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Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis and pseudotumour cerebri

Akhila Kumar Panda1, Vachan Jayant Mehta, Siddharth Maheshwari

  • 1Department of Neurology, Institute of Human Behaviour & Allied Sciences IHBAS, Delhi, India. drakhilapanda@gmail.com

BMJ Case Reports
|August 22, 2013
PubMed

Insights

Subacute sclerosing panencephalitis (SSPE) is a rare, progressive brain disease caused by persistent measles virus infection. Early diagnosis using clinical, imaging, and CSF markers is crucial, especially in endemic areas.

Area of Science:

  • Neurology
  • Virology
  • Pediatrics

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a chronic, progressive encephalitis resulting from persistent measles virus infection, typically affecting children and young adults.
  • The disease commonly presents between ages 5 and 15, but varied clinical manifestations can complicate diagnosis.

Observation:

  • A 13-year-old boy presented with intellectual decline, headache, papilledema, cranial nerve palsy, and myoclonus.
  • Neuroimaging suggested acute disseminated encephalomyelitis (ADEM) or pseudotumor cerebri, posing a diagnostic challenge.

Findings:

  • Cerebrospinal fluid (CSF) and serum measles antibody titers confirmed the diagnosis of SSPE.
  • The case highlights diagnostic difficulties in regions with endemic measles and variable vaccination coverage.

Implications:

  • Accurate diagnosis of SSPE requires integrating clinical findings with neuroimaging, EEG, and specific CSF/serum markers.
  • Improved measles vaccination and awareness are critical, particularly in developing countries, to prevent SSPE.

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