Abstract
Subacute sclerosing panencephalitis (SSPE) is a rare and progressive inflammatory
disease of central nervous system due to aberrant measles virus with an outcome that
is nearly always fatal. In acute fulminant SSPE, the disease rapidly evolves leading
to death within 3 months of the diagnosis. We report here four cases of fulminant
SSPE with atypical presentations, two of them presented at very early age with history
of congenital measles infection in first case and gait abnormality as initial symptom
in second case; acute disseminated encephalomyelitis (ADEM) with refractory seizures
in third case, unilateral myoclonus with hemiparesis in fourth case at the onset of
disease, respectively. The typical periodic electroencephalographic (EEG) complexes,
elevated cerebrospinal fluid (CSF), and serum antimeasles antibodies in our patients
led to the diagnosis of SSPE. A high index of clinical suspicion in fulminant type
with awareness of atypical features, EEG, and CSF studies are of paramount importance
in establishing its diagnosis.
Keywords
subacute sclerosing panencephalitis - atypical - early onset - fulminant - acute disseminated
encephalomyelitis - asymmetric presentation - EEG
