Oral Session

Oral Session 17 一般口演17

O17-1 Overlap of brain dysfunction between autism in thberous sclerosis and epileptic encephalopathy 結節性硬化症の自閉症スペクトラム症とてんかん性脳症との関連：脳機能の部分的重複 Yui Kunio（油井邦雄）¹，今高城治²，佐々木ひと美¹，岡西徹³ ¹Department of Urology and Tuberous Sclerosis Board, Fujita Hearlth University School of Medicine ²Department of Pediatrics, Dokkyo Medical University ³Seirei General Hospital, Department of Pediatric Neurology Most children with tuberous sclerosis (TSC) have co-occurrence of seizures and autism spectrum disorder (ASD). Association between ASD symptoms and seizure remains unclear.　This study may untangle the neurobiological bases of this association.Case 1 was a 11-year-old male. At age 13 months, he experienced epileptic spasms. From age 17 months, he received various antiepileptics, inducing seizure remission. His electroencephalography (EEG) revealed spike and wave activities at age 15 years. Everolimus, that is mTOR inhibitor for the treatment of TSC, disappeared his epileptic spasms and EEG discharge, and the core ASD symptoms.Case 2 was a ten-year-old male. At age five months, he experienced West syndrome. At age 10 years, his EEG revealed disorganized with high voltage irregular slow waves intermixed with multifocal spikes. Everolimus disappeared and autistic social impairment, but not epileptiform activities. Case 3 was a eight-year-old girl. At age four months and seven years, she experienced a relapsing-remitting of focal seizure with impaired awareness. At age seven years, her EEG revealed high voltage irregular slow waves intermixed with spikes and polyspikes (hypsarrhythmia). Everolimus improved social impairment, but not seizure. At age nine years, she received complete corpus callosotomy, disappearing seizures and EEG discharges.Case 4 was a six-year-old girl. At age six months, she exhibited infantile spasms. At age six months, she experienced focal seizure with impaired awareness. Anticonvulsants were ineffective. Her EEG showed spike-and-wave complexes. Everolimus improved ASD symptoms but not epileptic activity. Conclusion Everolimus was not effective for epileptic activity, suggesting separate brain dysfunction between ASD and epileptic activity.		O17-2 Abnormal EEG prints in Epilepsy associated with focal cortical dysplasia 限局性皮質異形成（FCD）によるてんかん症例の脳波紋のホルマント可視化―ニューロン回路網に対するグリア細胞関与の視点― Takigawa Morikuni（瀧川守國）^1,2，鹿井博文¹，今村圭介¹ ¹IZUMI Mental Hospital Kagoshima, Japan ²Kagoshima Univ. Kagoshima, Japan 右後頭葉に限局性皮質異形成（FCD）を認めたてんかんの精神病理を、著者らが開発した脳波紋という新しい情報処理解析法で、脳内ニューロン回路網のホルマント構造の異常とグリア細胞の関与という視点から検討した。尚、臨床研究における利益相反はなく症例のプライバシーに配慮して倫理委員会の承認を得てデータを記号化し,かつ本症例に関する本患および保護者に説明し発表の同意を得た。<対象と方法>症例は、成人女性30歳代の局在関連性てんかんおよび症候群（ILEA）で、迂遠思考、粘着気質、過剰な依存および不機嫌で、チーム連携支援でも対応が困難であった。本症例のEEGを経年ごとに調べると右後頭葉に限局した持続的な棘波の出現が見られた。MRIのFLAIR法で棘波部分に対応した右後頭部に高信号の限局性皮質異形性（FCD : focal cortical dysplasia）が認められた。解析法としては、著者らがウエーブレット法（時間‐周波数解析）を用いて、新しく開発した手法を声紋におけるホルマントのごとく可視化した。また、脳波紋のホルマント可視化には密度分布強調法で弱いホルマントの速波帯域のネットワーク網状構造も可視化した。<結果および考察>本症例の臨床脳波は声紋と同様に周波数のホルマント構造で表示し対照の健康成人と対比すると、α帯域のホルマント構造の制御維持が弱く断裂的で、脳波のホルマント構造の乱れが著明である。このことは、安静閉眼時と光刺激（9Hz）でも同様であった。これまで神経生理学的視点からは活動電位を発生しないグリア細胞の脳の高次機能への関与は軽視されてきた。しかし近年、グリア細胞がニューロンの単なる支持細胞ではなく高次機能を制御することも指摘されつつある。グリオーシスを伴う本症例のてんかんの精神病理を臨床の場からグリアとニューロンの相互機能の制御不全として示唆した。

O17-3 A case study of 70 years old male who suffered from epilepsy seizures and disturbance of consciousness for 5 month,and died of bacteremia てんかん発作に続き、5か月間意識障害が持続・消長したのち、菌血症で死亡した70歳男性 Ishikawa Bunnoshin（石川文之進）¹，鈴木三夫¹，茅野真男¹，石川雅枝¹，村瀬活郎¹，中村正明¹，原田元² ¹Hotokukai-Utsunomiya Hospital ²Research Institute for EEG Analysis [Object]When the case was a child, he suffered from encephalopathy and developed epilepsy and mental retardation. When he was 70 years old, he showed epileptic seizure and persistent disturbance of consciousness, and died for bacteremia 5 month later. We report the diagnosis, course of treatment and EEG analysis.[Family]None of his family showed neuropsychiatric disorders.[Medical history]When he was a child, he suffered from whooping cough and had a fever. The encephalopathy resulted in mental retardation. He graduated junior high school and worked at woodworking shop until he became 56 years old. When he was 16 years old, he developed epilepsy. When he was 20 years old, he started to cure the epilepsy. When he was 70 years old, he lost his consciousness and delirium state continued for 1 week. Anti-epileptic drugs were administered through stomach tube because oral intake was difficult. He continued to talk to himself. He was infected from CV catheter and suffered from bacteremia.[Examination findings]His EEG showed slowed down alpha wave mixed with much delta wave, and this is abnormal findings. His brain MRI showed Lacunar infarct at left hemisphere and left basal ganglia.[Conclusion]When he was a child, he suffered from whooping cough resulted in slight mental retardation. When he was 43 years old, had a thymectomy surgery for myasthenia gravis. When he was 70 years old, he had epileptic seizure and delirium state continued. He died from bacteremia. According to the decorticate state under delirium state, slight brain atrophy and slow alpha wave mixed with delta wave in EEG, we diagnosed as prolonged delirium state.(In the presentation, we respected privacy confidentiality obligations and made sufficient consideration for maintaining anonymity)