Neuropediatrics
DOI: 10.1055/s-0040-1715623
Short Communication

Atypical Presentation of Fulminating Subacute Sclerosing Panencephalitis: A Case Series

Pranab K. Dey
1  Department of Paediatrics, R. G. Kar Medical College and Hospital, Kolkata, West Bengal, India
,
Arindam Ghosh
2  Department of Paediatrics, Midnapore Medical College, Midnapore, West Bengal, India
› Author Affiliations
Funding No funding source.

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.

Financial Disclosure

Nothing.


Contributors' Statement

P.K.D. and A.G. worked up, managed, and collected patient's data and drafted the manuscript. The final manuscript was approved by both the authors.


Supplementary Material



Publication History

Received: 06 May 2020

Accepted: 11 June 2020

Publication Date:
16 October 2020 (online)

Georg Thieme Verlag KG
Stuttgart · New York