Clinical analysis of catastrophic epilepsy in infancy and early childhood: Results of the Far-East Asia Catastrophic Epilepsy (FACE) study group |
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| Authors: | Hirokazu Oguni Taisuke Otsuki Katsuhiro Kobayashi Yushi Inoue Eiji Watanabe Kenji Sugai Akio Takahashi Shinichi Hirose Shigeki Kameyama Hitoshi Yamamoto Shinichiro Hamano Koichi Baba Hiroshi Baba Seung-Chyul Hong Heung-Dong Kim Hoon-Chul Kang Guoming Luan Tai-Tong Wong |
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| Affiliation: | 1. Department of Pediatrics, Tokyo Women’s Medical University, Tokyo, Japan;2. Department of Neurosurgery, National Center Hospital of Neurology and Psychiatry, Kodaira, Japan;3. Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital, Okayama, Japan;4. National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan;5. Department of Neurosurgery, Jichi Medical University, Tochigi, Japan;6. Department of Pediatric Neurology, National Center Hospital of Neurology and Psychiatry, Kodaira, Japan;g Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan;h Department of Functional Neurosurgery, Epilepsy Center, Nishi-Niigata Chuo National Hospital, Niigata, Japan;i Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Japan;j Department of Neurology, Saitama Children’s Medical Center, Japan;k Department of Neurosurgery, National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan;l Department of Neurosurgery, National Nagasaki Medical Center, Nagasaki, Japan;m Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University, Seoul, Republic of Korea;n Department of Pediatrics, Pediatric Epilepsy Clinics, Severance Children’s Hospital, Epilepsy Research Center, Yonsei University College of Medicine, Seoul, Republic of Korea;o Department of Neurosurgery, Sanbo Brain Institute, Beijing, China;p Department of Neurosurgery, Taipei Veterans General Hospital and School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC |
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| Abstract: | Purpose: We studied children younger than 6 years old who developed catastrophic epilepsy and were registered in the FACE study group to clarify their clinical characteristics and prevalence of seizure as well as epilepsy types. Subjects: Subjects were prospectively recruited from children with epilepsy who satisfied the following criteria and underwent intensive examination between 2009 and 2012 in 14 collaborative centers: (1) younger than 6 years old and (2) more than 10 seizures/month refractory to all available medical treatments including ACTH therapy, leading to significant psychosocial morbidity. Methods: We analyzed epilepsy onset age, predominant seizure type, etiology, neuropsychological findings, and syndromic classification according to the pre-determined registration format. Results: A total of 314 children were enrolled in this study. Epilepsy onset age in 239 cases (80%) was younger than 12 months. The most frequent seizure type was epileptic spasms (ES), followed by generalized tonic seizures (GTS), which accounted for 42% and 20%, respectively. West syndrome (WS) was the most frequent epileptic syndrome and accounted for 37%, followed by unclassified epilepsy at 21%, neocortical epilepsy at 19%, Lennox–Gastaut syndrome at 12%, Dravet syndrome at 4%, Rasmussen syndrome at 2%, and others. The two most frequent causes of epilepsy were cortical dysplasia and chromosomal anomalies, as shown in 16% and 6%, respectively. However, the etiology of nearly one half of all patients remained unknown. Psychomotor development was already worse than a moderate degree in 62% of subjects at the first examination. Conclusion: The highest proportion of catastrophic epilepsy was WS and its related syndromes featuring ES and GTS, followed by neocortical epilepsy, whose psychomotor development was significantly retarded at examinations. |
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| Keywords: | Catastrophic epilepsy Young children West syndrome Etiology Epilepsy surgery Classification |
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