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Case of fulminant-SSPE associated with measles genotype D7 from India: an autopsy study

Anita Mahadevan1, Sunil R Vaidya, Niteen S Wairagkar

  • 1Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India.

Neuropathology : Official Journal of the Japanese Society of Neuropathology
|April 4, 2008
PubMed

Insights

A rare, rapidly progressing neurological disorder, subacute sclerosing panencephalitis (SSPE), occurred in an 18-year-old Indian man. This fulminant case presented unusually without myoclonus, highlighting diagnostic challenges in post-measles complications.

Area of Science:

  • Neurology
  • Virology
  • Pathology

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological complication following measles infection.
  • Low vaccination rates in India contribute to the continued prevalence of SSPE.
  • The fulminant, acute form of SSPE is exceptionally uncommon.

Observation:

  • An 18-year-old male in South India presented with an ultra-short, 19-day course of fulminant SSPE.
  • Clinical presentation included hemiparesis and progressive cognitive decline, notably without myoclonus.
  • MRI revealed extensive demyelination in frontal and parieto-occipital regions, involving nuclear areas.

Findings:

  • Cerebrospinal fluid (CSF) showed anti-measles antibodies and oligoclonal bands, despite normal protein and cell counts.
  • Autopsy revealed florid necrotizing leukoencephalitis with acute demyelination, but sparse measles viral antigen in oligodendroglia.
  • Measles virus was isolated from the brain, with hypermutation in the M gene confirming the diagnosis.
  • Phylogenetic analysis identified the virus as belonging to the rare D7 genotype in India.

Implications:

  • This case expands the clinical spectrum of SSPE, particularly the fulminant form.
  • The findings underscore the importance of considering SSPE even with atypical presentations.
  • The presence of a rare viral genotype suggests potential for novel viral evolution and transmission dynamics in SSPE.

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