Functional hemispherotomy in Rasmussen syndrome in the absence of classic MRI findings

Epilepsy Behav Case Rep. 2016 Dec 5:7:24-27. doi: 10.1016/j.ebcr.2016.11.003. eCollection 2017.

Abstract

A 7-year-old previously healthy girl presented with a left-sided focal seizure without impaired consciousness and subsequently developed epilepsia partialis continua. Initial MRI was normal, and the subsequent images only showed a focal T2/FLAIR hyperintense area without cortical atrophy. She was diagnosed with Rasmussen syndrome by pathology and promptly treated with functional hemispherotomy. Rasmussen syndrome is a rare progressive neurological disorder, the only definitive cure for which is hemispheric disconnection. The disease presents a management dilemma, especially early in disease course without characteristic neuroimaging features. A high index of suspicion, multidisciplinary approach, and clear timely communication with the family are critical.

Keywords: ASDs, antiseizure drugs; EEG, electroencephalogram; EPC, epilepsia partialis continua; EVD, external ventricular drain; Early diagnosis; Functional hemispherectomy; MRI, magnetic resonance imaging; PET, positron emission tomography; Peri-insular functional hemispherotomy; RS, Rasmussen syndrome; Rasmussen syndrome; Rasmussen's encephalitis.

Publication types

  • Case Reports