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Detailed information of SUBACUTE SCLEROSING PANENCEPHALITIS
SUBACUTE SCLEROSING PANENCEPHALITIS
DEFINITION:
A slow viral infection of the brain characterized by chronic
encephalitis caused by a persistent measles viral infection.
EPIDEMIOLOGY:
- incidence: rare
- incidence decreased after immunization initiation in 1963
- risk 8.5/million cases of natural measles infection
- risk 0.7/million cases after immunization
- age of onset:
- 5-15 years in 85% of cases
- (5-15 years after a natural measles infection or
immunization)
- risk factors:
- M > F (2:1)
- urban > rural
- measles before age 18
PATHOGENESIS:
- pathogenesis involves the accumulation of incomplete measles
virus that cannot be cleared by B or T cell mechanisms
- measles genomes in SSPE are larger and contain multiple
mutations
- begins in cortical grey matter -> subcortical grey & white
matter -> lower structures
PATHOLOGY:
- inflammation, necrosis, gliosis, and repair
- panencephalitis involves cortical and subcortical grey and
white matter and blood vessels with an increasing number of
glial cells
CLINICAL FEATURES:
- mild to severe measles with full recovery or immunization
- mean interval between immunization and SSPE: 8 years
- mean interval between measles and SSPE : 12 years
2. Clinical Course
- insidious onset
- subtle changes in behaviour and declining school work:
- followed by overtly bizarre behaviour and dementia
- occasional headaches
2. 2nd Clinical Stage - Neurological Changes
- seizures
- myoclonic - symmetrical involving axial muscles
- generalized tonic-clonic develop later
- movement disorders
- cerebellar ataxia, chorea, choreoathetosis, dystonia,
progressive bulbar palsy, spasticity
- optic changes
- chorioretinitis, macular pigmentation, optic atrophy,
papilledema, retinopathy
- dementia progresses to stupor and coma in either flaccid
or spastic decorticate postures
- few patients live longer than 3 years after diagnosis
INVESTIGATIONS:
- IgG and IgM to measles virus
2. Cerebral Spinal Fluid
- elevated IgG and IgM fractions to measles virus on
oligoclonal electrophoresis
- normal cell count
- normal or elevated total protein
3. EEG
- 1st Stage - moderate nonspecific slowing
- 2nd Stage - episodes of "suppression-burst";high amplitude
slow and sharp waves recur at intervals of 3-5 sec on a slow
background
4. Imaging Studies
- variable cortical atrophy & ventricular enlargement
- normal or single or multiple focal low-density lesions in
the white matter
MANAGEMENT:
- anticonvulsants, physiotherapy, etc.
2. Medications
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Pediatric Database - SUBACUTE SCLEROSING PANENCEPHALITIS
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