A multidisciplinary study of autosomal dominant lateral temporal lobe epilepsy (ADLTE)

2008 Fiscal Year Final Research Report

• Project/Area Number: 18590935
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: Neurology
• Research Institution: Kyoto University
• Principal Investigator: IKEDA Akio Kyoto University, 医学研究科, 准教授 (90212761)
• Co-Investigator(Kenkyū-buntansha): KAWAMATA Jun 京都大学, 医学研究科, 助教 (60360814) ; ISHIZU Kouichi 京都大学, 医学研究科, 准教授 (50314224) ; MATSUMOTO Riki 京都大学, 医学研究科, 助教 (00378754)
• Co-Investigator(Renkei-kenkyūsha): NAGAMINE Takashi 札幌医科大学, 医学部, 教授 (10231490) ; USUI Keiko 静岡てんかん, 神経医療センター, 医師 (60402872) ; KINOSHITA Masako 国立病院機構宇多野病院, 医長 (90402883)
• Project Period (FY): 2006 – 2008
• Keywords: 常染色体優性外側側頭葉てんかん / 部分てんかん / LGI1 遺伝子 / genotype / phenotype

Research Abstract

側頭葉てんかんは成人てんかんで最も多く、常染色体優性外側側頭葉てんかん(ADLTE)はイオンチャンネル以外の遺伝子(LGI1)異常が発見され、その病態を検討した.3家系でのLGI1 のアミノ酸置換を伴う点変異は、exon8 の1645G>A と1642C>T、exon7 の677T>G で、家系間と家系内で症状差を認め、外側・内側側頭葉症状、パニック発作などが混在した.FDG-PET、MRI で外側側頭葉の糖代謝低下と容量低下を示した.音刺激での誘発発作を有する患者では、全頭型脳磁場計測装置で聴覚誘発磁場振幅の異常増強を認めた.本疾患では、genotype とphenotype の相違を決定する修飾因子の存在が示唆され、今後は全国規模の多数例の解析と動物モデルでの病態研究を予定する.

Research Products

• [Journal Article] Improved cerebral function in mesial temporal lobe epilepsy after subtemporal amydgalohoppocampectomy (2009)
  • Author(s): Takaya S, Mikuni N, Mitsueda T, Satow T, Taki J, Kinoshita M, Miyamoto S, Hashimoto N, Ikeda A, Fukuyama H
  • Journal Title: Brain 132 Pages: 185-94
• [Journal Article] Negative motor seizure arising from negative motor area : is it ictal apraxia? (2009)
  • Author(s): Ikeda A, Hirasawa K, Kinoshita M, Hitomi T, Matsumoto R, Mitsueda T, Ta ki J, Mikuni N, Hori T, Fukuyama H, Hashimoto N, Shibasaki H, Takahashi R
  • Journal Title: Epilepsia (in press)
• [Journal Article] Mutations in LGI1 gene in Japanese families with autosomal dominant lateral temporal lobe epilepsy : the first report from Asian families (2009)
  • Author(s): Kawamata J, Ikeda A, Fujita Y, Usui K, Shimohama S, Takahas hi R
  • Journal Title: Epilepsia (in press)
• [Journal Article] Temporal dynamics of Japanese morphogram and syllabogram processing in the left basal temporal area studied by event-related potentials (2009)
  • Author(s): Usui K, Ikeda A, Nagamine T, Matsuhashi M, Kinoshita M, Mikuni N, Mi yamoto S, Hashimoto N, Fukuyama H, Shibasaki H
  • Journal Title: J Clin Neurophysiol (in press)
• [Journal Article] 稀発大発作と振戦様ミオクローヌスを認めたUnverricht-Lundborg 病の成人例 (2009)
  • Author(s): 近藤孝之、山中崇弘、山門穂高、川又純、冨本秀和、人見健文、池田昭夫、高橋良輔
  • Journal Title: 臨床神経 49 Pages: 43-47
• [Journal Article] Leucine-rich glioma-inactivated 1(LGI1)変異を伴う常染色体優性外側側頭葉てんかんの1 例 (2009)
  • Author(s): 藤田祐之、池田昭夫、門野賢太郎、川又純、冨本秀和、福山秀直、高橋良輔
  • Journal Title: 臨床神経学 (印刷中)
• [Journal Article] 閃光刺激誘発スパイクの自動検出 (2009)
  • Author(s): 西田茂人、杉剛直、池田昭夫、長峯隆、柴崎浩、中村政俊
  • Journal Title: 臨床神経生理学 (印刷中)
• [Journal Article] Application of the 2001 diagnostic scheme and the 2006 ILAE report of seizure and epilepsy : a feedback from the clinical practice of adult epilepsy (2008)
  • Author(s): Kinoshita M, Takahashi R, Ikeda A
  • Journal Title: Epileptic Disord 10 Pages: 206-12
• [Journal Article] Usefulness of MEG magnetometer for spike detection iinpatients with mesial temporal lobe epileptic focus (2008)
  • Author(s): Enatsu R, Mikuni N, Usui K, Matsubayashi J, Taki J, Begum T, Matsumoto R, Ikeda A, Nagamine T, Fukuyama H, Hashimoto N
  • Journal Title: Neuroimage 41 Pages: 1206-1219
• [Journal Article] Hemispheric asymmetry of the arcuate fasciculus in patients with unilateral language dominance defined by Wada test : A diffusion tensor tractography study (2008)
  • Author(s): Matsumoto R, Okada T, Mikuni N, Mitsueda T, Taki J, Sawamoto N, Hanakawa T, Miki Y Hashimoto N, Fukuyama H, Takahashi R, Ikeda A
  • Journal Title: J Neurol 255 Pages: 1703-11
• [Journal Article] 発作時にGerstmann 症候群を呈した症候性頭頂葉てんかんの1例 (2008)
  • Author(s): 下竹昭寛、藤田祐之、池田昭夫、冨本秀和、高橋潤、高橋良輔
  • Journal Title: 臨床神経 48 Pages: 208-210
• [Journal Article] 成人難治性てんかん患者におけるクロバザムおよび活性代謝物の体内動態に関する解析 (2008)
  • Author(s): 安田幸代、矢野育子、橋田亨、木下真幸子、池田昭夫、高橋良輔、乾賢一
  • Journal Title: TDM 研究 25 Pages: 165-169
• [Journal Article] Comparison between motor evoked potential recording and fiber tracking for estimating pyramidal tracts near brain tumors (2007)
  • Author(s): Mikuni N, Okada T, Nishida N, Taki J, Enatsu R, Ikeda A, Miki Y, Hanakawa T, Fukuyama H, Hashimoto N
  • Journal Title: J Neurosurg 106 Pages: 128-133
• [Journal Article] Fibers from the dorsal premotor cortex elicit motor-evoked potential in a cortical dysplasia (2007)
  • Author(s): Mikuni N, Okada T, Taki J, Matsumoto R, Nishida N, Enatsu R, Hanakawa T, Ikeda A, Miki Y, Urayama S, Fukuyama H, Hashimoto N
  • Journal Title: Neuroimage 34 Pages: 12-18
• [Journal Article] Dynamic change of proximal conduction in demyelinating neuropathies : a cervical magnetic stimulation combined with maximum voluntary contraction (2007)
  • Author(s): Hitomi T, Kaji R, Murase N, Kohara N, Mezaki T, Nodera H, Ikeda A, Shibasaki H
  • Journal Title: Clin Neurophysiol 118 Pages: 741-750
• [Journal Article] Efficacy of low-dose, add-on therapy of clobazam (CLB) is produced by its major metabolite, N-desmethyl-CLB (2007)
  • Author(s): Kinoshita M, Ikeda A, Begum T, Terada K, Shibasaki H
  • Journal Title: J Neurol Sci 263 Pages: 44-8
• [Journal Article] Altered plasticity of the human motor cortex in pakinson's disease (2006)
  • Author(s): Ueki Y, Mima T, Kotb MA, Sawada H, SaikiH, Ikeda A, Begum T, Reza F, Nagamine T, Fukuyama H
  • Journal Title: Ann Neurol 59 Pages: 60-71
• [Journal Article] Evidence of a wide distribution of negative motor areas in the perirolandic cortex (2006)
  • Author(s): Mikuni N, Ohara S, Ikeda A, Hayashi N, Nishida N, Taki J, Enatsu R, Matsumoto R, Shibasaki H, Hashimoto N
  • Journal Title: Clin Neurophysiol 117 Pages: 33-40
• [Journal Article] "Burst and slow complexes " in nonconvulsive (2006)
  • Author(s): Ikeda A, Matsui M, Hase Y, Hitomi T, Takahashi Y, Shibasaki H, Shimohama S
  • Journal Title: epileptic status. Epileptic Disord 8 Pages: 61-64
• [Journal Article] "Burst and slow complexes " in nonconvulsive (2006)
  • Author(s): Ikeda A, Matsui M, Hase Y, Hitomi T, Takahashi Y, Shibasaki H, Shimoh ama S
  • Journal Title: epileptic status. Epileptic Disord 8 Pages: 61-64
• [Journal Article] Widdess-Walshl P : Are epilepsy classifications based on epileptic syndromes and seizure types outdated (2006)
  • Author(s): Luders HO, Acharya J, Alexopoulosl A, Baumgartner, Bautista J, R. Burgess R, Carreno M, Diehl B, Dinner D1, Ebner A, Foldvary N, Godoy J, Hamer H, Ikeda A, Kallen K, Kellinghaus C, Kotagal P, Lachhwanil D, Loddenkemper T , ManI J, Matsumoto R, Moddel G, Nair D, Noachtar S, 0' Donovan CA, Rona S, Rosenow F, Schuelel S, Szabo CA, Tandon N, Tanner A
  • Journal Title: Epileptic Disord 8 Pages: 81-85
• [Journal Article] Generators and temporal succession of giant somatsensory evoked potentials epicortical recording from sensorimotor cortex (2006)
  • Author(s): Hitomi T, Ikeda A, Matsumoto R, Kinoshita M, Taki J, Usui K, Mikuni N, Nagamine T, Hashimoto N, Shibasaki H, Takahashi R
  • Journal Title: Clin Neurophysiol 117 Pages: 1481-1486
• [Journal Article] Bereitschaftspotentials recorded from the lateral part of the superior frontal gyrus in humans (2006)
  • Author(s): Ohara S, Ikeda A, Matsuhashi M, Satow T, Kunieda T, Mikuni N, Baba K, Mihara T, Miyamoto S, Shibasaki H
  • Journal Title: Neurosci Lett 399 Pages: 1-5
• [Journal Article] Low-frequency electric cortical stimulation decreases interictal and ictal activity in human epilepsy (2006)
  • Author(s): Yamamoto J, Ikeda A, Kinoshita M, Matsumoto R, Satow T, Takeshita K, Matsuhashi M, Mikuni N, Miyamoto S, Hashimoto N, Shibasaki H
  • Journal Title: Seizure 15 Pages: 520-527
• [Journal Article] Narcolepsy without cataplexy : two subtypes based on CSF hypoicretin-1/ orexin-A findings (2006)
  • Author(s): Oka Y, Inoue Y, Kanbayashi T, Kuroda K, Miyamoto M, Miyamoto T, Horiuchi F, Ikeda A, Shimizu T, Shibasaki H
  • Journal Title: Sleep 29 Pages: 1439-1443
• [Journal Article] Subtemporal hippocampectomy preserving the basal temporal language area for intractable mesial temporal lobe epilepsy- preliminary results (2006)
  • Author(s): Mikuni N, Miyamoto S, Ikeda A, Satow T, Taki J, Takahashi J, Ohigashi Y, Hashimoto N
  • Journal Title: Epilepsia 47 Pages: 1347-53
• [Journal Article] Prefrontal hypofunction in patients with intractable mesial temporal lobe epilepsy (2006)
  • Author(s): Takaya S, Hanakawa T, Hashikawa K, Ikeda A, Sawamoto N, Nagamine T, Ishizu K, Fukuyama H
  • Journal Title: Neurology 67 Pages: 1674-1676
• [Journal Article] Proposed diagnostic schema for the classification of epileptic seizures and eipilepsies (ILAE, 2001) : proposal from Japan Epilepsy Society (2006)
  • Author(s): Iinuma K, Morimoto K, Akiyama T, Ikeda A, Kurimoto M
  • Journal Title: Epilepsia 47 Pages: 1588-1591
• [Journal Article] Ipsilateral facial sensory and motor response to basal fronoto-temporal cortical stimulation : evidence suggesting direct activation of cranial nerves (2006)
  • Author(s): Begum T, Ikeda A, Matsuhashi M, Mikuni N, Miyamoto S, Hashimoto N, Nagamine T, Fukuyama H, Shibasaki H
  • Journal Title: Epilepsy Res 71 Pages: 216-222
• [Journal Article] A step-by-step resection guided by electrocorticography for nonmalignant brain tumors associated with long-term intractable epilepsy (2006)
  • Author(s): Mikuni N, Ikeda A, Takahashi JA Nozaki K, Miyamoto S, Taki W, Hashimoto N
  • Journal Title: Epilepsy and Behavior 8 Pages: 560-564
• [Journal Article] Rapid recovery from coma with multifocal PLEDs in a patient with severe dementia (2006)
  • Author(s): Begum T, Ikeda A, Yoshioka A, Sawada H, Fukuyama H, Shibasaki H
  • Journal Title: Int Med 45 Pages: 141-144
• [Journal Article] Epileptic polyopia with right temporal lobe epilepsy as studied by FDG-PET and MRI : a case report (2006)
  • Author(s): Mitsueda-Ono T, Ikeda A, Noguchi E, Takaya S, Fukuyama H, Shimohama S, Takahashi R
  • Journal Title: J Neurol Sci 247 Pages: 109-111
• [Journal Article] Heterogenous epileptogenicity and cortic function within malformation of cortical development : a case report (2006)
  • Author(s): Kinoshita M Ikeda A, Taki J, Usui K, Matsumoto R, Mikuni N, Takahashi JB, Fukuyama H, Hashimoto N, Takahashi R
  • Journal Title: J Neurol Sci 251 Pages: 129-133
• [Journal Article] 一次体性感覚野の皮質異形成にともない難治部分発作と皮質反射性ミオクローヌスを示した1例 (2006)
  • Author(s): 中川寧子、松本理器、池田昭夫、三国信啓、松橋眞生、花川隆、福山秀直、下濱俊
  • Journal Title: 臨床神経 46 Pages: 335-338
• [Journal Article] 副甲状腺機能低下症による低カルシウム血症を合併した側頭葉てんかんの1例 (2006)
  • Author(s): 竹内幸喜、池田昭夫、大塚快信、斎木英資、下濱俊、高橋良輔
  • Journal Title: 臨床神経 46 Pages: 510-512
• [Journal Article] 食後に離人症体験、おかしみの情動、失語から始まるeating epilepsyの1例 (2006)
  • Author(s): 斉木英資、池田昭夫、石倉大輔、下濱俊、高橋良輔
  • Journal Title: てんかん研究 24 Pages: 63-67
• [Presentation] 特別シンポジウム、てんかん外科と地域医療「神経内科からの観点」 (2009)
  • Author(s): 池田昭夫
  • Organizer: 第32回日本てんかん外科学会
  • Place of Presentation: 東京
  • Year and Date: 2009-01-22
• [Presentation] Magnetoencephalography(MEG)and Epileptiform Pattern (2008)
  • Author(s): Ikeda A
  • Organizer: 1st CAAE EEG and Clinicl Neurophysiological Conference, Tianjin
  • Place of Presentation: Tianjin, China
  • Year and Date: 20080925-27
• [Presentation] Movement-related changes in human cortical activity : an input from direct cortical recording, Workshop 4 (2008)
  • Author(s): Ikeda A, Nagamine T, Matsuhashi M, Taki J
  • Organizer: MEG based analysis of spontaneous cortical oscillations
  • Place of Presentation: Sapporo, Japan
  • Year and Date: 20080825-29
• [Presentation] てんかんの外科手術のための神経生理診断、教育講演 (2008)
  • Author(s): 池田昭夫
  • Organizer: 第42回日本臨床神経生理学会
  • Place of Presentation: 神戸
  • Year and Date: 2008-11-14
• [Presentation] Maturation of EEG : Development of the awake and & sleep pattern (2008)
  • Author(s): Ikeda A
  • Organizer: EEG workshop on 12th AOCN, 16th IANCON, 2008
  • Place of Presentation: New Delhi, India
  • Year and Date: 2008-10-22
• [Presentation] Normal EEG : from adlescent to elderly (2008)
  • Author(s): Ikeda A
  • Organizer: EEG workshop on 12th AOCN, 16th IANCON, 2008
  • Place of Presentation: New Delhi, India
  • Year and Date: 2008-10-22
• [Presentation] てんかんの症候学Update (2008)
  • Author(s): 池田昭夫
  • Organizer: 第42回日本てんかん学会教育講演
  • Place of Presentation: 東京
  • Year and Date: 2008-10-18
• [Presentation] てんかん治療の新たなる展開 (2008)
  • Author(s): 池田昭夫
  • Organizer: 第50回日本小児神経学会セミナー
  • Place of Presentation: 横浜
  • Year and Date: 2008-05-29
• [Presentation] Spectrum of epileptic disorders in Japan, JNS-KNA- 1st Joint Symposium, International Comparison of Neurological Disorders (2008)
  • Author(s): Ikeda A
  • Organizer: focusing on spinocerebellar atrophies and epilepsies
  • Place of Presentation: Yokohama, Japan (Symposium)
  • Year and Date: 2008-05-17
• [Presentation] てんかん診療Update、難治てんかんの最近の治療 (2008)
  • Author(s): 池田昭夫
  • Organizer: 第49回日本神経学会総会セミナー
  • Place of Presentation: 横浜
  • Year and Date: 2008-05-15
• [Presentation] 難治性てんかんの治療戦略 (2008)
  • Author(s): 池田昭夫
  • Organizer: 第28回日本脳神経外科コングレスセミナー
  • Place of Presentation: 横浜
  • Year and Date: 2008-05-09
• [Presentation] 特別セッション : てんかん外科における関連診療科との協力体制 「てんかん外科への紹介のタイミング」大学病院の神経内科の立場から (2008)
  • Author(s): 池田昭夫
  • Organizer: 第31回日本てんかん外科学会
  • Place of Presentation: 浜松
  • Year and Date: 2008-01-24
• [Book] 失神、痙攣、症候編、内科実践マニュアル (日本臨床内科医会編) (2009)
  • Author(s): 池田昭夫(分担執筆)
  • Total Pages: 30-33
  • Publisher: 日本医学出版
• [Book] てんかん、神経疾患、内科実践マニュアル (日本臨床内科医会編) (2009)
  • Author(s): 池田昭夫(分担執筆)
  • Total Pages: 590-598
  • Publisher: 日本医学出版
• [Book] Event-related potentials (ERPs) in patients with epilepsy : from current state to future prospect, Progress in Epileptic Disorders (2008)
  • Author(s): Ikeda A, Inoue Y(eds)(分担執筆)
  • Publisher: John Libbey, Paris
• [Book] Event-related potentials in epilepsy surgery (2008)
  • Author(s): Usui K, Ikeda A(分担執筆)
  • Total Pages: 858-867
  • Publisher: In : Luders HO (eds), Textbook of epilepsy surgery, Taylor and Francis Book Ltd
• [Book] Cortical mapping using evoked potentials and Bereitschafts- potentials (2008)
  • Author(s): Ikeda A, Shibasaki H(分担執筆)
  • Total Pages: 1036-1048
  • Publisher: In : Luders HO (eds), Textbook of epilepsy surgery, Informa UK Ltd
• [Book] DC recordings to localize the ictal onset zone (2008)
  • Author(s): Ikeda A(分担執筆)
  • Total Pages: 659-666
  • Publisher: In : Luders HO (eds), Textbook of epilepsy surgery, Informa UK Ltd
• [Book] Epilepsy surgery and ERPs : invasive study in epilepsy for motor function (2008)
  • Author(s): Ikeda A(分担執筆)
  • Total Pages: 204-216
  • Publisher: In : Ikeda A, Inoue Y(eds), Event-related potentials (ERPs) in patients with epilepsy : from current state to future prospect, Progress in Epileptic Disorders
• [Book] ERP study in Epilepsy : introductory remarks (2008)
  • Author(s): Luders HO, Ikeda A(分担執筆)
  • Total Pages: 3-10
  • Publisher: In : Ikeda A, Inoue Y(eds), Event-related potentials (ERPs) in patients with epilepsy : from current state to future prospect, Progress in Epileptic Disorders
• [Book] 機能性疾患、神経・運動器疾患、わかりやすい内科学(井村裕夫編)第3版 (2008)
  • Author(s): 池田昭夫(分担執筆)
  • Total Pages: 826-837
  • Publisher: 文光堂、東京
• [Book] けいれん・不随意運動、 症候・検査編、わかりやすい内科学(井村裕夫編)第3版 (2008)
  • Author(s): 池田昭夫(分担執筆)
  • Total Pages: 1315-1320
  • Publisher: 文光堂、東京