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

• Published in: Epilepsy & behavior case reports Vol. 7; no. C; pp. 24 - 27
• Main Authors: Nagahama, Yasunori, Joshi, Charuta, Dlouhy, Brian, Wu, Angela Y, Abel, Taylor J, Baumbach, Gary, Kawasaki, Hiroto
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.01.2017; Elsevier
• Subjects: Case Report; Early diagnosis; Functional hemispherectomy; Neurology; Peri-insular functional hemispherotomy; Rasmussen syndrome; Rasmussen's encephalitis; RS; magnetic resonance imaging; Early diagnosis; MRI; EEG; Peri-insular functional hemispherotomy; electroencephalogram; Rasmussen syndrome; EVD; Functional hemispherectomy; antiseizure drugs; EPC; external ventricular drain; positron emission tomography; epilepsia partialis continua; Rasmussen's encephalitis; PET; ASDs; MRI, magnetic resonance imaging; PET, positron emission tomography; RS, Rasmussen syndrome; EEG, electroencephalogram; EPC, epilepsia partialis continua; ASDs, antiseizure drugs; EVD, external ventricular drain
• ISSN: 2213-3232; 2213-3232
• DOI: 10.1016/j.ebcr.2016.11.003

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.