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TRANSCRIPT
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IMAGE OF THE WEEK
THELENGANA A PG 1STYRFROM IMCU WARD
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• 15 Yrs old female presented with h/o Fever 2 days Asymptomatic 10 days Headache,vomiting
Altered sensorium for 1 weekNo h/o seizures/visual disturbanceNo h/o vaccination /exanthematous illness
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O/E vitals were stable CNS examn :Pt was drowsy , arousable with painful stimulus PERL , DEM preserved exaggerated DTR B/L plantar extensor fundus examination – B/L disc edemaOther systemic examination was unremarkable
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OPEN RING SIGN
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MULTIPLE SCLEROSIS
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DAWSONS FINGERS
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CNS TUBERCULOSIS
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CNS TOXOPLASMOSIS
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PML
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ACUTE DISSEMINATED ENCEPHALOMYELITIS
Inflammatory, nonvasculitic, demyelinating, immune mediated, monophasic and polysymptomatic disease of the central nervous system
Post infectious encephalomyelitis,Post vaccination encephalomyelitis
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PATHOGENESIS
• Molecular mimickery: brain vaccines– Th2 lymphocytes have increased reactivity to
myelin basic protein
• Inflammatory cascade concept: – CNS infections triggering immune response,
damage to BBB, brain specific antigens spills into systemic circulation and initiates immunologic process
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ADEM
PRODROMAL PHASE
ALTERED SENSORIUM
MENINGISMUS
NEUROSYCHIATRIC DISORDER
B/L OPTIC NEURITIS
COMPLETE TRANSVERSE MYELITIS
SEIZURES
ATAXIA
MONOPHASIC
POLYSYMPTOMATIC
MULTIPLE SCLEROSIS
NO PRODROMAL PHASE
PRESERVED AWARENESS
NO MENINGISMUS
NO NEUROPSYCHIATRIC
UNILATERAL OPTIC NEURITIS
INCOMPLETE
DIPLOPIA
RELAPSING
POLYPHASIC
MONOSYMPTOMATIC
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INVESTIGATIONS
CSF ANALYSISCT BRAINMRI – T2 , FLAIR, CONTRAST – MTREEG,VEP
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NEUROIMAGING
• MRI: extensive, multifocal, subcortical
white matter abnormalities
• MRI: subcortical white matter, may be grey matter also,
• CT may be normal in 50% cases• Convalescent MRI helpful in diffrentating with
MS, new lesions in MS
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MRI FeaturesADEM
• Patchy, poorly marginated areas of increased signal intensity; large, asymmetric, multiple
• Four patterns:– ADEM with less than 5 mm lesions– Large, confluent lesions with edema and mass
effect– ADEM with additional symmetric bithalamic
involvement– Acute hemorrhagic encephalomyelitis (worst
prognosis)
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RDEM MDEM
RECURRENCE OF ADEM
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TREATMENT
• Broad spectrum antibiotics and acyclovir until an Infectious etiology is excluded.
• Methylprednisolone in a dose of 30 mg/kg per day intravenously up to a maximum dose of 1000 mg per day X 5 days
• Plasmapharesis • Intravenous immunoglobulin• Cyclosporin , cyclophosphamide• Methylpred + IVIG
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PROGNOSIS
• Mortality: 10% in older studies, Now <2%• Morbidity: visual, motor, autonomic, and intellectual
deficits and epilepsy.
– Problems persist after the first few weeks of illness in only about 35% of cases, and in most of these patients, the deficits resolve within 1 year of onset.
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FOLLOW UP
• The long-term (10-y follow-up) risk of patients with ADEM for development of MS is 25%.
• Risk for MS is highest in children whose ADEM onset was – (1) afebrile, – (2) without mental status change, – (3) without prodromal viral illness or immunization, – (4) without generalized EEG slowing, – (5) associated with an abnormal CSF immune
profile