Lissencephaly Type I Associated with Lennox-Gastaut Syndrome in a 20-Year-old man : a Case Report
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概要
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Lissencephaly is associated with various types of intractable epilepsy. However, complication by Lennox-Gastaut syndrome is rare. We report an adult patient with Lissencephaly type I complicated by Lennox-Gastaut syndrome, along with a review of the literature. Although mild asphyxia was noted in the history of birth, there were no recognized multiple anomalies. His developmental milestones were severely delayed. Partial seizures frequently occurred at the age of 2 months. Brain CT revealed a smooth surface of the brain cortex, and so he was diagnosed with type I lissencephaly. He was treated with several kinds of oral administrations of anti-convulsants, such as phenobarbital, valproric acid, and clonazepam, but progressed into infantile spasms with West syndrome. At the age of 20, he was repeatedly hospitalized due to respiratory infection, and aspiration pneumonia with diffuse aspiration bronchitis. Deformity of the thorax and ventilation disorder associated with severe scoliosis and respiratory muscle atrophy were also noted. His epilepsy was intractable, and tonic and axial seizures repeatedly occurred for a prolonged period. On electroencephalography, a high-amplitude 1.5-Hz spike-and-slow wave complex was dominant in the frontal region, and a rapid rhythm also appeared, based on which Lennox-Gastaut syndrome was diagnosed. Epileptic surgery and tracheotomy were recommended to his parents, but they did not consent. The patient died of rapid aggravation of respiratory infection and the frequent occurrence of epileptic seizures, in addition to chronic respiratory disorder, at 20 years of age.
- 獨協医科大学の論文
著者
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Hirao Jun-ichi
Department Of Pediatrics Dokkyo Medical University
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Imataka George
獨協医科大学 医学部小児科
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George Imataka
Department Of Pediatrics1 And Rediology2 Dokkyo Medical University School Of Medicine
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George Imataka
Department Of Pediatrics 1 Neurosurgery 2 And Radiology 3 Dokkyo Medical University School Of Medici
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Imataka George
Department Of Pediatrics Dokkyo Medical University School Of Medicine
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Osamu Arisaka
Department Of Pediatrics1 And Rediology2 Dokkyo Medical University School Of Medicine
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Arisaka O
Department Of Pediatrics Dokkyo Medical University School Of Medicine
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Arisaka Osamu
Dep. Of Pediatrics Dokkyo Medical Univ.
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Arisaka Osamu
Department Of Pediatrics Dokkyo University School Of Medicine
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HIRABAYASHI HIDEAKI
Department of Otorhinolaryngology and Bronchoesophagology, Dokkyo University School of Medicine
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YAMANOUCHI HIDEO
Department of Pediatrics, Dokkyo University School of Medicine
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Mitsui Masahko
Department of Pediatrics, Dokkyo Medical University Tochigi
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Konno Wataru
Department of Otorhinolaryngology and Bronchoesophagology, Dokkyo Medical University
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Yamanouchi Hideo
Department Of Pediatrics Dokkyo Medical University
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Yamanouchi Hideo
Department Of Child Neurology National Center Hospital For Mental Mervous And Muscular Disorders Ncn
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Konno Wataru
Department Of Otorhinolaryngology And Bronchoesophagology Dokkyo Medical University
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Mitsui Masahiko
Department Of Pediatrics Dokkyo Medical University
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Arisaka Osamu
Department Of Pediatrics 1 Neurosurgery 2 And Radiology 3 Dokkyo Medical University School Of Medici
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Imataka George
Department Of Pediatrics 1 Neurosurgery 2 And Radiology 3 Dokkyo Medical University School Of Medici
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Osamu Arisaka
Department Of Pediatrics 1 Neurosurgery 2 And Radiology 3 Dokkyo Medical University School Of Medici
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Hirabayashi Hideaki
Department Of Otorhinolaryngology And Bronchoesophagology Dokkyo Medical University
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Arisaka Osamu
Department of Pediatrics, Dokkyo Medical University School of Medicine
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Arisaka Osamu
Department of Pediatrics ,Dokkyo Medical University School of Medicine
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Imataka George
Department of Pediatrics ,Dokkyo Medical University School of Medicine
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Negishi Masaho
Department of Pediatrics, Dokkyo Medical University
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