Key result
Four cases of fulminant subacute sclerosing panencephalitis present with atypical features including early onset.
Case Report (n=4)
Highlights the importance of high clinical suspicion and EEG/CSF studies for diagnosing atypical fulminant subacute sclerosing panencephalitis.
Atypical SSPE features may delay diagnosis in children; extends phenotypic spectrum but leaves criteria open for validation.
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.
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Dey et al. (2020) conducted a case report in Fulminant subacute sclerosing panencephalitis (SSPE) (n=4). Atypical presentation of fulminant SSPE was evaluated. Four cases of fulminant subacute sclerosing panencephalitis presented with atypical features such as early age onset, congenital measles, gait abnormality, ADEM, and unilateral myoclonus.
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