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Late-onset chronic progressive panencephalitis developed in a 12-year-old boy with congenital rubella syndrome from whose brain rubella virus was isolated. Progressive dementia began at eight, and ataxia, choreiform movements, myoclonic seizures, and fine perimacular pigmentation appeared at 11 years of age. The cerebrospinal fluid was minimally pleocytotic and had a total protein of 156 mg per deciliter, of which 52 percent was gamma globulin. Electroencephalography demonstrated generalized medium and occasional high-voltage slowing without burst suppression. The antibody titer to rubella virus (hemagglutination inhibition) was 1:8192 in serum and 1:256 in cerebrospinal fluid. Antibody titer to measles virus (complement fixation) was less than 1:8 in serum. Microscopical study of biopsied brain tissue at the age of 11 disclosed panencephalitis similar to subacute sclerosing panencephalitis, but with perivascular deposits and without inclusion bodies. Rubella virus was isolated from the brain by cocultivation with CV-1 cells. |
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ataxic gait
brain biopsy
cerebrospinal fluid,gammaglobulin of
cerebrospinal fluid,oligoclonal IgG in
children
chorea
congenital infection,CNS
dementia
electroencephalogram,abnormalities of
encephalitis
encephalitis,viral
gait disorder
hearing loss
intellectual deterioration
maculopathy
mimics
movement disorder
myoclonus,epilepsy
pleocytosis of cerebrospinal fluid
rubella encephalitis,progressive
rubella syndrome
rubella virus
seizure
serologic testing
serologic testing of cerebrospinal fluid
slow virus infection of CNS
subacute sclerosing panencephalitis(S.S.P.E.)Dawson's disease
viral infection
virus,slow
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