Language recovery after left hemispherotomy for Rasmussen encephalitis

Purpose: Hemispherotomy (H) is the gold standard treatment to cure epilepsy in Rasmussen encephalitis (RE). Linguistic prognosis after surgery remains the main issue when the dominant hemisphere is involved. The topic of the present research is to specify the long-term linguistic profile of the right hemisphere after left dominant H for RE.

Methods: We followed 6 children 8.4 to 14.6 years of age who underwent left H for RE. Preoperatively, four children experienced aphasia, but for two, worsening occurred after surgery. Age at H ranged from 4.1 to 8.4 years. The mean duration of epilepsy was 1.2 years and 5.6 years for follow-up. Neuropsychological evaluation included longitudinal follow-up of intellectual efficiency measurement and a long-term outcome of language using various components of receptive and expressive oral speech with computerized tasks.

Key findings: Preoperatively, verbal comprehension index (VCI) was dramatically decreased in 4/6 patients, and performance reasoning index (PRI) was low in 5/6 participants, demonstrating a global impact of RE itself. Postoperatively, all children recovered sufficiently to attend a regular VCI (above 70) in a mean of 5 years after H, and 5/6 recovered normal or adapted school. There was a dissociation in favor of VCI, while PRI decreased in 5/6 patients. We found a specific linguistic profile for these children recovering language in the right hemisphere: normal verbal comprehension, and weakness of grammatical judgment, word repetition, statement production, semantic verbal fluency and metaphonological abilities. Language recovery scores were statistically correlated with those of Working Memory Index.

Significance: This study emphasizes for the first time the ability of the right hemisphere to functionally reorganize language over a long period of time following left H for RE. Syntactic abilities and phonology remain low and support the hypothesis of an early left hemispheric specialization. Nevertheless, lexico-semantic processes recover in the right hemisphere that could reflect a pre-existing potential of both hemispheres. Our results support a decision to proceed to H in classical left RE disease until the late childhood even if there is no complete aphasia before surgery. These data should be taken in account in the overall postoperative follow-up and rehabilitation strategy.