An 8-year-old boy presents with Intellectual deterioration and myoclonus. The diagnosis is
(B) SSPE # SUBACUTE SCLEROSING PANENCEPHALITIS (SSPE) is a rare progressive demyelinating disease of the CNS associated with a chronic infection of brain tissue with measles virus.> Most patients give a history of primary measles infection at an early age (2 years), which is followed after a latent interval of 6 to 8 years by the development of insidious intellectual decline and mood and personality changes.> Typical signs of a CNS viral infection, including fever and headache, do not occur. Focal and/or generalized seizures, myoclonus, ataxia, and visual disturbances occur as the disease progresses. The EEG shows a characteristic periodic pattern with bursts every 3 to 8 s of high-voltage, sharp slow waves, followed by periods of attenuated ("flat") background.> The CSF is acellular with a normal or mildly elevated protein level and a markedly elevated globulin level (>20% of total CSF protein). CSF antimeasles antibody levels are invariably elevated, and oligoclonal antimeasles antibodies are often present.> CT and MRI show evidence of multifocal white matter lesions and generalized atrophy. Measles virus can be cultured from Drain tissue, and viral genome can be detected by in situ hybridization or PCR amplification.> Treatment with isoprinosine (Inosiplex) (100 mg/kg per day), alone or in combination with intrathecal or intraventricular interferon has been reported to prolong survival and produce clinical improvement in some patients but has never been subjected to a controlled clinical trial.
GSS
SSPE
Kuru
C-J disease
Miscellaneous