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Adult fulminant subacute sclerosing panencephalitis: pathological and molecular studies--a case report

J B Souraud1, A Faivre, D Waku-Kouomou

  • 1Department of Pathology, HIA Sainte-Anne, Toulon, France. jsouraud@hotmail.com

Clinical Neuropathology
|June 20, 2009
PubMed

Insights

Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder caused by measles virus. This case highlights a rare adult-onset SSPE, emphasizing diagnostic challenges in unexplained neurological conditions.

Area of Science:

  • Neurology
  • Virology
  • Pathology

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder typically affecting children, resulting from persistent measles virus infection.
  • Adult-onset SSPE is exceptionally rare and often presents atypically, posing diagnostic challenges.

Observation:

  • A case of fulminant SSPE in a 25-year-old male is presented.
  • Brain biopsy revealed encephalitis with characteristic intranuclear and intracytoplasmic inclusion bodies, inflammation, and gliosis.

Findings:

  • Immunohistochemistry confirmed measles virus presence using an anti-measles antibody.
  • RT-PCR detected measles virus RNA, with phylogenetic analysis identifying a C2 genotype.

Implications:

  • This case underscores the importance of considering adult-onset SSPE in the differential diagnosis of unexplained subacute neurological diseases.
  • Early recognition and diagnosis are crucial for managing this rare and severe infectious neurological disorder.

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