Adult-onset Rasmussen's Syndrome with associated cortical dysplasia.

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Adult-onset Rasmussen's Syndrome with associated cortical dysplasia.

Epilepsy Behav Case Rep. 2019;11:54-57

Authors: Szabó CÁ, Garvin R, Hafeez S, Seifi A, Leary L, Bhavaraju-Sanka R, Henry JM, Papanastassiou AM

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.

PMID: 30705820 [PubMed]

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