Rasmussen's encephalitis: experience from a developing country based on a group of medically and surgically treated patients.

PURPOSE: To describe the attributes of patients with Rasmussen's encephalitis (RE) seen in a tertiary epilepsy referral center in southern India and to enquire factors helpful in predicting responsiveness to immunotherapy.
METHODS: We diagnosed RE based on the European consensus criteria. To assess the factors that could potentially predict the natural course and therapeutic outcome, we subcategorized our patients according to age at onset (<or> 6 years), duration from onset to presentation (<or> 2 years), immunotherapy versus surgery, and early (<or=2 years from the onset) versus late surgery.
RESULTS: The median age at disease onset of 19 patients was 6.0 years (range 2.3-13 years). Epilepsia partialis continua (EPC) and hemiparesis were noted in 14 (73.6%) and 16 (84.2%) patients, respectively. One patient, who presented with dysarthria due to tongue EPC, did not have hemiparesis despite having had the disease for over 15 years. The MRI findings in majority conformed to stage 3 of Bien classification. While 9/10 patients treated by surgery achieved seizure-freedom, only 1/11 patients who received immunotherapy did so. One patient expired due to subsequent development of contralateral hemispheric disease following successful hemispherectomy. None of the factors such as age at onset, age at presentation, presence/absence of antecedents, seizure burden, MRI stage predicted responsiveness to immunotherapy.
CONCLUSION: This study from a developing country, in addition to substantiating the well known characteristics of RE, noted the following unusual findings: isolated lingual EPC abolished by focal cortical resection, bilateral RE, putaminal atrophy and absence of hemiparesis despite long standing disease.