Adult-onset Rasmussen's Syndrome with associated cortical dysplasia

Charles A Szabo, Rachel E Garvin, Shaheryar Hafeez, Ali Seifi, Linda D Leary, Ratna K Bhavaraju-sanka, James M Henry, Alexander M Papanastassiou

Research output: Contribution to journalArticle

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

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Epilepsia Partialis Continua
Malformations of Cortical Development
Encephalitis
Electroencephalography
Seizures
Hemispherectomy
Status Epilepticus
Cholinergic Receptors
Temporal Lobe
Immunosuppressive Agents
Serum
Neuroimaging
Cerebrospinal Fluid
Stroke
Biopsy
Antibodies
Brain
Pharmaceutical Preparations
Therapeutics

Keywords

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

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Behavioral Neuroscience

Cite this

Adult-onset Rasmussen's Syndrome with associated cortical dysplasia. / Szabo, Charles A; Garvin, Rachel E; Hafeez, Shaheryar; Seifi, Ali; Leary, Linda D; Bhavaraju-sanka, Ratna K; Henry, James M; Papanastassiou, Alexander M.

In: Epilepsy and Behavior Case Reports, Vol. 11, 01.01.2019, p. 54-57.

Research output: Contribution to journalArticle

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AU - Henry, James M

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