Adult-onset Rasmussen's Syndrome with associated cortical dysplasia

• Main Authors: C. Ákos Szabó, Rachel Garvin, Shaheryar Hafeez, Ali Seifi, Linda Leary, Ratna Bhavaraju-Sanka, James M. Henry, Alex M. Papanastassiou
• Format: Article
• Language: English
• Published: Elsevier 2019-01-01
• Series: Epilepsy and Behavior Case Reports
• Online Access: http://www.sciencedirect.com/science/article/pii/S2213323218301130

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, Epilepsia partialis continua, Cortical dysplasia, Functional hemispherectomy