passively extend pts knee on either side when hip is fully flexed Brudzinkis sign Ophisthotonus
iii. iv.
9. OTHER SYSTEMS CVS Look for Hypertension, valvular heart disease, [AF] TOF, Peripheral pulsation, carotid pulse & bruit RS: Look for Evidence of TB, lung abcess, bronchiectiasis, corpulmonale, bronchogenic carcinoma ABDOMEN: Look for Hepato-spleenomegaly, ascitis, polycystic diseases, hepatocellular failure, any mass
Mdl surface of Temp & Occp lobes & their Tentorial surface, Cerebellum, Medulla, Pons, Midbrain, Thalamus, Sub thalamus
Terminal occlusion=>visual cortex Thalamogeniculate branch=> various thalamic & capsular features like hemianopia, Speech arrest, tremor, dystonia, contralat sensory loss Main trunk=> all above effects
Vertebral artery branches a. Meningeal b. posterior, c. spinal, d. anterior spinal e. medulary f. postereior inferior cerbellar Infarction of posterior inferior cerebellar artery Infarct Level Paramedian infarct Basillary infarct Posterolat infarct (wallenberg s syndrome) Regions involved 12th nerve nucl, mdl lemniscus & medullary pyramid infr olive & pyramid Symp. Tract, spinothalamic tract, nuc of tract of 5th nerve, lower vestibular nuc, infr cerbellar peduncle, 9th & 10th nerve nuclei Clinical Effects tongue weakness, paralysis& absent uni/bi lat position sense Contralat ataxia & motor adaptation failure and contralat hemiplegia Ipsilat horners, facial numbness & ataxia, loss of contralat pinprick sensation & vestibular disturbance, speech & swallowing difficulty
Basilar artery branches 1. Pontine artery 2. Labyrinthine artery 3. anterior inferior Cerebellar artery Infarction of anterior inferior cerebellar artery Infarct Level Regions involved Clinical Effects
6th & 7th nerve nuclei & mdl lemniscal tract 6th & 7th nerve fascicles & pyramidal pathways Symp. Pathway , Spinothalamic tract, middle cerebellar peduncle, 5th nerve nucleus, vestibular, cochlear & eye movemt control nuclei
Lat gaze failure , LMN facial wekness & loss of contra lat body position sense Diplopia, LMN facial weakness & contralat UMN facial weakness Ipsilat sensory loss in face Ipsilat clumsiness of body & loss of contralat pin prick sensation, Nystagmus & horners syndrome
4. superior Cerebellar artery Infarction of superior cerebellar artery Infarct Level Paramedian infarct Basillary infarct (webers syndrome) Posterolat infarct Regions involved Red nucleus & 3rd nerve nucleus 3rd nerve fascicule & cerebral peduncle Spinothalamic, Mdl lemniscus, & symp. Tracts & superior cerebellar peduncle Clinical Effects Contralat ataxia & 3rd nerve palsy Ipsilat 3rd nerve palsy & contralat hemiplegia (face & body) Total contralat sensory loss, ipsilat hoirners syndrome& sometimes ipsilat ataxia
Stroke
Definition acute neurological injury occurring due to vascular pathological processes that manifest as brain infarct /hemorrhage Major causes Age, Obesity, HT, Smoking, DM, Alcohol, Oral contraceptives Hematological causes Polycythemia, thrombocytopenia, Sickle cell disease, leukemia, prorein C & S deficiency Cardiac causes Rheumatic valve disease, atrial fibrillation , myocardial infarction, cardiomyopathy, patent foramen ovale Unusual causes Marfans syndrome, AIDS, cervical irradiation, drug abuse, scleroderma Clinical classification 1. completed stroke 2. evolving stroke Rapid onset Persistent ND that does not progress beyond 96 hours Gradual step wise development of ND
3. transaient ischemic attack 4. Reversible Ischemic Neurological Deficit (RIND) Blood supply of Internal capsule
Focal ND resolves completely within 24 hours The ND completely resolves within 1- 3 weeks
5. Superior half by lenticulostriate , branch of MCA 6. Inferior half anteriorly ACA (Hubners artery), 7. Posterior limb 8. Anterior 1/3 posterior communicating 9. Posterior 2/3 anterior choroidal artery 10. ACA supplies bladder & leg areas Blood supply of spinal cord 11. Anterior 2/3rd of cord - one anterior spinal artery 12. Posterior 2/3rd of cord - a pair of posterior spinal artery
Spinal cord
Relationship of spinal segments Vertebrae Lower cervical T1-6 T7 -9 T10 T11 T12 L1 Position of fibres Column Posterior column Lateral & Anterior Column Segmental level of spinal cord +1 +2 +3 Overlies L1 & 2 L3 & 4 L5 sacral & coccygeal segments
Position of Fibres Fibres from Lower Limb are placed medially near central canal & from Upper Limb are placed laterally Corticospinal & Spinothalamic tracts
Touch , Position, movement, Vibration & pressure sense Cervical, thoracic, lumbar, sacral
Lesions
o Localization of segmental level Order of importance First note Segmental symptoms like root pain, hyperalgesia, atrophic paralysis Next is upper limit of sensory loss upper limit of area of analgesia indicates lower segment compressed Remember that sensory level for pain sensation suggest that, lesion is several segments lower than actual site Segment Foramen magnum signs Atrophy of sterno mastoid muscle Downbeat Nystagmus C2 sensory loss & cerebellar signs Horners syndrome Cervicomedullary junction (hemiplegia cruciata) C2 segment Lower cranial nerve palsy Paralysis of ipsilateral lower limb & contralateral UL (due to arm fibress ccrossing before the leg fibres at lower part of medulla) 1. Sub-occipital pain / sensory loss 2. Descending tract of Vnerve (pain & temp. loss over face 3. Exaggerated trapezius reflex Loss of trapezius reflex 1. Inverted biceps jerk 2. Inverted brachioradialis jerk (supinator jerk) 3. Sensory loss over deltoid 1. Dimished biceps & supinator reflex 2. Exaggerated finger flexor reflex 1. 2. 3. 4. Paresis of flexors & extensors of wrist & fingers Preservation of biceps & supinator reflex Exaggerated finger flexor reflex Inverted triceps reflex
C3 C5
C6 C7
C8 & T1
Thoracic segments T3 T4 T6
1. Weakness & wasting of small muscles of hand 2. UMN signs in lower limb 3. Uni / bilat. Horners syndrome 1. Girdle pain / paraesthesia 2. Segmental LMN involvement 3. ANS dysfunction Sensory impairment in axilla Sensory impairment below level of nipple Abdominal reflex impaired
T10
+ ve beevors sign (intact upper abd reflex & absent lower abd. Reflex with pull of umbilicus >3 cm on raising the head) 1. 2. 3. 4. 1. 2. 3. 1. 2. 3. 4. 1. 2. 3.
T12
L3, L4
S!, S2
S3, S4
Abdominal reflex preserved Sensory loss in LL staring from level of groin Brisk ankle & knee jerk Absence of cremasteric reflex Hip flexion preserved Atrophic paralysis of quadriceps & adductors of hip Loss of knee jerk & ankle jerk Atrophic paralysis of intrinsic muscles of foot, calf muscles Knee jerk preserved Ankle jerk & plantar reflex lost Anal & bulbocavernous preserved Saddle anesthesia Bladder & bowel involved Normal reflexes in LL
Compression of spinal cord Effects of compression Slow spinal compression affects First the pyramidal tract Next posterior column Spinothalamic tract LMN signs Anterior horn cell Posterior root Posterior column Pyramidal tract LMN signs (weakness, wasting, fasciculation) Root pain/ girdle pain (trunk) Lhermittes sign (unpleasant / electric sensation) Constriction band around trunk UMN signs
Differentiating between
1. Intra-medullary & Extra medullary lesions
Motor UMN signs Spasticity & muscle spasm LMN signs Muscle atrophy Trohic changes Fasciculation Sensory Root pain . Funicular .. Dys& paraesthesias Dissociated sensory loss. Sacral sensation. Joint position sense . Lhermittes sign . ANS Bowel & bladder disturbance Investigation X-ray of spine Effect of lumbar puncture . 2.
Common & persistent 1or 2 segments at site of root compression Not common Rare Common Not present Rare Absent Lost Lost Present Late Bony changes seen Signs & symptoms are pptd / increased
Less common Wide due to antr. Horn involvement Present Common Rare Present Common Present Sacral sparing for Pain & Temp Spared Absent Early Not seen No such effect
Lesions of Conus medullaris Onset Symmetrical Disso. Sensory loss Present Root pain Rare Fasciculation Rare Decubitus ulcer Rare Bladder & bowel Early
Cauda equina Asymmetrical Absent Common Common Common Early / late depending on root involvement Epiconus
4.
Absent Paralysis of LL
muscles Short description of some CNS diseases Paraplegia Most common causes 2. 3. 4. 5. 6. Trauma (parasagittal region), Tumor TB Thrombosis- of sagittal sinus, ACA (uni/ bilat) Transeverse myelitis
Quadriplegia Types spastic & flaccid Spastic Flaccid Cerebral palsy, Cerebral anoxia, Brainstem SOL, Syringobulbia, MND, demyelinating disease, Craniovertebral anomaly, Fracture of cervical spine , cervical spondylosis, cervical cord tumors, hematomyelia Poliomyelitis, Polyneuropathy, Guilliane Barre Syndrome (GBS), Porphyria, diphtheria, Infectious mononucleosis, Polymyositis, Periodic paralysis Features Commonest type UMN & LMN signs (pyramidal tract & antr. Horn) LMN signs (antr. Horn) Onset > 50year, gradually progressive UMN signs(pyramidal tract) LMN signs (cranial nerve nuclei) UMN signs (cortico bulbar fibres) Weakness of facial & bulbar muscles Neural deafness Abnormal GTT Prognosis (years) 5 10 3 2 2 (Good longevity)
Motor neuron diseases Disease (% of total cases) Amyotrophic Lateral Sclerosis ALS (50 %) Progressive Muscular atrophy (10%) Primary lat. Sclerosis (5%) Progressive Bulbar palsy (25%) Pseudo Bulbar Palsy (10%) Madras MND (More males affected , 10-30 years)
Peripheral neuropathy Classification 1. based on mode of onset Acute <1 week Sub acute Chronic 1 week 1 month > 1 month
GBS, Porphyria, Toxins (vincristine), pan- autonomic DM, Amyloidosis, Paraneoplastic HIV Neuropathy, Hereditary Sensory neuropathy
2. based on system affected Motor GBS, Porphyria, Diptheria, Infectious mononucleosis, Lupus
Sensory Mixed
Toxic- Pb, Al, Thallium, Triortho cresyl phosphate, Opioid Idiopathic Toxic Cisplatinum, Vit B12, Enitro-furantoin, Pyridoxine DM, Amyloidosis, Leprosy, HIV Drugs- Alcohol, Dapsone, Phenytoin, INH, EMB, amiodarone Metal poison Au, Hg, Pb Industrial chemicals Hereditary neuropathy, CRF, PBS
3. symmetrical / asymmetrical 4. recurrent GBS, Porphyria, CPD, Beri-beri GBS (Guillian Barre Syndrome) Progressive weakness of arms & legs Areflexia, Hypertonia, Plantar flexor Usually Symmetrical Mild sensory symptoms /signs No bladder involvement Myasthenia Gravis Onset - Insidious / sub acute Ptosis- Uni / bi lateral; Pupils- spared Palatal palsy/ weakness Proximal weakness, weakness more in evening & disappears at rest Poly myositis Myopathies Fever, muscle ache & tenderness Symmetrical weakness of proximal & trunk muscles Ocular & facial muscles spared Pharyngeal muscles may be involved Reflexes usually depressed ECG changes Arrhythmia may occur Congenital & familial Onset gradual & slowly progressive Symmetrically bilat. Selective group of muscles wasted / hypertrophied Kyphoscoliosis No sensory / bladder disturbances Description of few types of myopathies Duchennes Inheritance Onset Distribution Sex linked recessive 3 years Proximal > distal Becker Sex linked recessive 15-20 years Same as Facioascapulohumera l Autosomal dominant 7-25 years Face, arms & scapula Limb girdle Autosomal recessive 20-30 years Shoulder &