Adult-onset Rasmussen's Syndrome with associated cortical dysplasia

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Abstract

We describe a 23-year-old woman with previous right temporal lobe surgeries for underlying cortical dysplasia, presenting with drug-resistant right hemispheric seizures and epilepsia partialis continua (EPC). After anti-seizure medication adjustments, she developed focal status epilepticus with progressive EEG and neuroimaging changes. Cerebrospinal fluid and serum autoimmune panels were negative except for an elevated serum acetylcholine-receptor antibody titer, but she underwent immunosuppressive therapy. Stereotactic-EEG evaluation demonstrated multifocal independent ictal patterns in the right hemisphere. Rasmussen's Syndrome was confirmed by brain biopsy, and a hemispherectomy was performed. This patient demonstrates the rare association of adult-onset EPC with cortical dysplasia, precipitously evolving into Rasmussen's Syndrome.

Original languageEnglish (US)
Pages (from-to)54-57
Number of pages4
JournalEpilepsy and Behavior Case Reports
Volume11
DOIs
StatePublished - Jan 1 2019

Keywords

  • Adult-onset Rasmussen Syndrome
  • Cortical dysplasia
  • Epilepsia partialis continua
  • Functional hemispherectomy

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Behavioral Neuroscience

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