Progressive Rubella Panencephalitis

Abstract

Progressive rubella panencephalitis (PRP) is a very rare disorder of the CNS. It is analogous to subacute sclerosing panencephalitis (SSPE) in many ways and is related to rubella virus. It develops about a decade after congenital rubella virus infection and can also occur years after childhood rubella infection. Children infected in utero with rubella may have multiple congenital defects including cataracts, retinal pigmentation, deafness, cardiac anomalies, and microcephaly with mental retardation. This disorder is nonprogressive. A number of these patients, however, develop progressive neurological symptoms during the second decade. A similar picture of neurological deterioration may occur in apparently healthy teenagers. The symptomatology and course of the disease resemble those of SSPE. The major clinical features of PRP are progressive dementia and signs of pyramidal and extrapyramidal abnormalities, with spastic paresis of the extremities, dysarthria, and hyperreflexia with extensor plantar responses and choreiform movements. Myoclonic seizures and other epileptic manifestations may be part of the clinical picture. Signs of cerebellar ataxia and nystagmus are prominent. Decreased vision with optic atrophy has been noted. Ongoing neurological deterioration eventually leads to death. The rate of progression of the illness is slower than in SSPE.