CM EXAM 4 PEER Abigail Wilhite

TUTORING REVIEW
Fever:
Lecture 35: Fever & Altered States of Consciousness
 Identify common clinical presentations
• Fever= rectal temp >100.4°F or 38°C  use rectal temp for children <5yo; rectal temp is most accurate
• Hyperpyrexia >100.4°F or 40°C
• Infants: no symptoms, irritability, lethargy, poor feeding, crying, tachycardia (every 1°C of fever of infectious cause
will raise HR by 10BPM), tachypnea, sleep changes
• Older children: no symptoms, body aches, headaches, difficulty sleeping, poor appetite, feeling hot/cold, tachycardia,
tachypnea, fatigue/malaise, chills/shivering, irritability/altered mental status, hallucinations
 Determine when treatment is needed
• Meds: acetaminophen (every 4-6 hrs dosing); ibuprophen (every 6-8 hrs); do NOT give aspirin to peds population 
Reyes sundrome (encephalopathy)
• Symptomatic measures: hydration, cool compresses/tepid baths, TLC
• You want to treat the cause, fever is just a symptom.; not necessary to treat fever in a self-limiting illness in a healthy
child
 Know the advantages and disadvantages of treatment
• Advantages:
• For treatment: child feels better, gives parents reassurance that they are helping, decrease risk of dehydration, may
help to prevent decompensation in child with heart/lung disease
• No treatment: fever is natural infection fighter, it slows the growth of viruses and bacteria; aids the body in
producing acute phase reactants & other immune defenses
• Disadvantages:
• Treatment: potential overdose, allergic rxn, side effects (ibuprophen- upset stomach, GI bleed; aspirin- Reyes)
• No treatment: febrile fever, dehydration, temp rising to dangerous level
• High fever better tolerated in a 5 yo vs a newborn
• Eval: take careful H&P (if pulse ox ~95 then it is signifying something is going on) and under labs *CSF studies & cx
Meningitis & Encephalitis:
 Recognize the signs and symptoms of meningitis in the pediatric population *a lot of symptoms overlap w/ fever
 Neonates & infants:
 Fever, irritability, lethargy, poor feeding, crying, sleep problems, respiratory symptoms/apnea, hypotonia, seizures, bulging fontanel, vomiting
 Older children:
 Fever/chills, body aches, headaches, sleeping problems, poor appetite, fatigue/malaise, irritability/altered mental status, nuchal rigidity
(Kernig/Brudzinski’s), photophobia, hearing loss, vomiting, seizures
 Distinguish the most common types of bacterial meningitis and the empiric treatment for each *TABLE
 Group B –no vaccine; Listeria –no vaccine; E. coli –no vaccine; Neisseria –vaccine not started until 11yo; Strep pneumo –vaccine at 2 months; H. influ –
vaccine 2 months *98% decline since intro
 Aseptic meningitis: virus –enterovirus, adenovirus, Herpes fam; seasonal (summer/spring); mimic bacterial symptoms (HA, fever, N/V, irritability); benign
& self-limiting except for HSV (tx w/ acyclovir)
 Identify the differences in meningitis and encephalitis including the presentation, infectious agents and CSF findings
 Encephalitis: Acute inflammatory process involving the parenchymal brain tissue; focal neuro signs (focal infxn), altered LOC/lethargy/change in behavior
(diffuse infxn), fever & HA are 1st symptoms, seizures *late symptom, self-limited disease *↓
 Neonates: fever, poor feeding, irritability, decreased perfusion, seizures  *same symptoms for meningoencephalitis
 Enterovirus: “hand, foot & mouth enterovirus”; direct person to person spread, Coxsackies A & B, echovirus
 Epstein barr virus: adolescents/young adults, infectious mono; present with focal symptoms; dx with EBV PCR of CSF; tx supportive
 Arbovirus: from mosquitoes & ticks; summer months; most common in US West Nile virus, LaCrosse, St. Loids & California encephalitis viruses
 Herpes simplex virus: dx w/ HSV PCR of CSF  tx w/ acyclovir decreasing mortality from 70% to 19%
 HSV-1 –severe, sporadic encephalitis in children & adults; brain involvement focal (temporal lobe), progress to coma & death if not tx
 HSV-2 –severe with diffuse brain involvement in neonates who usually contract from mother during delivery
Serious Bacterial Infection Septic Workup Age Under 1 month 1-2 months > 3 months
Asses the risk CBC, CRP
Bacterial Maternal flora: Strep pneumoniae Strep pneumoniae
Pneumonia Chest X-ray Agents Group B streptococcus N. Meningitidis N. Meningitidis
Bacteremia Blood cultures Listeria monocytogenes Group B strep (late onset) H. Influenzae type B
E. coli
Meningitis/encephalitis Lumbar puncture (CSF studies/culture)
Empiric Ampicillin & Gentamicin Vanc & cephalosporin Vanc & caphalosporin
Urinary tract infection UA & urine culture Antibiotics OR
Bacterial enteritis Stool culture Ampicillin & Cefotaxime
Febrile seizure:
 Recognize the classic presentation of simple febrile seizures most common type of seizure in childhood
 Generalized, tonic-clonic; <15 min duration, no recurrence in 24 hrs; no neuro abnormality
 Associated with temp ≥100.4 within the 1st 24 hours of illness; btwn 6 & 60 months; NOT result of CNS infxn or metabolic
imbalance
 Associated with common respiratory viral infxns & immunizations (DTP, MMR, MMRV)
 Formulate the work-up for suspected febrile seizure
 Detailed Hx pre-seizure, careful PE ***NO routine lab workup  not indicated: lumbar puncture (unless infx suspected, pre-tx
antibiotics), EEG, cranial imaging
Recognize the clinical presentation of acute disseminated encephalomyelitis (ADEM)
• Central (CNS) inflammatory & demyelinating disorder of childhood (5-8yo)
• Typically occurring after virus or rabies vaccine; benign self-limiting; tx w/ supportive care recovers in 6 months
• Fatique, lethargy, fever, HA, vomiting, CN abnormalities, altered LOC, ataxia, seizure, meningeal signs

Lumbar puncture: definitive dx made by eval of CSF  opening pressure, fluid color/clarity/turbidity, gram stain, CSF studies (WBC, RBC, protein,
glucose, PCR for viral), bacterial cx (24 hrs) **always do CT first to make sure ICP isn’t too high

CSF findings Bacterial Meningitis Aseptic meningitis Encephalitis

WBC   
glucose  normal normal
protein   
culture positive negative Likely negative
Opening pressure  Normal normal
 Lecture 36: Altered Mental Status
Consciousness=arousal + cognition  reticular activating system + cerebral cortex

Coma/Unconsciousness

 Confusion but NOT

coma/unconsciousness

DDx for AMS  AEIOU-TIPS* notes

Delirium: alteration in cognition + arousal but is

Prior Probability:
reversible
Infant
 Infection
 Trauma / abuse
 Metabolic

Child
 Toxic ingestion

Adolescent / Young Adult

 Toxic ingestion
 Recreational drug use
 Trauma

Simultaneous assessment with initial diagnostic & Elderly – AMS is common

stabilization procedure 

Medication related (perhaps 1/3)
Infection  UTI***
DON’T=dextrose-oxygen-Narcan-thiamin PE is key to determining if etiology is structural or  Metabolic disturbance

DON’T is both therapeutic & diagnostic metabolic/systemic  Trauma

Blood sugar test is 1st test for ALL patients w/ AMS Key point: focal neurologic deficit = structural etiology
 Altered
Metabolic Structural Cause Mental
Status
Pupils Equal, reactive. May be slow Unilateral dilated pupil + non- Suggests cerebralVital
herniation & is
or abnormal size but reactive = structural cause Signs
neurosurgical emergency
ABCD
Head-to-Toe Neurologic Exam
symmetric (asymmetry) Airway (Pupils, strength,
Exam
Breathing CN etc)
Posturing If present will be symmetric1 Asymmetry diagnostic of Circulation
- decorticate structural lesion Disability (GCS)
- decerebrate DON’T*
Evidence of YES Any Focal
Eye Movements Usually normal & symmetric2 Abnormal / asymmetric Head Trauma Deficit Found

* Diagnostic &
Doll’s Eye Test Structural Cause Likely
Therapeutic
Immediate Head CT
NO NO
Normal Abnormal
Negative

Metabolic Coma –
Non-drug induced
Pupils
Non contrast CT of brain is the study of Metabolic / Toxic Evaluation
Electrolytes / Hepatic function studies
choice if structural cause is suspected CBC
Equal Size Unequal size CXR if pneumonia suspected
Reactive Non reactive UA
ECG and cardiac enzymes
After DON’T & initial stabilization, tx is ETOH / Tox studies
TSH
Drug Toxicity Structural lesion
involving brainstem directed at specific cause of AMS Coagulation studies
ABG, co-oximetry
LP with CSF if indicated
Ammonia
EEG if hx of seizure
Head CT if concerning historical or physical Features
Additional tests based on history and physical
 Lower motor neuron syndrome Upper motor neuron syndrome
Lecture 37: Disorders of motor neuron, root plexus & peripheral nerves Flaccid weakness Spastic weakness
Decreased tone Increased tone
Increased deep tendon reflexes
Decreased deep tendon reflexes
And Babinski’s sign
Lower motor neuron syndrome: results from damage to motor neuron, nerve root/plexus, and peripheral
Upper motor neuron syndrome: results from damage to the descending motor pathways Profound muscle atrophy Minimal muscle atrophy

Disorders affecting motor neurons: Fasciculations present Fasciculations absent

• Anterior Horn Cell Syndrome: weakness, atrophy, fasciculation, reduced muscle tone, DTRs reduced/absent, NO sensory changes
• Infantile progressive muscular atrophy of Werdnig-Hoffman; intermediate spinal muscular atrophy; Kugelberg-Welander disease; progressive spinal
muscular atrophy in motor neuron disease
• ALS: degenerative changes in anterior horn cell & corticospinal tract; diffuse lower motor neuron signs (atrophy, paresis, fasciculations); upper neuron signs
(paresis, spasticity, Babinski’s)
• Slightly more common in men, onset in 60s, life expectancy 3-5yrs, progressive, painless weakness/atrophy w/ LMN signs early on; spares the eyes &
bladder **tongue/hand atrophy
Radiculopathies:
Root Disease: radicular (dermatomal) pain is sharp & well localized, pain described as sharp, stabbing, electric & typically shoots/radiated down limb; pain is increased
by movement, coughing, sneezing, Valsalva maneuver
• Motor: weakness/atrophy of affected segment (myotome); fasciculation may occur in myotome; reflexes are decreased/absent if appropriate root affected
• Causes: trauma, zoster infection, Lyme disease, diabetes, tumors  intervertebral foramen may be compromised by herniated disk fragments, Ca deposits,
hypertrophied ligaments; infections may affect roots or mixed nerve
• C5: derm- sensory change on lateral upper arm; myo- motor weakness in either the levator scap, rhomboids, supra/infraspinatus, delts, biceps, or
brachioradialis; DTRs- either in biceps/brachioradialis reflexes
• C6: derm- senseory on lateral forearm, lateral hand & 1st/2nd digits; myo- weakness in biceps/pronator teres/flexor carpi radialis/brachioradialis/extensor
carpi radialis; DTRs: biceps/brachioradialis
• C7: derm- 3rd & 4th digits; myo- weakness of triceps/pronator teres/ flexor carpi radialis/extensor carpi radialis; DTRs- triceps maybe
• C8: derm- medial forearm/hand & 5th digit; myo-flexor pollicis longus/opens pollicis/abductor pollicis brevis/all lumbricals/abductor digiti minimi/flexor
& extensor carpi ulnaris; DTRs- triceps maybe, finger flexion
• L4: derm- knee & medial thigh; myo- motor weakness of quads/sartorius/tibialis ant; DTRs- patellar tendon
• L5: derm- lateral thigh/dorsomedial foot/large toe; myo- glut med & min/tibialis ant/plantar inversion & eversion/extensor hallucis longus; DTRs- none
• S1: derm- 5th digit/lateral foot/most sole; myo- knee flexion(biceps femoris)/gastric/soleus/toe flexors; DTRs- Achilles tendon
Plexopathy: difficult to localize due to complexity of plexus; **deficit involving more than one spinal/peripheral nerve w/ brachial plex being most common
• Motor or sensory; distal weakness; asymmetric/symmetric weakness; denervation changes;
• Brachial plexopathies: trauma most common; vaccine induced, post-radiation (months-years after), infectious (normally viral), mass lesions
(metastatic from breast/lung)
• Classical
• Erb-Duchenne syndrome: seen after traumatic separation of head & shoulder at birth, heavy backpacks; lesion at 5 th & 6th cervical
roots/upper tunk
• Waiter’s tip; arm adducted/internally rotated, forearm pronated, wrist flexed, finger function & grasp reflex are normal but
biceps reflex is absent on affected side
• Dejerine Klumpke syndrome: after trauma, surgery, mass lesions; lesion at 8th cervical & 1st thoracic roots/medial cord
• Paresis in wrist/finger flexors & hand intrinsic muscles; sensory changes on medial upper arm, medial forearm & ulnar aspect
of hand; triceps reflex may be reduced; finger flexion reflex is absent/reduced
Peripheral neuropathy: diabetes is most common cause
Diabetic neuropathy: middle aged w/ type 2; most obese & sedentary
• Types: small fiber, large fiber, CIDP, autonomic, proximal diabetic neuropathy
• Prone to compressive & cranial neuropathies; demyelination most common path process though axonal & dorsal root ganglion degeneration
occur relatively early in course of some patients
• Diabetic mononeuropathy: compressive neuropathies carpal tunnel, cubital tunnel, peroneal nerve entrapment, radial nerve injury; cranial
neuropathies facial nerve (Bell’s Palsy), trigeminal/glossopharyngeal neuralgia
• Diabetic polyneuropathies:
• Small fiber: distal, symmetrical, chronic, progressive with pain & autonomic disturbance without weakness & without sensory loss early on
• Large fiber: distal, symmetrical, chronic, progressive with pain, weakness, areflexia, & distal sensory loss
• Autonomic: distal or generalized, subacute to chronic, autonomic dysfunction varying from reddened skin to gastroparesis to postural
hypotension
Guilliain-Barre syndrome: “AIDP” common form of inflammatory autoimmune neuropathy
• Classic form: demyelination in peripheral nerve, @ junction of dorsal & ventral roots which may spread further along the course of nerve
• Rapidly progressive ascending polyneuropathy beginning either in feet/hands and moving up extremities
• Areflexic early on; sensory symptoms min; automimic dysfunction may be profound; respiratory distress life-threatening; following viral syndrome or
GI infection w/ Campylobacter jejuni
• CSF: elevated protein w/ normal cell count “albumio-cytologic dissociation”; dx: LP, NCV, EMG, serology studies w/ infection syndromes such as
HIV-1
• Tx: respiratory support; plasmapheresis, IVIg
CIDP: chronic, progressive demyelinating neuropathy where axons eventually degenerate
• Progressive sensory loss/disturbance, weakness, atrophy, autonomic changes often requiring assistance for ADL’s
• May plateau but improvement is seldom complete despite therapy; recovery rare
• Tx: immunomodulators  prednisone, IVIg, plasmapheresis, chemo
• Seen in many disorders, not just diabetes but clinical course is essentially the same**
Alcoholic neuropathy: common, milder with regards to weakness, sensory changes & pain
• Associated w/ poor general nutrition; associated w/ numerous other neuo deficits; axonal degeneration
AIDS: involvement of peripheral nerves seen ~40% at autopsy
• Axonal, distal, symmetric, sensorimotor neuropathy most common with sensory symptoms predominating
• Inflammatory demyelinating polyneuropathy; linked to autoimmune dysfunction/infection with proximal weakness and minimal sensory changes
• CSF increased protein & WBCs; tx: immunomodulators
• Other: polyradiculopathy, autonomic neuropathy, mononeuropathy multiplex
Charcot-Marie-Tooth: many variations but ALL genetic
• Most slowly progressive, sensorimotor forms that may be associated with severe pain, cerebellar dysfunction or cranial nerve signs & essential tremor
• Present in 1st-3rd decade w/ distal weakness of anterior compartment leading to foot drop, high arches, hammer toes; absent ankle jerks, steppage gait
 Lecture 38: Neurocutaneous Disorders

Tuberous sclerosis: AD or sporadic

• Mutation in either TSC1 (mutation produces hamartin) or TSC2 (mutation prevents production of tuberin)
• Rare genetic disease causing benign tumors to grow in brain, kidneys, heart, eyes, lungs & skin
• Hamartomas- benign tumor-like nodule
• CNS: hamartomas of cortex & ventricular walls; subependymal giant cell tumors
• Cortical tubers (surface), subependymal nodules (ventricular wall), giant-cell astrocytomas
• Skin lesions: adenoma sebaceous, hypomelanotic skin macules (ash leaf spots, shagreen patches, subungual fibromas)
• Visceral lesions: cardiac & renal tumors
• Clinical triad: mental retardation, epilepsy, skin lesions
• Tx: control the epilepsy, correct hydrocephalus, monitor other organs Subependymal nodules

Shagreen
patches
Subungual
fibromas

Adenoma Ash leaf spots

sebaceous Cortical tubers
 Neurofibromatosis:
• NF1: skin changes & deformed bones usually at birth
• “von Recklinghausen disease”
• Mutation in gene on chromosome 17 encoding protein neurofibromin; AD but up to 50% are spontanrous
• Skin findings: NIH criteria in notes
• Neurofibromas: most common benign tumor of NF1; tumors composed of schwann cells, fibroblasts, mast cells, vascular
component and develop along a nerve
• Café au lait spots: 6 or more spots > 1.5cm in diameter
• Axillary freckling: either axillary or inguinal freckling develop during puberty, common
• Lisch nodules on the iris
• Learning disabilities are present in ~50% of patients
• NF2: hearing loss, ringing in ears & poor balance starts as teen
• Chromosome 22; merlin gene AD
• Symmetrical vestibular schwannomas (acoustic neuromas) hallmark  causing hearling loss, tinnitus & problems w/ balance
• Some patients also develop cataracts in 1 or both eyes often in childhood
• Schwannomatosis: intense pain, rare

Café au lait spots Lisch nodules

NF2
NF1 Bilateral
vestibular
Axillary freckling schwannomas
neurofibromas
Sturge-Weber disease:
• “encephalotrigeminal angiomatosis”
• Rare congenital vascular disorder, facial capillary malformation (port wine stain) & associated capillary-venous
malformations affecting brain & eye
• Symptoms: port wine stains, seizures (other neurologic complications associated w/ angioma of leptomeninges),
glaucoma, learning disability
• Port wine stain: common, normally in area innervated by trigeminal n.; normally unilateral and can be on
trunk or extremities
• Seizures (83%): result of leptomeningeal angioma -100% of patients; may be hard to control & patients w/
seizures are more likely to have DD
• Glaucoma: 60%, normally unilateral, can be extremely painful; cause of outbursts in non-verbal patients
• DD 40-50% overall  can have hemiplegia, HA, emotional problems, behavior problems
• Tx: signs & symptpms  AEDs, eye drops/eye surgery for glaucoma, laser therapy for port wine, pT for
paralysis/weakness, brain surgery for seizures
Leptomeningeal angioma

Port wine stain

Von Hippel-Lindau disease:
• Formation of tumors/fluid-filled cysts which may be cancerous appearing during young adulthood
• Mutation in VHL gene AD; tumor suppressor gene; preventing production/abnormal prod. of VHL protein; cant
regulate cell survival/division leading to uncontrolled form. of cysts/tumors
• VHL syndrome type 1: low risk of pheochromocytomas
• VHL syndrome type 2: higher risk of pheochromocytomas, also development of renal cell carcinoma &
hemangioblastoma
• Hemangioblastoma: characteristic; newly formed blood vessels & noncancerous; develop in brain (cerebellum) &
spinal cord causing HA, vomiting, weakness, ataxia; can occur in retina causing vision loss
• Btwn 20-50% w/ VHL develop cysts/serous cystadenomas in the pancreas
• Tx: surgery or radiation therapy; tx growths while they are small and haven’t caused damage yet
Hemangioblastoma
 Lecture 41: Immune-Mediated Neurological Disease
OMAS – Strabismus, twitching (dancing eyes & feet syndrome), often occurs after infant neuroblastoma (originates in adrenal
medulla/retroperitoneal area and typically has spread by time of dx); anti-Hu immunoglobulins (adaptive immune system attempts to kill the
tumor and since both the brain and the tumor express Hu protein it causes an autoimmune condition). Can get post-infectious OMAS as adults.
Tx: immunosuppress with dexamethasone, ACTH, IVIG, benzo, AEDs, rehab

MS – chronic inflammatory, immune mediated disease attacks myelinated CNS axons; AutoAb vs. myelin + IL-12 pro-inflammation + Th17
cells (produces IL-17 recruiting Th1 cells, neutrophils, macs by chemotaxis; may originally come from molecular mimicry from a viral infxn) +
decreased Tregs (decreased Tregs allow inflammation to persist); myelin is lost, oligodendrocytes are destroyed & astrocytes become activated
most often affecting cerebrum, brainstem, spinal cord, optic nerve and breaking down the BBB; dx: repeated clinical attacks + brain MRI + CSF
IgG + EEG

GBS – Ascending symmetrical weakness, diminished reflexes, heart-lung complications (muscles of respiration are affected in 1/3 pf patients &
end up in respiratory failure), most patients make a good recovery, campylobacter or CMV molecular mimicry causing antibody attack of GM1-
GM-2 –recruit complement & macs to cause damages by stripping myelin off spinal roots/peripheral nerves; dx: LP albuminocytologic
dissociation, cauda-equina nerve root enhancement on MRI, nerve conduction studies show nerve root demyelination; tx: respiratory therapy

MG – Weakness of skeletal muscles (commonly affects eyes (ptosis), face (dysphagia), neck (dyspnea)); 30-40s – post-synaptic anti-ACh
receptor antibodies targeting the alpha-subunit of AChR; thymoma can cause the generation of these antibodies; dx: immunofluorescence of
anti-AChR at post-synaptic junction, edrophonium test, chest radiographs for thymic abnormality; tx: AchE inhibitor, IVIG, steroids, surg

LEMS – Weakness of skeletal muscles & decreased tendon reflexes – autoantibodies vs. presynaptic voltage-gated calcium channels (VGCC);
cant release ACh at presynaptic terminal, complement not involved; 40% of patients have SCLC and higher incidence after long term cigarette
smoking; dx: muscle weakness/tenderness, proximal muscles of legs/arms affected, reflexes reduced/absent but are increased after voluntary
contraction of a muscle group*, antibodies VGCC; tx: corticosteroids, IVIG
Lecture 42: Brain Death

Declaring death: it matters for organ procurement, family suffering, societal expense, limitation of resources
• Cessation and irreversibility of cessation of cardiopulmonary function or entire brain (including brainstem) function
• Generally for more than 6 hours  12 when there are no confirmatory tests and longer if uncertainty/children
• Coma exam is absolutely critical; any cortical/brainstem function disqualifies the declaration of brain death & you cant be brain dead if
you are actively seizing
• GCS  decorticate & decerebrate; must have no response to noxious stimuli, motor, verbal or eye opening
• Pupils: CN II & III; pupils must be fix but NOT necessarily dilated and are often mid or sometimes oblong
• Corneal exam: CN V & VII
• Oculocephalic reflex: Doll’s eyes
• Oculovestibular: cold water in the ear causing eyes to move
• Gag reflex: pharynx
• Cough: bronchial suction
• Respiration: no breathing over the ventilator or no breathing at all
• Spinal reflexes: these should still be intact  grasp, withdrawal, complex, Lazarus
• The process: lifeline is notified neuro exam performed determined by qualified expert death packet  death notes
• Other factors: temp (>90), BP-SBP 90, Meds, Seizures, metabolic
• Other tests: CT (no gyri/sulci present), EEG (flat for >30min), Nuclear, CTA (no blood flow above carotids), apnea test (often terminal
event, no breaking >2 minutes w/PaCO2 > 60), angiography (no blood flow above carotids and can be a good indicator) (although the
neuro exam is the best)
 Clinical Presentation Additional Data needed for MS Diagnosis
2+ attacks OR examination suggests 2+ lesions OR None
Lecture 46: Demyelinating Disorders of NS 1 MRI lesion with prior attack
2+ attacks OR examination suggests 1 lesion and 1 Dissemination in space by serial MRIs OR 2+ MRI
lesion on MRI = suggests dissemination in time lesions AND positive CSF OR await another attack in
different site
1 attack OR examination suggests 1 lesion and 2+ Dissemination in time by MRI
lesions on MRI = suggests dissemination in space OR await another attack in different site
1 attack OR examination suggests 1 lesion and 1 Dissemination in time by MRI
lesion on MRI OR second clinical attack
(Clinically Isolated Syndrome, CIS) - AND –
Dissemination in space by serial MRIs OR 2+ MRI
a. Recognize the common clinical manifestations of multiple sclerosis (MS). lesions AND positive CSF
• F>M; Caucasian>other; teens-40s; cumulative neuro impairment over time
• Double vision or vision loss (unilateral), dysarthria, aphasia, depression, difficulties w/ memory, learning, processing, reasoning, motor weakness or
spasticity, pain/numbness, problems with bladder/bowel control, sexual dysfunction, fatigue
• Optic neuritis: inflammation of the optic nerve, loss of vision in the affected eye, decreased color vision, pain w/ movement, slowed on VEP
• Eval: ophthalmologic eval (optic disc pallor OR acute disc edema), pupillary exam (afferent pupillary defect), VEP, optical coherence tomography
(acute edematous, chronic thinning); Tx: IV methylprednisolone (stops inflammation), MUST treat underlying cause (MS or B12 def.)
• Many different causes of optic neuritis  MS, viral infection, B12 deficiency, lupus, neurosyphilis
b. Recall the diagnostic criteria for MS. *table
• MRI-Dissemination in space (enhancing lesion in 2/4 locations), dissemination in time; CSF-inflammatory markers, >2 oligoclonal bands, elevated IgG
index (all compared to serum)
c. Identify common disorders that can mimic MS.
• Stroke, B12 deficiency, Hashimoto’s thyroiditis, progressive leukoencephalopathy
d. Recognize common treatments for relapsing episodes of MS and for MS symptoms.
• Disease tx: interferon beta (keeps t cells out of CNS), glatiramer acetate (makes t cells less aggressive), anti-neoplastic agents (really bad disease, heart
problems contraindicated); other meds sequester t cells in lymph nodes or mark T/B cells for auto-immune destruction
• Symptom tx: pain (NSAIDs, AEDs, PT), Bladder incontinence (cath, meds for freq.), spasticity (baclofen, stretching), fatigue (amantadine, SNRIs,
amphetamines *not FDA approved), cognitive (compensatory strategies, meds not helpful/harmful)
e. Select the appropriate diagnostic testing in the evaluation of these disorders
i. Eval: clinical hx, exam, LP, VEP (optic nerve), SSEP (spinal cord), BAER (brainstem)
Lecture 47: Demyelinating Disorders of the NS II
Identify the causative factors of these demyelinating disorders:
• Transverse myelitis
• Single episode of spinal cord demyelination, affecting cross-section OR several segments; symptoms include Brown-Sequard, bowel/bladder/sexual
dysfunction, spinal sensory level, weakness/spasticity
• Causes: virus, SLE, Sjogren’s, Antiphospholipid antibody syndrome, MS, idiopathic
• Tx: IV methylprednisolone  poor prognosis and worse than myelitis w/ MS & symptoms often persistent despite tx
• Progressive multifocal leukoencephalopathy (PML)
• Rapid onset &progressive behavioral, mental status changes & spasticity; MRI- non-enhancing, confluent lesions starting in post. brain white matter and
spreading forward; demyelinating damage in cerebral white matter
• JC virus activate, infects oligodendrocytes in CNS causing rapid demyelination; usually held in kidney and immunosuppression allows it to go to brain;
~20% of kids & 50% of adults have JC virus
• Eval: clinical findings, MRI, LP look for JC virus by PCR and antibodies; tx: reconstitute immune system (removing/tx immunosuppressant agent or
plasmapheresis) otherwise rapidly fatal
• Central pontine myelinolysis (CPM)
• Rapid onset of symptoms: confusion, slurred speech, facial droop, difficulty swallowing, eye movement abnormalities, weakness; seen typically in
ICU/hospital setting; alcoholic/metabolically compromised patient
• Causes: RAPID correction for low sodium (nutrient deficient) Wilson's disease, certain cancers
• Tx: supportive, best tx is prevention  rapid sodium correction
• Acute disseminated encephalomyelitis (ADEM)
• Similar to MS; single, multifocal/multisymptom neuro dysfunction; frequently post-infectious most often affecting brain & spinal cord
• Dx: demyelinating lesions on MRI, CSF shows signs of inflammation & demyelination and is single event unlike MS; Tx: IV methylprednisolone
• Leukodystrophies: progressive neuro dysfunction, progressive demyelination through CNS due to accumulation of excess substances; infantile, childhood,
adulthood onset; poor prognosis w/ no specific tx
• Adrenoleukodystrophy: peroxisomes cannot metab VLCFAs (build up in tests, adrenal glands, NS); childhood onset, X-linked recessive on ABCD1 gene;
bone marrow transplant sometimes curative & Lorenzo’s oil delays onset
• Krabbe disease: “globoid cell leuko”, infantile onset common, AR mutation in GALC gene  progressive accumulation of galactocerebrosidase in white
matter, globoid cells present
• Metachromatic leukodystrophy: def. of arylsulfatase A which build up in myelin in PNS & CNS causing progressive damage; AR defect in ARSA or PSAP
genes, no tx