今日の臨床サポート

特発性あるいは素因性全般てんかん、特発性あるいはself-limitedの焦点てんかん

著者: 中岡健太郎1) 愛知県精神医療センター

著者: 井上有史2) 静岡てんかん・神経医療センター

監修: 永山正雄 国際医療福祉大学大学院医学研究科 脳神経内科学

著者校正/監修レビュー済:2021/09/08

概要・推奨   

疾患のポイント:
  1. てんかんは大脳ニューロンの過剰な電気的興奮によって生じるてんかん発作が繰り返される慢性の病態。
  1. 脳の器質病変による症候性てんかんと、器質病変によらず基本的には遺伝的素因を背景とする特発性てんかんに分かれる。
  1. 特発性てんかんには特発性全般てんかん(あるいは素因性全般てんかん)と特発性焦点てんかん(self-limited、つまり年齢依存性の局在関連てんかん、あるいは部分てんかん)がある。
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
中岡健太郎 : 未申告[2021年]
井上有史 : 特に申告事項無し[2021年]
監修:永山正雄 : 未申告[2021年]

病態・疫学・診察

疾患情報(疫学・病態)  
  1. てんかんは大脳ニューロンの過剰な電気的興奮によって生じるてんかん発作が繰り返される慢性の病態。
  1. 脳の器質病変による症候性てんかんと、器質病変によらず基本的には遺伝的素因を背景とする特発性てんかんに分かれる。
  1. 特発性てんかんには特発性全般てんかん(あるいは素因性全般てんかん)と特発性焦点てんかん(self-limited、つまり年齢依存性の局在関連てんかん、あるいは部分てんかん)がある。
  1. 原則として、誘因なく2回以上発作が反復する(あるいは反復の蓋然性が高い)場合に治療を開始する[1]
問診・診察のポイント  
  1. 特発性てんかんにはそれぞれ特有の発作症状や好発年齢がある。<図表>

これより先の閲覧には個人契約のトライアルまたはお申込みが必要です。

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文献 

著者: W A Hauser, S S Rich, J R Lee, J F Annegers, V E Anderson
雑誌名: N Engl J Med. 1998 Feb 12;338(7):429-34. doi: 10.1056/NEJM199802123380704.
Abstract/Text BACKGROUND: Patients with a single unprovoked seizure have about a 35 percent risk of recurrence in the subsequent five years. We studied the risk of recurrence after two unprovoked seizures.
METHODS: We prospectively followed 204 patients with a first unprovoked seizure from the day of the initial seizure. Information was obtained from patients (and verified by a review of their medical records) about the dates and circumstances of any subsequent seizures. The risk of a second, third, and fourth seizure was estimated by the Kaplan-Meier method.
RESULTS: Of the 204 patients, 63 had a second seizure, 41 a third seizure, and 26 a fourth seizure. The mean age of the patients was 36 years, 10 percent were less than 16 years of age, 70 percent were male, 71 percent had epilepsy of unknown cause, and 66 percent had generalized seizures. The risk of a second unprovoked seizure was 33 percent. Among those with a second seizure, the risk of a third unprovoked seizure was 73 percent; among those with a third unprovoked seizure, the risk of a fourth was 76 percent. Most recurrences occurred within one year of the second or third seizure. The risk of a third seizure was higher in those with a presumed cause of epilepsy (relative risk, 1.9; 95 percent confidence interval, 1.0 to 3.4).
CONCLUSIONS: Although only about one third of patients with a first unprovoked seizure will have further seizures within five years, about three quarters of those with two or three unprovoked seizures have further seizures within four years.

PMID 9459646  N Engl J Med. 1998 Feb 12;338(7):429-34. doi: 10.1056/N・・・
著者:
雑誌名: Epilepsia. 1989 Jul-Aug;30(4):389-99.
Abstract/Text
PMID 2502382  Epilepsia. 1989 Jul-Aug;30(4):389-99.
著者: Tracy Glauser, Elinor Ben-Menachem, Blaise Bourgeois, Avital Cnaan, David Chadwick, Carlos Guerreiro, Reetta Kalviainen, Richard Mattson, Emilio Perucca, Torbjorn Tomson
雑誌名: Epilepsia. 2006 Jul;47(7):1094-120. doi: 10.1111/j.1528-1167.2006.00585.x.
Abstract/Text PURPOSE: To assess which antiepileptic medications (AEDs) have the best evidence for long-term efficacy or effectiveness as initial monotherapy for patients with newly diagnosed or untreated epilepsy.
METHODS: A 10-member subcommission of the Commission on Therapeutic Strategies of The International League Against Epilepsy (ILAE), including adult and pediatric epileptologists, clinical pharmacologists, clinical trialists, and a statistician evaluated available evidence found through a structured literature review including MEDLINE, Current Contents and the Cochrane Library for all applicable articles from 1940 until July 2005. Articles dealing with different seizure types (for different age groups) and two epilepsy syndromes were assessed for quality of evidence (four classes) based on predefined criteria. Criteria for class I classification were a double-blind randomized controlled trial (RCT) design, >or=48-week treatment duration without forced exit criteria, information on >or=24-week seizure freedom data (efficacy) or >or=48-week retention data (effectiveness), demonstration of superiority or 80% power to detect a RESULTS: A total of 50 RCTs and seven meta-analyses contributed to the analysis. Only four RCTs had class I evidence, whereas two had class II evidence; the remainder were evaluated as class III evidence. Three seizure types had AEDs with level A or level B efficacy and effectiveness evidence as initial monotherapy: adults with partial-onset seizures (level A, carbamazepine and phenytoin; level B, valproic acid), children with partial-onset seizures (level A, oxcarbazepine; level B, None), and elderly adults with partial-onset seizures (level A, gabapentin and lamotrigine; level B, None). One adult seizure type [adults with generalized-onset tonic-clonic (GTC) seizures], two pediatric seizure types (GTC seizures and absence seizures), and two epilepsy syndromes (benign epilepsy with centrotemporal spikes and juvenile myoclonic epilepsy) had no AEDs with level A or level B efficacy and effectiveness evidence as initial monotherapy.
CONCLUSIONS: This evidence-based guideline focused on AED efficacy or effectiveness as initial monotherapy for patients with newly diagnosed or untreated epilepsy. The absence of rigorous comprehensive adverse effects data makes it impossible to develop an evidence-based guideline aimed at identifying the overall optimal recommended initial-monotherapy AED. There is an especially alarming lack of well-designed, properly conducted RCTs for patients with generalized seizures/epilepsies and for children in general. The majority of relevant existing RCTs have significant methodologic problems that limit their applicability to this guideline's clinically relevant main question. Multicenter, multinational efforts are needed to design, conduct and analyze future clinically relevant RCTs that can answer the many outstanding questions identified in this guideline. The ultimate choice of an AED for any individual patient with newly diagnosed or untreated epilepsy should include consideration of the strength of the efficacy and effectiveness evidence for each AED along with other variables such as the AED safety and tolerability profile, pharmacokinetic properties, formulations, and expense. When selecting a patient's AED, physicians and patients should consider all relevant variables and not just efficacy and effectiveness.

PMID 16886973  Epilepsia. 2006 Jul;47(7):1094-120. doi: 10.1111/j.1528・・・
著者: Tracy Glauser, Elinor Ben-Menachem, Blaise Bourgeois, Avital Cnaan, Carlos Guerreiro, Reetta Kälviäinen, Richard Mattson, Jacqueline A French, Emilio Perucca, Torbjorn Tomson, ILAE Subcommission on AED Guidelines
雑誌名: Epilepsia. 2013 Mar;54(3):551-63. doi: 10.1111/epi.12074. Epub 2013 Jan 25.
Abstract/Text The purpose of this report was to update the 2006 International League Against Epilepsy (ILAE) report and identify the level of evidence for long-term efficacy or effectiveness for antiepileptic drugs (AEDs) as initial monotherapy for patients with newly diagnosed or untreated epilepsy. All applicable articles from July 2005 until March 2012 were identified, evaluated, and combined with the previous analysis (Glauser et al., 2006) to provide a comprehensive update. The prior analysis methodology was utilized with three modifications: (1) the detectable noninferiority boundary approach was dropped and both failed superiority studies and prespecified noninferiority studies were analyzed using a noninferiority approach, (2) the definition of an adequate comparator was clarified and now includes an absolute minimum point estimate for efficacy/effectiveness, and (3) the relationship table between clinical trial ratings, level of evidence, and conclusions no longer includes a recommendation column to reinforce that this review of efficacy/evidence for specific seizure types does not imply treatment recommendations. This evidence review contains one clarification: The commission has determined that class I superiority studies can be designed to detect up to a 20% absolute (rather than relative) difference in the point estimate of efficacy/effectiveness between study treatment and comparator using an intent-to-treat analysis. Since July, 2005, three class I randomized controlled trials (RCT) and 11 class III RCTs have been published. The combined analysis (1940-2012) now includes a total of 64 RCTs (7 with class I evidence, 2 with class II evidence) and 11 meta-analyses. New efficacy/effectiveness findings include the following: levetiracetam and zonisamide have level A evidence in adults with partial onset seizures and both ethosuximide and valproic acid have level A evidence in children with childhood absence epilepsy. There are no major changes in the level of evidence for any other subgroup. Levetiracetam and zonisamide join carbamazepine and phenytoin with level A efficacy/effectiveness evidence as initial monotherapy for adults with partial onset seizures. Although ethosuximide and valproic acid now have level A efficacy/effectiveness evidence as initial monotherapy for children with absence seizures, there continues to be an alarming lack of well designed, properly conducted epilepsy RCTs for patients with generalized seizures/epilepsies and in children in general. These findings reinforce the need for multicenter, multinational efforts to design, conduct, and analyze future clinically relevant adequately designed RCTs. When selecting a patient's AED, all relevant variables and not just efficacy and effectiveness should be considered.

Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.
PMID 23350722  Epilepsia. 2013 Mar;54(3):551-63. doi: 10.1111/epi.1207・・・

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