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Related Experiment Videos

Double pathology in Rasmussen's encephalitis: etiologic considerations

E M Yacubian1, S Rosemberg, S K Marie

  • 1Department of Neurology, University of Sao Paulo School of Medicine, Brazil.

Epilepsia
|May 1, 1996
PubMed
Summary

Epilepsia partialis continua in a child showed progressive brain atrophy and "double pathology" on examination after surgery. This finding may offer new insights into Rasmussen's syndrome.

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Area of Science:

  • Neuroscience
  • Neuropathology
  • Pediatric Neurology

Background:

  • Epilepsia partialis continua (EPC) is a rare condition characterized by continuous focal seizures.
  • Rasmussen's syndrome is a progressive neurological disorder typically affecting one hemisphere of the brain, leading to frequent seizures and neurological deficits.

Observation:

  • A 7-year-old girl presented with a year-long history of EPC affecting the left face, arm, and leg.
  • Serial neuroimaging revealed progressive brain atrophy.
  • Medical treatments for the EPC were ineffective, necessitating a large fronto-temporal surgical resection.

Findings:

  • Neuropathological examination of the resected tissue demonstrated a 'double pathology'.
  • This included loss of cortical lamination and the presence of dysplastic neurons, indicative of a developmental abnormality (dysgenesis).

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  • Evidence of a chronic inflammatory process was also observed, including gliosis, microglial nodules, and perivascular cuffing.

    • Implications:

    • The co-occurrence of brain dysgenesis and chronic inflammation in this case provides a unique model for studying EPC.
    • This 'double pathology' may have significant implications for understanding the underlying pathophysiology of Rasmussen's syndrome.
    • Further research into these combined pathological features could lead to novel therapeutic strategies for both EPC and Rasmussen's syndrome.