Nationwide survey (incidence, clinical course, prognosis) of Rasmussen's encephalitis

Ayako Muto, Hirokazu Oguni, Yukitoshi Takahashi, Yukiyoshi Shirasaka, Yukio Sawaishi, Tamami Yano, Toru Hoshida, Hitoshi Osaka, Satoru Nakasu, Noriyuki Akasaka, Kenji Sugai, Akie Miyamoto, Satoru Takahashi, Motomasa Suzuki, Iori Ohmori, Shin Nabatame, Makiko Osawa

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Purpose: 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. Subjects & 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. Conclusion: 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.

Original languageEnglish
Pages (from-to)445-453
Number of pages9
JournalBrain and Development
Volume32
Issue number6
DOIs
Publication statusPublished - Jun 1 2010

Keywords

  • Chronic localized encephalitis
  • Epilepsia partialis continua
  • Immunomodulatory therapy
  • Intractable epilepsy
  • Nationwide survey
  • Rasmussen's encephalitis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Clinical Neurology

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  • Cite this

    Muto, A., Oguni, H., Takahashi, Y., Shirasaka, Y., Sawaishi, Y., Yano, T., Hoshida, T., Osaka, H., Nakasu, S., Akasaka, N., Sugai, K., Miyamoto, A., Takahashi, S., Suzuki, M., Ohmori, I., Nabatame, S., & Osawa, M. (2010). Nationwide survey (incidence, clinical course, prognosis) of Rasmussen's encephalitis. Brain and Development, 32(6), 445-453. https://doi.org/10.1016/j.braindev.2009.10.004