1) A 15-year-old female presented with fever, headache, vomiting and altered sensorium for a week and was diagnosed with acute disseminated encephalomyelitis (ADEM), an inflammatory disease of the central nervous system.
2) Investigations showed bilateral disc edema on fundus examination and MRI revealed extensive, multifocal white matter abnormalities.
3) Treatment involves high dose corticosteroids like methylprednisolone and intravenous immunoglobulin to reduce inflammation. The long-term risk of developing multiple sclerosis after ADEM is 25%.
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Adem
1. IMAGE OF THE WEEK
THELENGANA A
PG 1STYR
FROM IMCU WARD
2. 15 Yrs old female presented with h/o
Fever 2 days
Asymptomatic 10 days
Headache,vomiting
Altered sensorium for 1 week
No h/o seizures/visual disturbance
No h/o vaccination /exanthematous illness
3. 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 edema
Other systemic examination was unremarkable
13. ACUTE DISSEMINATED
ENCEPHALOMYELITIS
Inflammatory, nonvasculitic, demyeli
nating, immune
mediated, monophasic and
polysymptomatic disease of the
central nervous system
Post infectious encephalomyelitis,
Post vaccination encephalomyelitis
14. 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
17. 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
18. MRI Features
ADEM
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)
20. 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
21. 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.
22. 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