April 18, 2012

16 year old Male presenting with right sided weakness

7 days prior to admission

• Gradual onset of right sided weakness (clumsy, could not write well)• Complains of poor sensation over the right face. • Brought to an emergency room; Dx: Nerve impingement Reassurance• No relief of symptomsDay of admission• Was transferred to another correctional facility• Symptoms got worse• Can’t walk straight• Loss some feeling over the right leg.

PAST MEDICAL HISTORY Previously healthy; no previous episodesNo known allergies to food/medications

FAMILY HISTORY No known family history of any cardiac, respiratory, rheumatologic or neurologic conditions

PERSONAL-SOCIAL HISTORY Allen McQueen correctional facility for boys since 9 months ago. Receives all medical care/shots. Now in middle school with no issues and learning disabilities. Denies smoking/alcohol. 1 x illicit drug use. Sexual debut at 15 years old, had only 2 lifetime partners with inconsistent use of protection. Previously tested for STI - negative

System Findings

Vital signs

General Appearance

Awake, alert, very cooperative, not in acute distress

HEENT NCAT, Pinkish palpebral conjunctiva, anicteric sclerae, moist mucous membranes, nasal septum midline, normal TM’s, no palpable CLAD

Cardiovascular NSRRR, Normal S1 and S2, no murmurs, no rubs, no gallops

Respiratory CTA b/l, GAEB, no crackles, no wheezes

Abdomen Flat abdomen, non-distended, soft, normoactive bowel sounds,

Extremities No edema, no cyanosis, cap refill <2 s

System Findings

Mental Status

Cooperative patient and oriented to person, time and place; appropriate affect

Cranial Nerves

CN 2-12 intact

Motor/DTR

See diagram

Sensory Decreased cold sensation over the right cheek

Cerebellar

No ataxia, dysmetria nor nystagmus

Gait (+) Antalgic gait on the right

Meningeal

No meningeal signs

2+4/5

3+4/5

2+5/5

2+5/5

Neurologic Ischemic vs Hemorrhagic Stroke Peripheral nerve disease Demyelinating Disease

Concerned about ischemic stroke Labs were ordered IVF started MRI obtained

CBC

Parameter Results

WBC count 5.3

Hemoglobin 15.9

Hematocrit 45.1

Platelets 201

N 56

L 32

CHEMISTRIES

Parameter Results

Na+ 133

K+ 4.4

Cl- 100

CO2 29

BUN 14

Crea 1.0

Glucose 97

Calcium 9.5

CHEMISTRIES OTHER TESTS

Parameter Results

ALT 28

AST 24

Bilirubin 1.0

Albumin 4.1

Total Protein 6.5

Magnesium 21

Phosphorus 4.9

Parameter Results

Vitamin B12 557

RPR Non-reactive

Hepa C Ag Non-reactive

Hepa C Ab Non-reactive

HIV Negative

ANA Negative

ESR 10

CSF Cell count – normalCSF Proteins – 23CSF Glucose – 79

CSF Oligoclonal bands - Negative

Vincent Patrick Uy, MDPGY-1

No single diagnostic test is specific to particular diagnosis

Must rely on History and PE, clinical features and imaging to arrive at a plausible diagnosis

Uncommon in the pediatric age group

Think of RARE disorders!

Acute Disseminated Encephalomyelitis

Multiple Sclerosis Optic Neuropathy Transverse Myelitis Neuromyelitis

Optica

AdrenoleukodystrophiesCytoskeletal disorders (Alexander’s

disease)Myelin Basic Protein disorderOrganic acid diseases (Canavan

disease)Disorders of energy metabolism

(MELAS)Krabbe disease

CNS lymphomas High-grade glioma

Large area of tumefactive demyelination

CSF studies show atypical cells

Systemic Lupus Erythomatosus

Behcet’s disease Neurosarcoidosis

Look for other symptoms

Anti-dsDNA, anti-phospholipids, elevated ACE enzymes

May be a clinically isolated syndrome or part of another condition

Monocular in most adults; bilateral in most children 12-15 years old.

Symptoms can progress from several hours to days

Risk factor for developing frank Multiple Sclerosis

Headache Painful eye

movements Partial or complete

vision loss Relative afferent

pupillary defect Papillitis (75% in

the acute phase)

Spinal cord disease without evidence of compression

Inflammation of the spinal cordMay present as a clinically isolated

disorder or part of other conditions.Most cases are idiopathicSubset of patients develop TM after

vaccination

Weakness, paresthesias, urinary and bladder problems

Hyperacute presentation

MRI shows contrast enhancing lesions on the cord

Idi0pathic Transverse Myelitis Spontaneous but partial recovery in 1-3

months 40% will have a persistent disability

Devic’s DiseaseCombination of:

Optic neuritis Transverse Myelitis Seropositivity to NMO IgG antibodies >3 contiguous spinal levels involved

Presentation can be primarily progressive with a fulminant course on presentation

Acute or subacute onset of multifocal neurologic deficits with encephalopathy Often follows a viral infection or

vaccinationVery uncommon illnessAutoimmune disorder of the CNS,

triggered by environmental factors, in genetically susceptible individuals

CLASSIFICATION DESCRIPTION

Classic Monophasic; happens only once

Relapsing Recurs more than 3 months after recovery - or - More than 1 month from stopping steroids

Recurrent Subtype of relapsing ADEM presenting with similar symptoms and MRI lesions

Multiphasic Subtype of relapsing ADEM presenting with different symptoms and different MRI lesions

• Patients with ADEM, no matter what form, should have complete recovery to baseline.

Clinical Presentation Febrile illness occurs in about 50-75% of

children a month before neurologic symptoms appear.

Headache and meningeal signs Encephalopathy (changes in mental

status) Acute hemiparesis, cerebellar ataxia,

optic neuritis and spinal cord dysfunction are also common

Treatment Fever, meningisimus, headaches,

change in mental state, etc. Treat for suspected meningitis/encephalitis

Imaging may be obtained High dose IV Glucocorticoids treatment

for 3-5 days + steroid taper for 4-6 weeks▪ Methylprednisolone (10-30 mg/kg/d)▪ Dexamethasone (1 mg/kg/d)

Chronic disease characterized by repeated episodes of demyelination separated by space and time

Pediatric multiple sclerosis age of onset before 16 years old

2.5/100,000 children

Genetic Susceptibility Higher risk in monozygotic twins Certain HLA subtypes are associated

with increased riskEnvironmental factors

Epstein-Barr Virus infection Vitamin D deficiency

SUBTYPES OF MULTIPLE SCLEROSIS

Description

RELAPSING REMITTING MS • Repeated episodes of MS with partial or complete recovery between episodes

• 97-99% of children

PRIMARY PROGRESSIVE MS • Continuous and worsening disease activity over time.

• Very rare in both adults and children

CLINICAL ISOLATED SYNDROME • Does not fit the diagnostic criteria of MS.

• Acute demyelination x 1 episode

Diagnostic Criteria for Diagnosis of MS1. Dissemination in Space2. Dissemination in Time

Dissemination in Space T2 lesions on MRI in at least two of the

four MS-typical regions of the brain:▪ Periventricular▪ Juxtacortical▪ Infratentorial▪ Spinal Cord

Development of a further clinical attack implicating a different CNS site

Dissemination in Time Simultaneous presence of asymptomatic

gadolinium-enhancing and non enhancing lesions at anytime or a new T2 lesion on a follow-up MRI (irrespective of timing)

Development of a second clinical attack

Clinically Isolated Syndrome INCREASED RISK for MS▪ Age 10 years or older▪ Optic nerve lesions▪ MRI pattern very typical for MS

DECREASED RISK for MS▪ Spinal cord lesions▪ Acute Mental status change (ADEM)

Treatment strategy (Acute setting) IV pulse methylprednisolone 20-30

mg/kg/d x 5 days Steroid taper 1 mg/kg/day for 4-6 weeks

if without complete resolution May re-treat with IV pulse

methylprednisolone if symptoms recur during steroid taper

Treatment strategy (Chronic Therapy) Glatiramer acetate Interferons

Monitoring patients after and ACUTE ATTACK

Completion of steroid taper

Neurologic exam at 1/3/6 months

thereafter

Clinically stable

Annual Physical Examination

Symptomatic

Send for MRI

Received 5 day high dose methylprednisolone therapy via IV

Completed steroid taper with prednisone PO

On follow-up 4/3/2012 Complete resolution of symptoms with return to baseline

Dismissed as a case of Acute Demyelination – Clinically Isolated Syndrome

A 9 year old girl with anxiety disorder was diagnosed with acute demyelination because of sudden onset of left sided weakness and ataxia. Typical MS lesions which included the cerebellum and spinal cord were discovered after MRI. She comes to the clinic for the first time after completing 6 weeks of steroid taper. On further questioning, mom has missed several doses of her prednisone at home. So far, she never experienced symptoms again. Mom is concerned that the catastrophe might happen again. Which of the following conditions increases her risk to develop frank multiple sclerosis?

A. Non compliance to steroid taper

B. Her young ageC. Typical lesions were

seen on MRID. Concomitant anxiety

disorderE. She doesn’t have

risk factors that increase her risk to develop MS.

A 17 year old boy was brought to the ER for change in mental status. Ultimately, he was diagnosed with ADEM. He recovered after 5 days in the ICU and is now back to baseline. Which of the following environmental triggers could have increased his risk for developing the condition.

A. Receiving an unrecalled vaccine from the PMD a month ago during annual visit

B. Getting daily morning sun exposure for 15-20 minutes

C. Blurring of vision without eye pain

D. Experiencing an exudative sore throat which was negative on rapid strep test

E. Spider bite 2 weeks ago