Dados do Trabalho

Título

Dominant side Hemispherotomy in Rasmussen Encephalitis in late childhood: a case report

Apresentação do caso

A 9-year-old right-handed girl, without clinical history, developed epilepsia partialis continua over the right face and progressive right hemiparesis. Anti-seizure medications were initiated, without appropriate seizure control. Brain magnetic resonance imaging (MRI) showed left progressive atrophy. Video-EEG monitoring revealed epileptiform discharges and slow waves over the left hemisphere, as well as seizures arising from the left hemisphere. Neuropsychological assessment showed average IQ and mild language disturbance consistent with dominant hemisphere impairment. Functional MRI exhibited left side dominance of language.Rasmussen Encephalitis (RE) diagnosis was made according to Bien’s criteria and left hemispherotomy (HP) was performed by the age of 10 years old. Histopathology examination of the biopsy confirmed RE diagnosis.Over immediate postoperative period, patient presented with right hemiplegia, severe amnestic aphasia and speech apraxia. Multi-professional rehabilitation assistance was initiated during hospitalization and continued after discharge. During following months, she developed progressive right hemiparesis improvement and become able to walk without assistance. Her language skills also improved and she recovered her pre-surgical cognitive status. 2 years after surgery she remains seizure free and Functional MRI revealed right dominance for language.

Discussão

RE is a rare neurological condition, characterized by unilateral hemispheric atrophy, refractory epilepsy and progressive neurological deterioration. The pathophysiology is not completely known, although evidence of an autoimmune process has been described. HP is the treatment of choice, as long as it provides the greatest chances of seizure control, although can also promote functional impairment. When high cortical functions are at risk during pre-surgical evaluation, especially for older children, and for the left hemisphere, the possibility of cognitive functions decline post-surgery may prevent a decision to perform it. Nevertheless, it is worth realizing that even when HP is performed in the context of late onset disease, even in the dominant side, the majority of patients keep their pre-operative cognitive status, like our case.

Comentários finais

This RE case demonstrates the possibility of performing HP in the dominant side with good seizure and functional outcome, even in late childhood.