Nationwide survey (incidence,clinical course,prognosis) of Rasmussen’s encephalitis |
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| Authors: | 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 |
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| Institution: | 1. Department of Pediatrics, Tokyo Women’s Medical University, Tokyo, Japan;2. National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan;3. Shirasaka Clinic, Hyogo, Japan;4. Department of Pediatrics, Akita University School of Medicine, Akita, Japan;5. Division of Orthopedics and Traumatology, Medical Center for Emergency and Critical Care, Nara Prefectural Nara Hospital, Nara, Japan;6. Division of Neurology, Kanagawa Children’s Medical Center, Kanagawa, Japan;g Department of Neurosurgery, Shiga University of Medical Science, Shiga, Japan;h Department of Pediatrics, Epilepsy Center, Nishi-Niigata Chuo National Hospital, Shiga, Japan;i Department of Child Neurology, National Center of Neurology and Psychiatry, Tokyo, Japan;j Department of Pediatrics, Asahikawa Habilitation Center for Disabled Children, Hokkaido, Japan;k Department of Pediatrics, Asahikawa-Kosei General Hospital, Hokkaido, Japan;l Department of Pediatrics, Okazaki City Hospital, Aichi, Japan;m Department of Cellular Physiology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, Japan;n Department of Pediatrics, Osaka University, Osaka, Japan;o Department of Pediatrics, Tokyo Women’s Medical University Yachiyo Medical Center, Chiba, Japan |
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| 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. |
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| Keywords: | Rasmussen&rsquo s encephalitis Chronic localized encephalitis Intractable epilepsy Epilepsia partialis continua Immunomodulatory therapy Nationwide survey |
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