Diagnostic dilemmas in fulminating sub-acute sclerosing pan-encephalitis (SSPE) with atypical presentation

ABSTRACT

@#Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal disease of the central nervous system caused by a persistent measles virus in the brain. It is clinically characterized by insidious onset of intellectual deterioration and behavioral changes followed by myoclonic jerks and eventually complete neurologic deterioration. In about 10% of patients, the clinical manifestations of SSPE are not typical and that may cause a delay in the diagnosis. We report here 3 cases of SSPE with atypical presentations. Bilateral vision loss, hemiparesis, ataxia and acute encephalopathy with focal seizures were respectively the presenting symptoms at the onset of disease. The typical periodic EEG complexes in two patients and positive CSF measles IgG antibody led to the diagnosis of SSPE.