Neuropediatrics 2006; 37 - THP121
DOI: 10.1055/s-2006-945944

MULTI-INSTITUTIONAL COLLABORATIVE STUDY OF RASMUSSEN ENCEPHALITIS IN JAPAN -RETROSPECTIVE CLINICAL AND THERAPEUTIC ANALYSIS

A Muto 1, H Oguni 1, Y Takahashi 2, Y Shirasaka 3, Y Sawaishi 4, T Yano 4, T Hoshida 5, H Osaka 6, S Nakasu 7, N Akasaka 8, S Takahashi 9, A Miyamoto 10, M Osawa 1
  • 1Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan
  • 2National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan
  • 3Shirasaka Clinic, Hyogo, Japan
  • 4Department of Pediatrics, Akita University School of Medicine, Akita, Japan
  • 5Division of Orthopedics and Traumatology, Medical Center for Emergency and Critical Care, Nara Prefectural Nara Hospital, Nara, Japan
  • 6Division of Neurology, Kanagawa Children's Medical Center, Kanagawa, Japan
  • 7Department of Neurosurgery, Shiga University of Medical Science, Shiga, Japan
  • 8Department of Pediatrics, Epilepsy Center, Nishi-Niigata Chuo National Hospital, Shiga, Japan
  • 9Department of Pediatrics, Asahikawa-Kosei general Hospital, Hokkaido, Japan
  • 10Department of Pediatrics, Asahikawa Habilitation Center for Disabled Children, Hokkaido, Japan

Objectives: Rasmussen's syndrome (RS) is characterized by intractable partial epilepsy and slowly progressive neurological deficits affecting only one side of the body due to chronic localized encephalitis. Attention has progressively been paid to the immunological treatment, since evidence of chronic viral and immune-mediated inflammatory process underlying this disorder has been accumulated since 1980's. We retrospectively studied the long-term clinical course and treatments in Japanese patients with RS collecting from eight medical facilities.

Methods: The subjects were 16 patients including 5 males and 11 females. All of them satisfied the clinical and neuroimaging criteria of RS including 4 pathologically proven cases. The medical records, EEG and neuroimaging were retrospectively reviewed.

Results: The age at epilepsy onset ranged from 1.5 year to 13 years. All patients had lateralized focal motor seizures including 10 patients with EPC. 14 patients had hemiparesis. The various types of immunological treatment were given for 15 patients including prednisolone (PSL) in 5, intravenous immunoglobulin (IVIG) in 3 and both PSL and IVIG in 5. Although the follow-up period was variable, the relatively good seizure control has been maintained in 4 patients receiving IVIG with or without a combination of PSL. Finally, 7 patients underwent the surgical treatment (hemispherectomy 2, focal resective surgery 5) and 2 of them have maintained worth-while improvement.

Conclusion: The 16 cases were subclassified into 9 with typical rapidly progressive type, 6 with slowly progressive type and 1 with atypical one. IVIG with or without a combination of PSL appeared to be most effective and may be tried first.