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Rasmussen encephalitis in childhood.

M Topçu1, G Turanli, F M Aynaci

  • 1Department of Pediatric Neurology, Hacettepe University, Faculty of Medicine, Ankara, Turkey. mtopcu@genetic.gen.hun.edu.tr

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|August 14, 1999
PubMed
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Early surgical intervention for Rasmussen encephalitis in children can lead to effective seizure control and aid in diagnosis. This pediatric neurology study highlights the potential benefits of timely surgical treatment for intractable epilepsy.

Area of Science:

  • Pediatric Neurology
  • Neurosurgery
  • Epileptology

Background:

  • Rasmussen encephalitis is a rare, chronic inflammatory neurological disease affecting one cerebral hemisphere, leading to progressive neurological deficits and intractable epilepsy.
  • Intractable epilepsy in pediatric patients often necessitates exploring advanced treatment modalities beyond conventional anticonvulsant therapies.

Observation:

  • Six pediatric patients diagnosed with Rasmussen encephalitis underwent surgical treatment for drug-resistant epilepsy.
  • Diagnostic evaluations included MRI, SPECT, WADA tests, and PCR for viral studies in relevant cases.
  • Seizures, often presenting as epilepsia partialis continua, were refractory to multiple anticonvulsant drugs and showed only temporary response to intravenous immunoglobulin therapy in some cases.

Findings:

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  • Resective surgery with electrocorticography was the chosen surgical approach.
  • One patient, who underwent surgery three months after seizure onset, achieved complete seizure control.
  • The mean postsurgical follow-up was 32.3 months, indicating the need for long-term monitoring.

    • Implications:

    • Early surgical intervention in Rasmussen encephalitis may offer both diagnostic confirmation and effective seizure management.
    • This approach could potentially halt disease progression and improve long-term neurological outcomes in affected children.
    • Further research into optimal timing and surgical techniques for Rasmussen encephalitis is warranted.