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Atypical presentations of subacute sclerosing panencephalitis in two neurologically handicapped cases

E Demir1, A Ozcelik, E Arhan

  • 1Gazi University, School of Medicine, Department of Child Neurology, Ankara, Turkey. ercdemir28@yahoo.com

Neuropediatrics
|February 6, 2010
PubMed

Insights

Subacute sclerosing panencephalitis (SSPE) can present atypically in neurologically handicapped patients. Preexisting conditions may alter clinical and electrophysiological findings, impacting diagnosis and progression.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Neurodegenerative Disorders

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurodegenerative disease resulting from persistent measles virus infection.
  • Typical SSPE manifestations include cognitive decline, myoclonus, and characteristic electroencephalogram (EEG) patterns.

Observation:

  • Two cases of neurologically handicapped individuals presented with atypical SSPE features.
  • Patient 1 exhibited acute encephalopathy with seizures and hemiplegia, mimicking encephalitis, and showed decreased hippocampal diffusion on MRI.
  • Patient 2 displayed asymmetrical myoclonias contralateral to hemiparesis, with absent periodic EEG complexes.

Findings:

  • Preexisting neurological deficits can significantly modify the clinical and electrophysiological presentation of SSPE.
  • Diffusion-weighted imaging may reveal decreased diffusion in SSPE, potentially correlating with rapid disease progression.
  • Absence of typical EEG complexes can occur in patients with prior brain damage.

Implications:

  • SSPE should be considered in the differential diagnosis of acute encephalopathy, especially with lateralizing signs or unexplained seizures in at-risk individuals.
  • Atypical presentations highlight the importance of considering SSPE even when classic signs are absent.
  • Understanding these variations aids in earlier diagnosis and management of SSPE.

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