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Case Report: An Unusual Case of Subacute Sclerosing Panencephalitis with Distinctive Clinical and Neuroimaging

Ravindra Kumar Garg1, Shweta Pandey1, Harish Nigam1

  • 1Department of Neurology, King George's Medical University Uttar Pradesh, Lucknow, India.

The American Journal of Tropical Medicine and Hygiene
|March 13, 2023
PubMed

Insights

Subacute sclerosing panencephalitis (SSPE), a fatal measles complication, presented unusually in a child with distinct neuroimaging findings. This case highlights rare cystic lesions in the white matter, prompting further investigation into their pathology.

Area of Science:

  • Neurology
  • Pediatrics
  • Infectious Diseases

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal neurological disorder invariably linked to measles virus infection.
  • It is more prevalent in measles-endemic regions and characterized by severe cognitive and motor decline.

Observation:

  • A 9-year-old boy presented with a 5-month history of progressive neurological deterioration, including myoclonus, akinetic mutism, and dystonic storms.
  • Clinical presentation included inappropriate emotional lability and marked right-sided dystonic posturing.
  • Electroencephalography showed periodic discharges, and cerebrospinal fluid revealed elevated anti-measles IgG antibodies.

Findings:

  • Neuroimaging demonstrated severe diffuse cerebral atrophy and periventricular white matter abnormalities.
  • Unusually, multiple small, discrete cystic lesions were observed in the periventricular white matter on T2/FLAIR MRI sequences.
  • The patient received intrathecal interferon-α, but remains in an akinetic-mute state.

Implications:

  • This case underscores the diverse clinical and neuroimaging spectrum of SSPE.
  • The presence of unique cystic lesions in SSPE warrants further research to elucidate their pathological basis.
  • Understanding these atypical features may improve diagnostic accuracy and inform future therapeutic strategies for SSPE.

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