Adult-onset Rasmussen's Syndrome with associated cortical dysplasia

Epilepsy Behav Case Rep. 2018 Nov 28:11:54-57. doi: 10.1016/j.ebcr.2018.11.004. eCollection 2019.

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.

Keywords: Adult-onset Rasmussen Syndrome; Cortical dysplasia; Epilepsia partialis continua; Functional hemispherectomy.

Publication types

  • Case Reports