IMAGE OF THE WEEK

THELENGANA A PG 1STYRFROM IMCU WARD

• 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

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

OPEN RING SIGN

MULTIPLE SCLEROSIS

DAWSONS FINGERS

CNS TUBERCULOSIS

CNS TOXOPLASMOSIS

PML

ACUTE DISSEMINATED ENCEPHALOMYELITIS

Inflammatory, nonvasculitic, demyelinating, immune mediated, monophasic and polysymptomatic disease of the central nervous system

Post infectious encephalomyelitis,Post vaccination encephalomyelitis

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

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

INVESTIGATIONS

CSF ANALYSISCT BRAINMRI – T2 , FLAIR, CONTRAST – MTREEG,VEP

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

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)

RDEM MDEM

RECURRENCE OF ADEM

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

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.

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