Nervous System

Diseases &
Disorders
Introduction to Human Diseases
Nervous System Anatomy
Divisions of the nervous system:
Central Nervous System
Brain and spinal cord
Peripheral Nervous System
Peripheral nerves
Autonomic Nervous System
Symphathetic nervous system
Parasympathetic nervous system
CNS anatomy
Major parts of the brain
Cerebrum
Cognitive center (thought, memory)
Level of consciousness
Cerebellum
Center of balance & coordination
Brain stem
Center of automatic functions
Blood pressure, respiration, etc.
How Microbes Enter the Nervous System
Skull or backbone fractures
Medical procedures
Along peripheral nerves
Blood or lymph
The Nervous System
Figure 22.1
Microbial Diseases of the
Nervous System
Bacteria can grow in the cerebrospinal fluid in the subarachnoid space of the
CNS.
The blood brain barrier (capillaries) prevents passage of some materials (such
as antimicrobial drugs) into
the CNS.
Meningitis: Inflammation of meninges.
Encephalitis: Inflammation of the brain.
The Meninges and Cerebrospinal Fluid
Figure 22.2
Bacterial Meningitis
Fever, headache, and stiff neck
Followed by nausea and vomiting
May progress to convulsions and coma
Diagnosis by Gram stain or latex agglutination of CSF
Treated with cephalosporins
Bacterial Meningitis
Figure 22.3
Haemophilus influenzae
Meningitis
Occurs mostly in children (6 months to 4 years).
Gram-negative aerobic bacteria, normal throat
microbiota
Capsule antigen type b
Prevented by Hib vaccine
Neisseria Meningitis,
Meningococcal Meningitis
N. meningitidis
Gram-negative aerobic cocci, capsule
10% of people are healthy nasopharyngeal carriers
Begins as throat infection, rash
Serotype B is most common in the United States
Vaccination recommended for college students.
Neisseria Meningitis,
Meningococcal Meningitis
Figure 22.4
Streptococcus pneumoniae Meningitis,
Pneumococcal Meningitis
Gram-positive diplococci
70% of people are healthy nasopharyngeal carriers
Most common in children (1 month to 4 years)
Mortality: 30% in children, 80% in elderly
Prevented by vaccination
Listeriosis
Listeria monocytogenes
Gram-negative aerobic rod
Usually foodborne; it can be
transmitted to fetus.
Reproduce in phagocytes.
Figure 22.5
Tetanus
Clostridium tetani
Gram-positive, endospore-forming, obligate anaerobe
Grows in deep wounds.
Tetanospasmin released from dead cells blocks relaxation pathway in muscles.
Prevention by vaccination with tetanus toxoid (DTP) and booster (dT).
Treatment with tetanus immune globulin.
Tetanus
Figure 22.6
Botulism
Clostridium botulinum
Gram-positive, endospore-forming, obligate anaerobe
Intoxication comes from ingesting botulinal toxin.
Botulinal toxin blocks release of neurotransmitter causing flaccid paralysis.
Prevention
Proper canning
Nitrites prevent endospore germination in sausages.
Botulism
Treatment: Supportive care and antitoxin.
Infant botulism results from C. botulinum growing
in intestines.
Wound botulism results from growth of C. botulinum
in wounds.
Botulism
Type A
60-70% fatality
Found in CA, WA, CO, OR, NM.
Type B
25% fatality
Europe and eastern United States
Type E
Found in marine and lake sediments
Pacific Northwest, Alaska, Great Lakes area
Leprosy
Mycobacterium leprae
Acid-fast rod that grows best at 30C.
Grows in peripheral nerves and skin cells.
Transmission requires prolonged contact with an infected person.
Tuberculoid (neural) form: Loss of sensation in skin areas; positive lepromin
test
Lepromatous (progressive) form: Disfiguring nodules over body; negative
lepromin test
Leprosy
Figure 22.9
Poliomyelitis
Poliovirus
Transmitted by ingestion.
Initial symptoms: Sore throat and nausea
Viremia may occur; if persistent, virus can enter the CNS; destruction of motor
cells and paralysis occurs in <1% of cases.
Prevention is by vaccination (enhanced-inactivated polio vaccine).
Poliomyelitis
First vaccine made in Canada by Connaught Medical
Research Laboratories
Poliomyelitis
Figure 22.11
Rabies Virus (Rhabdovirus)
Transmitted by animal bite.
Virus multiplies in skeletal muscles, then brain cells causing
encephalitis.
Initial symptoms may include muscle spasms of the mouth and
pharynx and hydrophobia.
Furious rabies: Animals are restless then highly excitable.
Paralytic rabies: Animals seem unaware of surroundings.
Preexposure prophylaxis: Infection of human diploid cells
vaccine.
Postexposure treatment: Vaccine plus immune globulin.
Rabies Virus (Rhabdovirus)
Figure 22.12
Rabies Virus (Rhabdovirus)
Figure 22.12
Rabies Virus (Rhabdovirus)
Figure 22.12
Rabies Virus (Rhabdovirus)
Figure 22.12
Rabies Virus (Rhabdovirus)
Figure 22.12
Rabies Virus (Rhabdovirus)
Figure 22.12
Rabies Virus (Rhabdovirus)
Figure 22.12
Negri Bodies in Neurons of Brain
Rabies Virus (Rhabdovirus)
Figure 22.13
Arboviral Encephalitis
Arboviruses are arthropod-borne
viruses that belong to several
families.
Prevention is by controlling
mosquitoes.
Figure 22.14
Arboviral Encephalitis
Encephalitis Reservoir Mosquito vector U.S. distribution
Western equine Birds, horses Culex

Eastern equine Birds, horses Aedes, Culiseta

St. Louis Birds Culex


California
Small
mammals
Aedes
West Nile
Birds,
mammals
Culex, Aedes
Cryptococcus Neoformans Meningitis
(Cryptococcosis)
Figure 22.15
Cryptococcus Neoformans Meningitis
(Cryptococcosis)
Soil fungus associated with pigeon and chicken droppings.
Transmitted by the respiratory route; spreads through blood to the CNS.
Mortality up to 30%.
Treatment: Amphotericin B and flucytosine.
African Trypanosomiasis
Trypanosoma brucei gambiense infection is chronic
(2 to 4 years).
T. b. rhodesiense infection is more acute (few months).
Transmitted from animals to humans by tsetse fly.
Prevention: Elimination of the vector.
Treatment: Eflornithine blocks an enzyme necessary
for the parasite.
Parasite evades the antibodies through antigenic variation.
African Trypanosomiasis
Figure 22.16
Naegleria fowleri
Protozoan infects nasal
mucosa from swimming
water.
Figure 22.17
Transmissible Spongiform
Encephalopathies
Caused by prions
Sheep scrapie
Creutzfeldt-Jakob disease
Kuru
Bovine spongiform encephalopathy
Transmitted by ingestion or transplant or inherited.
Chronic and fatal
Transmissible Spongiform
Encephalopathies
Figure 22.18
Meninges
Dura
Outermost layer
Thick, fibrous
Arachnoid
Thin, filmy like a spiderweb
Pia
Thin, vascular layer of loose connective
tissue, inseparable from the outer brain
surface
Headaches
Due to irritation, inflammation of any pain-sensitive
structure
Brain itself is not a source of headache
Diagnostic procedures and testing:
Physical exam (PE), CT scan, LP (lumbar puncture or spinal
tap), EEG, routine x-rays, MRA, MRI and more
Migraine Headache
Recurrent, usually severe headache of vascular origin
More common in females (3 to 1)
Family history in 70-80%
Onset common in adolescence or early adulthood
80% have first migraine by 30YOA
Usually become less frequent with age
Migraine Headache
10-20% US population have migraines
2
nd
most common cause of HA in US
Muscle tension HA is #1
S/S:
Intense throbbing headache, uni-or
bilateral
Nausea, vomiting, dizziness, tinnitus
Visual disturbances
With or without aura (classical vs.
common)
Duration from 4-72 hours
Migraine Headache
Etiology:
Vasoactive chemicals (peptides) in brain (serotonin and
dopamine)
Stimulate inflammatory cascade
This causes vasodilation
Serotonin appears to be most important factor
Subarachnoid Headache
Due to a subarachnoid hemorrhage
80% SAH due to ruptured intracranial aneurysm
Berry aneurysm, saccular, acquired due to hemodynamic
stress at bifurcations
About 28,000 SAH/yr in US
Mean age is 50 YOA
Accounts for 6-8% strokes in US
Subarachnoid Headache
Most common etiology of non-traumatic intracranial
hemorrhages
S/S: worst headache of my life
Rapid onset, neck stiffness (nuchal rigidity), low back
pain, photophobia, nausea & vomiting, seizures in 10-25%
10% mortality immediately
Up to 60% mortality in 1
st
month
Head Trauma
#1 cause of trauma deaths in US
Many possible mechanisms of injury:
Falls
Motor vehicle crashes
Assaults
Shaken Baby Syndrome
Vigorous episode of shaking the baby by the arms, torso,
or chest
Most victims less than 3 years of age
Produces characteristic injury pattern:
Neurological injury
Mental retardation, seizures, hearing loss
Intracranial hemorrhage or edema, coma
Retinal hemorrhages
Shaken Baby Syndrome
One version of child abuse
First described in 1946
Part of overall pattern of child abuse:
Multiple fractures
Bruises in all stages of healing, often axial
Often abuse from non-biological parent or caregiver
Shaken Baby Syndrome
Brain injuries:
Hemorrhage (SDH)
Cerebral edema & raised pressure
More common
Prognosis:
1/3
rd
mortality
1/3
rd
with permanent neurological disabilities
Epidural and Subdural
Hematomas
Epidural Hematoma
Hemorrhage above the dura layer
Arterial bleeding
Parietal skull fx, middle meningeal artery
lucid interval
Subdural Hematoma
Below the dura
Venous bleeding
More common
EDH & SDH
S/S:
Altered level of consciousness (coma or lethargy),
headache
Hemiparesis (right or left)
Unilateral dilated pupil
Treatment:
Neurosurgical drainage of the hematoma
Cerebral Concussion
Disruption of brain function without actual physical
brain injury
Typically follows a blow to the head
Often, not always, has loss of consciousness
S/S: headache, nausea, dizziness, amnesias,
disorientation, vertigo, photophobia
Concussion
Testing is done to rule out other injuries
Treatment: supportive, observation
Cerebral Contusion
Bruising of the brain tissue
Cerebral edema around the contusion is common
Mechanism: a blow to the head, usually localized force
S/S: headache, variable loss of consciousness, variation
from concussion-like symptoms to coma with
hemiparesis
Cerebral Contusion
Testing: CT scan to examine extent of brain injury &
possible brain herniation
Treatment: variable
Monitoring of ICP, meds to decrease brain swelling, usually
surgery is not helpful, other supportive care in ICU
Disorders that cause paralysis
Hemiplegia
Loss of muscle control & sensation on one side of the body
(L or R)
Paraplegia
Loss of muscle control & sensation on the lower part of
the trunk and lower extremities
Quadriplegia
Paralysis of all four extremities
Hemiplegia
Etiology: most frequently stroke
Also intracranial tumor or hemorrhage
Other S/S: weakness of half of the face, aphasia,
agnosia, apraxia, agraphia, alexia, etc.
Paraplegia & Quadriplegia
Etiology: most commonly spinal cord injuries due to
trauma
Other S/S: loss of bowel & bladder control, sexual
dysfunction
S/S of quadriplegia also include:
Low blood pressure and pulse
Variable loss of respiratory control
CNS Infections
Acute Bacterial Meningitis
Acute inflammation & infection of the CSF & the meninges
Etiology: bacterial, viral, or fungal
Source may be spread from blood or nasopharynx
Most severe type = bacterial
Most common bacteria involved are Strep pneumoniae &
Neisseria meningitidis
Acute Bacterial Meningitis
S/S: Headache, nausea, vomiting, fever, seizure,
nuchal rigidity, drowsiness, coma
Testing: lumbar puncture makes the diagnosis
Characteristic rash with Neisseria m.
Treatment: antibiotics or antifungals and supportive
care
Brain Abscesses
Localized collection of pus
Most commonly in:
Cerebellum
Frontal or temporal lobes of cerebrum
Etiology: spread from some other nearby or distant
infection
Brain abscesses
S/S: depending on location, may resemble hemorrhage
or CVA
Treatment:
Antibiotics
Surgical drainage of the abscess
Peripheral Nerve Diseases
Peripheral neuritis (peripheral neuropathy)
Noninflammatory degenerative disease of nerve supplying
the distal extremities
Commonly males, 30-50 YOA
Etiology: numerous, including alcohol, heavy metal, drug,
poisons, TB & infections, diabetes, lupus, nutritional, etc
Peripheral neuropathy
S/S: muscle weakness, paresthesias, pain, tenderness,
atrophy, loss of reflexes
Gradual in onset usually
Diagnostics: EMG
Treatment: varies with cause
Bells Palsy
Disorder of the facial nerve (cranial nerve VII)
Unilateral paralysis/paresis of the facial muscles
Usually transient
Typically in patients 20-60 YOA
Bells Palsy
Etiology: idiopathic technically
Viral etiology is strongly suspected
Autoimmune, ischemic, etc
S/S: facial asymmetry, drooping mouth, drooling,
incomplete closure of eye (Bells phenomenon)
Treatment: antiviral drugs and steroids usually
Cerebrovascular Accident
(CVA)
Focal neurological impairment due to lack of blood
supply to an area of the brain lasting more than 24
hours
Clinical condition is called stroke
2 types of stroke:
Ischemic: embolic or thrombotic, 75% CVAs
Hemorrhagic: 25% CVAs
CVA
Risk factors: same as for cardiovascular disease,
smoking, HTN, hyperlipidemia, diabetes, etc. TIAs,
oral contraceptives
S/S: hemiplegia or hemiparesis, dysphagia, speech
impairment, diplopia & loss of visual fields, lack of
coordination, confusion, sensory impairment
CVA
Diagnostics: CT scan to rule out hemorrhage or tumor
Treatment:
Thrombolytic medicines Clot busters if stroke symptoms
of 3 hrs or less
Supportive care, nutritional, PT & OT
Transient Ischemic Attacks
(TIA)
Focal neurological impairment due to transient lack of
blood supply to an area of the brain, lasting less than 24
hrs.
TIAs indicate increased risk of CVA in the future
Resolve completely
Often 20-40 minutes in duration
Transient Ischemic Attacks
Symptoms: most commonly upper extremity
clumsiness, weakness, paresthesias, visual field cuts
(curtain-like)
Treatment: surgery for carotid plaque, anticoagulants
Epilepsy
Chronic brain disorder characterized
by seizures (abnormal, rapid, intense
neuronal discharge)
Types of seizures:
Generalized (gran mal)
Involve entire body, unconscious
Partial: part of the body is involve,
conscious
Petit mal: momentary, frequent
unconscious episodes
Epilepsy
Etiology: mostly idiopathic
Trauma, tumors, CNS infection, drugs & toxins, etc
S/S: in classic generalized seizure
Aura, tonic, clonic, post-ictal phases
Respiratory insufficiency & cyanosis
Incontinence of bowel & bladder
Epilepsy
Treatment: acute and long-term anticonvulsant meds
Degenerative Neurological
Diseases
Alzheimers Disease
Fatal, long-term disease of the brain
Characterized by neurofibrillary tangles and senile
plaques
Gradual & permanent deterioration of memory, judgment,
cognitive and verbal skills
Geriatric disorder in general
Alzheimers Disease
Etiology: unknown
S/S: previous section, end-stage is complete
inattention to self
Diagnosis of exclusion
Treatment: some meds slow progression, supportive
care of patient and family
Parkinsons Disease
Movement disorder characterized by muscle rigidity &
tremors
Usually males over 65 YOA
Etiology: unknown, related to dopamine deficiency in
brain
Parkinsons Disease
S/S: bradykinesia, involuntary tremors, muscular
rigidity
Treatment: dopamine replacement meds, PT,
supportive
Multiple Sclerosis (MS)
Chronic, progressive, demyelinating autoimmune
disorder
Makes antibodies to myelin
Females (2:1)
Occurs during adult years, not geriatric
About 25,000 new cases/yr in US
MS
S/S: variable, may last hours to weeks
Classically: eye symptoms, paresthesias, paralysis, mood
swings, balance problems, often sudden onset with
relapses
Treatment: beta-interferon products, corticosteroids,
glatiramen acetate (synthetic myelin protein)
Usual cause of death is due to chronic disability issues
Amyotrophic Lateral Sclerosis
(ALS)
Adult-onset motor neuron disease
Progressive degeneration & loss of motor function
Called Lou Gehrigs Disease (1941)
Males between 50-60 YOA
About 6/100,000 adult population in US
25,000-30,000 cases total in US
ALS
S/S: progressive muscle weakness, atrophy, and
fasciculations (twitching)
Eventual involvement of mouth, respiratory muscles, end-
stage is complete paralysis
75-80% begin with limb involvement
Slurred speech, hoarseness, dysphagia, tripping &
stumbling, trouble with usual daily activities
ALS
Sporadic form (nonhereditary) 90-95% cases
Inherited version is autosomal dominant
Mean time till ventilator dependent or death: 2-4 years
May survive 5-10 years on ventilator
Cancers of the CNS
Primary brain tumors
Benign or malignant
Most pediatric brain tumors are primary
Examples are astrocytoma, glioblastoma
Secondary brain tumors
Metastases from other sites
Most common tumors of the brain