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Evolution of certain typical and atypical features in a case of subacute sclerosing panencephalitis

Tushar Premraj Raut1, Maneesh Kumar Singh, Ravindra Kumar Garg

  • 1Department of Neurology, Chhatrapati Shahuji Maharaj Medical University, Lucknow, Uttar Pradesh, India.

BMJ Case Reports
|December 26, 2012
PubMed

Insights

Subacute sclerosing panencephalitis (SSPE), a rare measles virus complication, presents subtly but progresses severely. This case highlights atypical features and stagewise deterioration, emphasizing diagnostic challenges in pediatric neurological disorders.

Area of Science:

  • Neurology
  • Virology
  • Pediatrics

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a rare, progressive central nervous system inflammatory disease.
  • It is caused by persistent measles virus infection, primarily affecting children and adolescents.
  • Early clinical and neuroimaging findings can be subtle and non-specific.

Observation:

  • A 14-year-old girl presented with myoclonic jerks and cognitive decline, initial subtle symptoms of SSPE.
  • During disease progression, she developed cortical vision loss and atypical extrapyramidal features, including segmental and hemifacial dystonia.
  • The patient ultimately reached a bedbound vegetative state.

Findings:

  • Electroencephalogram (EEG) revealed characteristic periodic discharges, a key diagnostic marker for SSPE.
  • Cerebrospinal fluid (CSF) serology confirmed the presence of measles virus antibodies.
  • The case illustrates the stagewise progression and diverse neurological manifestations of SSPE.

Implications:

  • This case underscores the importance of considering SSPE in pediatric patients with unexplained neurological decline, even with atypical presentations.
  • Early recognition and diagnosis are crucial for potential management strategies, despite the typically poor prognosis.
  • Understanding the varied clinical course of SSPE aids in better patient counseling and future research directions.

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