Transcript Case Conference

April 18, 2012
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16 year old Male presenting with right sided
weakness
7 days prior to admission
Day of 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 symptoms
• 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 episodes
No 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
2+
4/5
2+
5/5
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
3+
4/5
2+
5/5
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Neurologic
 Ischemic vs Hemorrhagic Stroke
 Peripheral nerve disease
 Demyelinating Disease
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Concerned about ischemic stroke
 Labs were ordered
 IVF started
 MRI obtained
CBC
CHEMISTRIES
Parameter
Results
Parameter
Results
WBC count
5.3
Na+
133
Hemoglobin
15.9
K+
4.4
Hematocrit
45.1
Cl-
100
Platelets
201
CO2
29
N
56
BUN
14
L
32
Crea
1.0
Glucose
97
Calcium
9.5
CHEMISTRIES
OTHER TESTS
Parameter
Results
Parameter
Results
ALT
28
Vitamin B12
557
AST
24
RPR
Non-reactive
Bilirubin
1.0
Hepa C Ag
Non-reactive
Albumin
4.1
Hepa C Ab
Non-reactive
Total Protein
6.5
HIV
Negative
Magnesium
21
ANA
Negative
Phosphorus
4.9
ESR
10
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CSF Cell count – normal
CSF Proteins – 23
CSF Glucose – 79
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CSF Oligoclonal bands - Negative
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Vincent Patrick Uy, MD
PGY-1
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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!
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Acute Disseminated
Encephalomyelitis
Multiple Sclerosis
Optic Neuropathy
Transverse Myelitis
Neuromyelitis Optica
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Adrenoleukodystrophies
Cytoskeletal disorders (Alexander’s disease)
Myelin Basic Protein disorder
Organic acid diseases (Canavan disease)
Disorders of energy metabolism (MELAS)
Krabbe disease
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CNS lymphomas
High-grade glioma
Large area of
tumefactive
demyelination
 CSF studies show
atypical cells
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Systemic Lupus
Erythomatosus
 Behcet’s disease
 Neurosarcoidosis
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Look for other
symptoms
 Anti-dsDNA, antiphospholipids,
elevated ACE enzymes
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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)
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Spinal cord disease without evidence of
compression
Inflammation of the spinal cord
May present as a clinically isolated disorder or
part of other conditions.
Most cases are idiopathic
Subset of patients develop TM after
vaccination
Weakness,
paresthesias, urinary
and bladder problems
 Hyperacute
presentation
 MRI shows contrast
enhancing lesions on
the cord
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Idi0pathic Transverse Myelitis
 Spontaneous but partial recovery in 1-3 months
 40% will have a persistent disability
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Devic’s Disease
Combination of:
 Optic neuritis
 Transverse Myelitis
 Seropositivity to NMO IgG antibodies
 >3 contiguous spinal levels involved
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Presentation can be primarily progressive
with a fulminant course on presentation
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Acute or subacute onset of multifocal
neurologic deficits with encephalopathy
 Often follows a viral infection or vaccination
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Very uncommon illness
Autoimmune 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.
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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
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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)
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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
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Genetic Susceptibility
 Higher risk in monozygotic twins
 Certain HLA subtypes are associated with
increased risk
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Environmental 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
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Diagnostic Criteria for Diagnosis of MS
1. Dissemination in Space
2. Dissemination in Time
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Dissemination in Space
 T2 lesions on MRI in at least two of the four MS-
typical regions of the brain:
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Periventricular
Juxtacortical
Infratentorial
Spinal Cord
 Development of a further clinical attack
implicating a different CNS site
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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
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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)
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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
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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
Symptomatic
Annual Physical
Examination
Send for MRI
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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?
Non compliance to
steroid taper
B. Her young age
C. Typical lesions were seen
on MRI
D. Concomitant anxiety
disorder
E. She doesn’t have risk
factors that increase her
risk to develop MS.
A.
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.
B.
C.
D.
E.
Receiving an unrecalled
vaccine from the PMD a
month ago during
annual visit
Getting daily morning
sun exposure for 15-20
minutes
Blurring of vision
without eye pain
Experiencing an
exudative sore throat
which was negative on
rapid strep test
Spider bite 2 weeks ago