Shingo Numoto1, Hirokazu Kurahashi2, Atsushi Sato3, Masaya Kubota4, Takashi Shiihara5, Tohru Okanishi6, Ryuta Tanaka7, Ichiro Kuki8, Tetsuhiro Fukuyama9, Mitsuru Kashiwagi10, Mitsuru Ikeno11, Kazuo Kubota12, Manami Akasaka13, Masakazu Mimaki14, Akihisa Okumura2. 1. Department of Pediatrics, Aichi Medical University, 1-1 Yazako Karimata, Nagakute, Aichi, 480-1195, Japan. numoto.shingo.068@mail.aichi-med-u.ac.jp. 2. Department of Pediatrics, Aichi Medical University, 1-1 Yazako Karimata, Nagakute, Aichi, 480-1195, Japan. 3. Department of Pediatrics, The University of Tokyo Hospital, Tokyo, Japan. 4. Division of Neurology, National Center for Child Health and Development, Tokyo, Japan. 5. Department of Neurology, Gunma Children's Medical Center, Shibukawa, Gunma, Japan. 6. Department of Child Neurology, Seirei Hamamatsu General Hospital, Hamamatsu, Japan. 7. Department of Child Health, Ibaraki Pediatric Education and Training Station, University of Tsukuba, Mito, Japan. 8. Department of Pediatric Neurology, Osaka City General Hospital, Osaka, Japan. 9. Division of Neurology, Nagano Children's Hospital, Nagano, Japan. 10. Department of Pediatrics, Hirakata City Hospital, Osaka, Japan. 11. Department of Pediatrics, Faculty of Medicine, Juntendo University, Tokyo, Japan. 12. Department of Pediatrics, Gifu University Graduate School of Medicine, Gifu, Japan. 13. Department of Pediatrics, School of Medicine, Iwate Medical University, Morioka, Japan. 14. Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan.
Abstract
OBJECTIVE: We examined the clinical manifestations of acute encephalopathy (AE) and identify risk factors for AE in children with tuberous sclerosis complex (TSC). METHODS: The clinical data of 11 children with clinically diagnosed TSC associated with AE and 109 children with clinically diagnosed TSC alone aged 4 years or older were collected from 13 hospitals. RESULTS: Of the 11 children with AE, 5 had histories of febrile seizures (FS), and all had histories of febrile status epilepticus (FSE). AE developed within 24 h after fever onset in all children with seizures lasting 30 min or longer. All children developed coma after seizure cessation. Head magnetic resonance imaging (MRI) revealed widespread abnormalities in the cerebral cortex, subcortical white matter, corpus callosum, basal ganglia, and thalamus. One child died; seven had severe neurological sequelae; and the other three, mild sequelae. Logistic regression analysis revealed that a history of FSE was correlated with the development of AE. SIGNIFICANCE: AE in children with TSC was characterized by sudden onset after fever, followed by coma, widespread brain edema evident on MRI, and poor outcomes. A history of FSE was a risk factor for the development of AE.
OBJECTIVE: We examined the clinical manifestations of acute encephalopathy (AE) and identify risk factors for AE in children with tuberous sclerosis complex (TSC). METHODS: The clinical data of 11 children with clinically diagnosed TSC associated with AE and 109 children with clinically diagnosed TSC alone aged 4 years or older were collected from 13 hospitals. RESULTS: Of the 11 children with AE, 5 had histories of febrile seizures (FS), and all had histories of febrile status epilepticus (FSE). AE developed within 24 h after fever onset in all children with seizures lasting 30 min or longer. All children developed coma after seizure cessation. Head magnetic resonance imaging (MRI) revealed widespread abnormalities in the cerebral cortex, subcortical white matter, corpus callosum, basal ganglia, and thalamus. One child died; seven had severe neurological sequelae; and the other three, mild sequelae. Logistic regression analysis revealed that a history of FSE was correlated with the development of AE. SIGNIFICANCE: AE in children with TSC was characterized by sudden onset after fever, followed by coma, widespread brain edema evident on MRI, and poor outcomes. A history of FSE was a risk factor for the development of AE.
Entities:
Keywords:
Clinical neurology history; Infantile spasms; MRI; Prognosis; Status epilepticus