Open AccessFulminant adult-onset subacute sclerosing panencephalitis
Author(s) -
Anthony Faivre,
Jean-Baptiste Souraud,
Aileen McGonigal,
P. Alla,
Jacques Grapperon,
J. Valance
Publication year - 2009
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr.09.2008.0922
Subject(s) - subacute sclerosing panencephalitis , medicine , status epilepticus , fulminant , cerebrospinal fluid , encephalopathy , pathology , pediatrics , acute disseminated encephalomyelitis , brain biopsy , disease , measles , immunology , epilepsy , measles virus , psychiatry , vaccination
We present the case of a young adult who developed acute encephalopathy with severe status epilepticus and rapid deterioration to vegetative state and death within 6 weeks. Although the clinical picture, MRI and EEG findings were atypical, the hypothesis of subacute sclerosing panencephalitis (SSPE) was suggested by markedly increased intrathecal IgG synthesis in the cerebrospinal fluid, and diagnosis was confirmed by the presence of high antimeasles antibodies in cerebrospinal fluid and brain biopsy findings. Acute SSPE is an exceptionally rare and little-known form of SSPE with protean symptomatology, and this case is to our knowledge the first observation of SSPE presenting with status epilepticus in adults. Our case reinforces the need to include, even in developed countries, SSPE as a diagnostic possibility in unexplained acute encephalopathies.