今日の臨床サポート

症候性てんかん

著者: 井上有史 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:2020/11/06
参考ガイドライン:
  1. てんかん診療ガイドライン2018
患者向け説明資料

概要・推奨   

  1. てんかんの原因には構造性、素因性、感染性、代謝性、免疫性、および原因不明がある。これらの原因を年齢ごとに鑑別していくことが推奨される。なお、特発性てんかんは素因性に含まれる。特発性てんかんを鑑別することが重要である(推奨度1)
  1. てんかん発作は焦点(部分)発作と全般発作に分けられる。それぞれにより治療方針が異なるため、問診にてどちらの発作なのかを把握することが推奨される(推奨度1)
  1. てんかんの診断において脳波検査は最も有用な検査である。しかし、1回の通常脳波検査だけでは診断が可能でない場合もあり、睡眠賦活を含めた複数回の脳波検査が必要となる(推奨度1)
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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:
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]

改訂のポイント:
  1. 定期レビューを行い、てんかん診療ガイドライン2018および国際抗てんかん連盟の新しいてんかん・発作分類(2017)にしたがって加筆修正を行った。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 症候性てんかんとは、脳器質病変のある(あるいは想定される)てんかんで、特発性てんかんに対するものである。脳の形成異常や獲得性脳病変を含む。
  1. 急性の脳侵襲で生じる急性症候性発作は、慢性の病態である症候性てんかんとは区別される。
  1. 症候性てんかんはてんかんの60%で、あらゆる年齢層で生じる。なお、60歳以降のてんかん有病率は1.5%で、加齢に伴い増加する。
  1. 成人の症候性てんかんでは焦点てんかんが多いが、小児期には West症候群(ウェスト症候群、点頭てんかん)や Lennox-Gastaut 症候群(レノックス・ガストー症候群)などの全般てんかんもある。
  1. 原則として、誘因なく2回以上反復する(あるいは反復の蓋然性が高い)場合に治療を開始する。
病歴・診察のポイント  
  1. 発作はけいれん発作(強直間代発作)とは限らない。自律神経症状や、知覚、運動、認知の変容を前兆として自覚することがある(意識の保たれた発作)。意識を失って行動が変化する(口部や身振りの自動症などを伴う)意識減損発作が多い。小児期には欠神発作、スパスム、強直発作、ミオクロニー発作などの全般発作も多い。

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文献 

著者: A Krumholz, S Wiebe, G Gronseth, S Shinnar, P Levisohn, T Ting, J Hopp, P Shafer, H Morris, L Seiden, G Barkley, J French, Quality Standards Subcommittee of the American Academy of Neurology, American Epilepsy Society
雑誌名: Neurology. 2007 Nov 20;69(21):1996-2007. doi: 10.1212/01.wnl.0000285084.93652.43.
Abstract/Text OBJECTIVE: The Quality Standards Subcommittee of the American Academy of Neurology develops practice parameters as strategies for patient care based on analysis of evidence. For this practice parameter the authors reviewed available evidence relevant to evaluating adults presenting with an apparent unprovoked first seizure.
METHODS: Relevant questions were defined and addressed by multiple searches of medical literature. Each article was then reviewed, abstracted, and classified using an established evidence scoring system. Conclusions and recommendations were based on a standard three-tiered scheme of evidence classification.
RESULTS: For adults presenting with a first seizure, a routine EEG revealed epileptiform abnormalities in approximately 23% of patients, and these were predictive of seizure recurrence. A brain imaging study (CT or MRI) was significantly abnormal in 10% of patients, indicating a possible seizure etiology. Laboratory tests such as blood counts, blood glucose, and electrolyte panels were abnormal in up to 15% of individuals, but abnormalities were minor and did not cause the seizure. Overt clinical signs of infection such as fever typically predicted significant CSF abnormalities on lumbar puncture. Toxicology screening studies were limited, but report some positive tests. Recommendations: EEG should be considered as part of the routine neurodiagnostic evaluation of adults presenting with an apparent unprovoked first seizure (Level B). Brain imaging with CT or MRI should be considered as part of the routine neurodiagnostic evaluation of adults presenting with an apparent unprovoked first seizure (Level B). Laboratory tests, such as blood counts, blood glucose, and electrolyte panels (particularly sodium), lumbar puncture, and toxicology screening may be helpful as determined by the specific clinical circumstances based on the history, physical, and neurologic examination, but there are insufficient data to support or refute recommending any of these tests for the routine evaluation of adults presenting with an apparent first unprovoked seizure (Level U).

PMID 18025394  Neurology. 2007 Nov 20;69(21):1996-2007. doi: 10.1212/0・・・
著者: Demetrios Velis, Perrine Plouin, Jean Gotman, Fernando Lopes da Silva, ILAE DMC Subcommittee on Neurophysiology
雑誌名: Epilepsia. 2007 Feb;48(2):379-84. doi: 10.1111/j.1528-1167.2007.00920.x.
Abstract/Text The purpose of this paper is to update the state of knowledge with respect to long-term monitoring (LTM) in epilepsy and to formulate recommendations regarding the application of LTM in clinical practice. LTM is an established technique in use both in a hospital setting and, increasingly, in an ambulatory and more recently in a community-based setting. There has been sufficient evidence to substantiate the claim that LTM is of crucial importance in documenting electroclinical correlations both in epilepsy and in paroxysmally occurring behavioral changes often mistaken for epilepsy. Internationally recognized neurophysiological equipment standards, data acquisition and data transfer protocols and widely accepted safety standards have made widespread access to LTM facilities in epilepsy possible. Recommendations on efficient and effective use of resources as well as regarding training and competencies for personnel involved in LTM in epilepsy have been formulated. The DMC Neurophysiology Subcommittee of the ILAE recommends use of hospital-based LTM in the documentation of seizures including its application for assessing seizure type and frequency, in the evaluation of status epilepticus, in noninvasive and invasive video/EEG investigations for epilepsy surgery and for the differential diagnosis between epilepsy and paroxysmally occurring nonepileptic conditions, in children and in adults. Ambulatory outpatient and community-based LTM may be used as a substitute for inpatient LTM in cases where the latter is not cost-effective or feasible or when activation procedures aimed at increasing seizure yield are not indicated. However, outpatient ambulatory monitoring may be less informative than is inpatient monitoring in some cases because: (1) reduction of medication to provoke seizures may not be safe as an outpatient; (2) faulty electrode contacts cannot quickly be noticed and repaired; (3) the patient may move out of video surveillance; and (4) duration of ambulatory monitoring can be limited by technical constraints.

PMID 17295634  Epilepsia. 2007 Feb;48(2):379-84. doi: 10.1111/j.1528-1・・・
著者: A Marson, A Jacoby, A Johnson, L Kim, C Gamble, D Chadwick, Medical Research Council MESS Study Group
雑誌名: Lancet. 2005 Jun 11-17;365(9476):2007-13. doi: 10.1016/S0140-6736(05)66694-9.
Abstract/Text BACKGROUND: The relative risks and benefits of starting or withholding antiepileptic drug treatment in patients with few or infrequent seizures are unclear. We sought to compare policies of immediate versus deferred treatment in such patients and to assess the effects of these policies on short-term recurrence and long-term outcomes.
METHODS: We undertook an unmasked, multicentre, randomised study of immediate and deferred antiepileptic drug treatment in 1847 patients with single seizures and early epilepsy. Outcomes comprised time to first, second, and fifth seizures; time to 2-year remission; no seizures between years 1 and 3 and between years 3 and 5 after randomisation; and quality of life. Analysis was by intention to treat.
FINDINGS: 404 patients invited to join the trial did not consent to randomisation; 722 were subsequently assigned immediate treatment with antiepileptic drugs and 721 were assigned deferred treatment. Immediate treatment increased time to first seizure (hazard ratio 1.4 [95% CI 1.2 to 1.7]), second seizure (1.3 [1.1 to 1.6]), and first tonic-clonic seizure (1.5 [1.2 to 1.8]). It also reduced the time to achieve 2-year remission of seizures (p=0.023). At 5-years follow-up, 76% of patients in the immediate treatment group and 77% of those in the deferred treatment group were seizure free between 3 and 5 years after randomisation (difference -0.2% [95% CI -5.8% to 5.5%]). The two policies did not differ with respect to quality of life outcomes or serious complications.
INTERPRETATION: Immediate antiepileptic drug treatment reduces the occurrence of seizures in the next 1-2 years, but does not affect long-term remission in individuals with single or infrequent seizures.

PMID 15950714  Lancet. 2005 Jun 11-17;365(9476):2007-13. doi: 10.1016/・・・
著者: David M Labiner, Anto I Bagic, Susan T Herman, Nathan B Fountain, Thaddeus S Walczak, Robert J Gumnit, National Association of Epilepsy Centers
雑誌名: Epilepsia. 2010 Nov;51(11):2322-33. doi: 10.1111/j.1528-1167.2010.02648.x.
Abstract/Text This document was developed by the members of the Committee to Revise the Guidelines for Services, Personnel, and Facilities at Specialized Epilepsy Centers. After discussions with the general membership they were adopted by the Board of the National Association of Epilepsy Centers. The Guidelines will be reviewed and updated when considered necessary by the Board.

PMID 20561026  Epilepsia. 2010 Nov;51(11):2322-33. doi: 10.1111/j.1528・・・
著者: L Couldridge, S Kendall, A March
雑誌名: Seizure. 2001 Dec;10(8):605-14. doi: 10.1053/seiz.2001.0652.
Abstract/Text This paper explores the background to epilepsy in terms of medical impact and psychosocial effects. The argument that information and counselling may be central to the person with epilepsy is explored. The evidence from primary research published between 1990 and 2000 investigating the information and counselling needs of people with epilepsy is appraised and synthesized. This paper seeks to answer the following questions: What are the information and counselling needs of people with epilepsy? What are the preferred formats, timing and delivery of information and counselling? What are the outcomes of information giving and counselling for people with epilepsy? The review suggests that there are unmet needs for personal and general information about epilepsy which may include individual or group education and counselling. Information related to gaining control for people with epilepsy and targeted public education may contribute to improved quality of life for people with epilepsy. Information is required which is individually relevant and could be delivered in small groups or as part of an individual counselling service. Specialist epilepsy clinics and specialist nurses can improve patient knowledge and communication and provide an effective and high quality service for people with epilepsy.

Copyright 2001 BEA Trading Ltd.
PMID 11792167  Seizure. 2001 Dec;10(8):605-14. doi: 10.1053/seiz.2001.・・・
著者: Lawrence J Hirsch, Nicolas Gaspard, Andreas van Baalen, Rima Nabbout, Sophie Demeret, Tobias Loddenkemper, Vincent Navarro, Nicola Specchio, Lieven Lagae, Andrea O Rossetti, Sara Hocker, Teneille E Gofton, Nicholas S Abend, Emily J Gilmore, Cecil Hahn, Houman Khosravani, Felix Rosenow, Eugen Trinka
雑誌名: Epilepsia. 2018 Apr;59(4):739-744. doi: 10.1111/epi.14016. Epub 2018 Feb 5.
Abstract/Text We convened an international group of experts to standardize definitions of New-Onset Refractory Status Epilepticus (NORSE), Febrile Infection-Related Epilepsy Syndrome (FIRES), and related conditions. This was done to enable improved communication for investigators, physicians, families, patients, and other caregivers. Consensus definitions were achieved via email messages, phone calls, an in-person consensus conference, and collaborative manuscript preparation. Panel members were from 8 countries and included adult and pediatric experts in epilepsy, electroencephalography (EEG), and neurocritical care. The proposed consensus definitions are as follows: NORSE is a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurological disorder, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause. FIRES is a subcategory of NORSE, applicable for all ages, that requires a prior febrile infection starting between 2 weeks and 24 hours prior to onset of refractory status epilepticus, with or without fever at onset of status epilepticus. Proposed consensus definitions are also provided for Infantile Hemiconvulsion-Hemiplegia and Epilepsy syndrome (IHHE) and for prolonged, refractory and super-refractory status epilepticus. This document has been endorsed by the Critical Care EEG Monitoring Research Consortium. We hope these consensus definitions will promote improved communication, permit multicenter research, and ultimately improve understanding and treatment of these conditions.

Wiley Periodicals, Inc. © 2018 International League Against Epilepsy.
PMID 29399791  Epilepsia. 2018 Apr;59(4):739-744. doi: 10.1111/epi.140・・・
著者: Patrick Kwan, Alexis Arzimanoglou, Anne T Berg, Martin J Brodie, W Allen Hauser, Gary Mathern, Solomon L Moshé, Emilio Perucca, Samuel Wiebe, Jacqueline French
雑誌名: Epilepsia. 2010 Jun;51(6):1069-77. doi: 10.1111/j.1528-1167.2009.02397.x. Epub 2009 Nov 3.
Abstract/Text To improve patient care and facilitate clinical research, the International League Against Epilepsy (ILAE) appointed a Task Force to formulate a consensus definition of drug resistant epilepsy. The overall framework of the definition has two "hierarchical" levels: Level 1 provides a general scheme to categorize response to each therapeutic intervention, including a minimum dataset of knowledge about the intervention that would be needed; Level 2 provides a core definition of drug resistant epilepsy using a set of essential criteria based on the categorization of response (from Level 1) to trials of antiepileptic drugs. It is proposed as a testable hypothesis that drug resistant epilepsy is defined as failure of adequate trials of two tolerated, appropriately chosen and used antiepileptic drug schedules (whether as monotherapies or in combination) to achieve sustained seizure freedom. This definition can be further refined when new evidence emerges. The rationale behind the definition and the principles governing its proper use are discussed, and examples to illustrate its application in clinical practice are provided.

PMID 19889013  Epilepsia. 2010 Jun;51(6):1069-77. doi: 10.1111/j.1528-・・・
著者: G Jannuzzi, P Cian, C Fattore, G Gatti, A Bartoli, F Monaco, E Perucca
雑誌名: Epilepsia. 2000 Feb;41(2):222-30.
Abstract/Text PURPOSE: To assess the clinical impact of monitoring serum concentrations of antiepileptic drugs (AEDs) in patients with newly diagnosed epilepsy.
METHODS: One-hundred eighty patients with partial or idiopathic generalized nonabsence epilepsy, aged 6 to 65 years, requiring initiation of treatment with carbamazepine (CBZ), valproate (VPA), phenytoin (PHT), phenobarbital (PB), or primidone (PRM) were randomly allocated to two groups according to an open, prospective parallel-group design. In one group, dosage was adjusted to achieve serum AED concentration within a target range (10-20 microg/ml for PHT, 15-40 microg/ml for PB, 4-11 microg/ml for CBZ, and 40-100 microg/ml for VPA), whereas in the other group, dosage was adjusted on clinical grounds. Patients were followed up for 24 months or until a change in therapeutic strategy was clinically indicated.
RESULTS: Baseline characteristics did not differ between the two groups. Most patients with partial epilepsy were treated with CBZ, whereas generalized epilepsies were most commonly managed with PB or VPA. PHT was used only in a small minority of patients. A total of 116 patients completed 2-year follow-up, and there were no differences in exit rate from any cause between the monitored group and the control group. The proportion of assessable patients with mean serum drug levels outside the target range (mostly below range) during the first 6 months of the study was 8% in the monitored group compared with 25% in the control group (p < 0.01). There were no significant differences between the monitored group and the control group with respect to patients achieving 12-month remission (60% vs. 61%), patients remaining seizure free since initiation of treatment (38% vs. 41%), and time to first seizure or 12-month remission. Frequency of adverse effects was almost identical in the two groups.
CONCLUSIONS: Only a small minority of patients were treated with PHT, the drug for which serum concentration measurements are most likely to be useful. With the AEDs most commonly used in this study, early implementation of serum AED level monitoring did not improve overall therapeutic outcome. and the majority of patients could be satisfactorily treated by adjusting dose on clinical grounds. Monitoring the serum levels of these drugs in selected patients and in special situations is likely to be more rewarding than routine measurements in a large clinic population.

PMID 10691121  Epilepsia. 2000 Feb;41(2):222-30.
著者:
雑誌名: Epilepsia. 1993 Jul-Aug;34(4):592-6.
Abstract/Text
PMID 8330566  Epilepsia. 1993 Jul-Aug;34(4):592-6.
著者: Ettore Beghi, Arturo Carpio, Lars Forsgren, Dale C Hesdorffer, Kristina Malmgren, Josemir W Sander, Torbjorn Tomson, W Allen Hauser
雑誌名: Epilepsia. 2010 Apr;51(4):671-5. doi: 10.1111/j.1528-1167.2009.02285.x. Epub 2009 Sep 3.
Abstract/Text PURPOSE: To consider the definition of acute symptomatic seizures for epidemiological studies, and to refine the criteria used to distinguish these seizures from unprovoked seizures for specific etiologies.
METHODS: Systematic review of the literature and of epidemiologic studies.
RESULTS: An acute symptomatic seizure is defined as a clinical seizure occurring at the time of a systemic insult or in close temporal association with a documented brain insult. Suggestions are made to define acute symptomatic seizures as those events occurring within 1 week of stroke, traumatic brain injury, anoxic encephalopathy, or intracranial surgery; at first identification of subdural hematoma; at the presence of an active central nervous system (CNS) infection; or during an active phase of multiple sclerosis or other autoimmune diseases. In addition, a diagnosis of acute symptomatic seizure should be made in the presence of severe metabolic derangements (documented within 24 h by specific biochemical or hematologic abnormalities), drug or alcohol intoxication and withdrawal, or exposure to well-defined epileptogenic drugs.
DISCUSSION: Acute symptomatic seizures must be distinguished from unprovoked seizures and separately categorized for epidemiologic purposes. These recommendations are based upon the best available data at the time of this report. Systematic studies should be undertaken to better define the associations in question, with special reference to metabolic and toxic insults, for which the time window for the occurrence of an acute symptomatic seizure and the absolute values for toxic and metabolic dysfunction still require a clear identification.

PMID 19732133  Epilepsia. 2010 Apr;51(4):671-5. doi: 10.1111/j.1528-11・・・

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