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