Epilepsy surgery for Rasmussen encephalitis: the UCLA experience

Abstract

OBJECTIVERasmussen encephalitis (RE) is a rare inflammatory neurological disorder typically involving one hemisphere and resulting in drug-resistant epilepsy and progressive neurological decline. Here, the authors present seizure outcomes in children who underwent epilepsy surgery for RE at a single institution.METHODSThe records of consecutive patients who had undergone epilepsy surgery for RE at the UCLA Mattel Children’s Hospital between 1982 and 2018 were retrospectively reviewed. Basic demographic information, seizure history, procedural notes, and postoperative seizure and functional outcome data were analyzed.RESULTSThe cohort included 44 patients, 41 of whom had sufficient data for analysis. Seizure freedom was achieved in 68%, 48%, and 22% of the patients at 1, 5, and 10 years, respectively. The median time to the first seizure for those who experienced seizure recurrence after surgery was 39 weeks (IQR 11–355 weeks). Anatomical hemispherectomy, as compared to functional hemispherectomy, was independently associated with a longer time to postoperative seizure recurrence (HR 0.078, p = 0.03). There was no statistically significant difference in postoperative seizure recurrence between patients with complete hemispherectomy and those who had less-than-hemispheric surgery. Following surgery, 68% of the patients could ambulate and 84% could speak regardless of operative intervention.CONCLUSIONSA large proportion of RE patients will have seizure relapse after surgery, though patients with anatomical hemispherectomies may have a longer time to postoperative seizure recurrence. Overall, the long-term data in this study suggest that hemispheric surgery can be seen as palliative treatment for seizures rather than a cure for RE.

Rasmussen encephalitis: Predisposing factors and their potential role in unilaterality

“…In the present series, females predominated (58.1%). Previous series reported an equal sex distribution: 114 of 228 pooled patients in eight nonoverlapping series with >15 patients were female (50.0%) 12,16,20–25 . This may be related to their younger age at onset; in the present series, too, the younger patients had an equal sex distribution.…”

“…Previous series reported an equal sex distribution: 114 of 228 pooled patients in eight nonoverlapping series with >15 patients were female (50.0%). 12,16,[20][21][22][23][24][25] This may be related to their younger age at onset; in the present series, too, the younger patients had an equal sex distribution. A review of late onset RE, by contrast, showed a 4:1 female preponderance.…”

“…In our cohort, the left hemisphere was more frequently affected than the right one (65.6%). Five previous series 21,24,25,27,28 that gave lateralization data suggested a higher frequency of right‐hemispheric RE (pooled data: 101/185, 54.6%). This may be related to these being predominantly surgical series; right‐hemispheric operations are more readily performed then left‐sided procedures.…”

“…The median/mean age at onset in our series (7/10 years) was higher than in previous smaller series that gave medians between 5 and 6.1 years 12,16,20,21 and means between 4 and 7.2 years. 12,16,[21][22][23][24] This difference may be related to the aformentioned series often being surgical series. Hemispherectomy/hemispheric deafferentation is performed more frequently in younger patients.…”

Seizure Outcomes and Reoperation in Surgical Rasmussen Encephalitis Patients

“…The existing literature primarily consists of retrospective studies with small sample sizes. [6][7][8][10][11][12][13] Patients commonly have limited clinical data, become lost to follow-up, or studies feature multiple surgery types (hemispherectomy, hemispherotomy, or single lobar resection). Except for a single study examining 5 total patients who all underwent functional hemispherectomy between 1978 and 1981, no existing literature assesses outcomes of patients with RE who all received hemispherectomy.…”