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Post hemispherectomy for Rassmussen encephalitis

Case contributed by Chris O'Donnell
Diagnosis certain

Presentation

Seizures and twitching. Past history of hemispherectomy for Rasmussen encephalitis as a child. Long standing right hemiplegia but otherwise functioning normally.

Patient Data

Age: 20 years
Gender: Male

Absence of the left cerebrum apart from the basal ganglia.  Note atrophy of the left cerebral peduncle (arrow) and filling of the left hemicranial space by CSF.

Case Discussion

This radical surgery is rarely performed in childhood for developmental lesions (mostly extensive cortical dysplasias), acute pathologies such as MCA infarct or trauma and progressive pathology such as Rasmussen encephalitis and Sturge-Weber syndrome. Despite the almost inevitable development of hemiplegia, most children have an excellent long-term outcome with reduction or absence of seizures, and normal intellectual function and speech.

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