Nationwide survey (incidence, clinical course, prognosis) of Rasmussen's encephalitis.
OBJECTIVE
Rasmussen's encephalitis (RE) is a progressive and catastrophic epileptic disorder caused by chronic localized encephalitis. We performed a nationwide survey of RE to assess the clinical picture, treatment effect, and prognosis of Japanese RE patients.


METHODS
The subjects were 27 patients (male:12; female:15) from 13 medical facilities. All of them satisfied the clinical and neuroimaging criteria for RE, including 14 pathologically proven cases.


RESULTS
They were divided into the childhood-onset rapidly progressive type (CORP, n=19), and late-onset slowly progressive type (LOSP, n=8). The mean age at epilepsy onset was 4 years and 4 months in CORP, and 16 years in LOSP. The mean period between the onset age of epilepsy and development of frequent seizures was 1 year and 4 months in the former, and 3 years and 4 months in the latter. The immunomodulatory treatment including high-dose steroid (n=14) and high-dose intravenous immunoglobulin therapies (IVIgG, n=12) achieved more than a 50% reduction in the seizure frequency in 5 (36%) and 4 (33%) patients, respectively. Eight and seven patients underwent focal cortical resection and functional hemispherectomy, leading to significant improvement in 5 of the 8 patients and excellent seizure control in all 7 patients, respectively.


CONCLUSIONS
Although the high-dose steroid and IVIG therapies may have alleviated the exacerbation of seizures in those with RE, they could not halt the disease progression. Functional hemispherectomy is still the only curative therapy for RE, despite the fact that the early introduction of this procedure remains controversial.
Muto
Ayako
A
Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan.
Oguni
Hirokazu
H
Takahashi
Yukitoshi
Y
Shirasaka
Yukiyoshi
Y
Sawaishi
Yukio
Y
Yano
Tamami
T
Hoshida
Toru
T
Osaka
Hitoshi
H
Nakasu
Satoru
S
Akasaka
Noriyuki
N
Sugai
Kenji
K
Miyamoto
Akie
A
Takahashi
Satoru
S
Suzuki
Motomasa
M
Ohmori
Iori
I
Nabatame
Shin
S
Osawa
Makiko
M
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001921:Brain / Q000187:drug effects / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018450:Disease Progression; D004660:Encephalitis / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D004827:Epilepsy / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007564:Japan / Q000453:epidemiology; D008297:Male; D011379:Prognosis; D011795:Surveys and Questionnaires; D055815:Young Adult