今日の臨床サポート

群発頭痛

著者: 今井昇 静岡赤十字病院 脳神経内科

監修: 高橋裕秀 昭和大学藤が丘病院 脳神経内科

著者校正/監修レビュー済:2020/05/14
参考ガイドライン:
  1. 日本頭痛学会(http://www.jhsnet.org/guideline.html):慢性頭痛の診療ガイドライン2013、群発頭痛の在宅酸素療法(HOT)ガイドライン
患者向け説明資料

概要・推奨   

  1. 群発頭痛の診断はICHD-3を用いて行う(推奨度1)。
  1. 群発頭痛の発作に対する第1選択薬として、わが国では、スマトリプタン 皮下注射が強く推奨される(推奨度1)。
  1. 群発頭痛の発作に対し90%より高濃度の酸素を7L/分、フェイスマスクを用い15分間吸入させる治療法が強く推奨される(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:
今井昇 : 特に申告事項無し[2021年]
監修:高橋裕秀 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 群発頭痛に対する在宅酸素療法の保険適用が承認された。これに伴い「群発頭痛の在宅酸素療法(HOT)ガイドラインが作成され、酸素療法の内容を修正した。
  1. 国際頭痛分類がICHD-3βからICHD-3に改訂され、診断基準の随伴症状の項目の「前頭部および顔面の紅潮」「耳閉感」が削除され、慢性群発頭痛の診断基準の寛解期の期間が「1カ月未満」から「3カ月未満」に変更された。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 群発頭痛の診断は、国際頭痛分類第3版(ICHD-3)の診断基準を用いる。
  1. 診断基準にある、今までの発作回数、頭痛の頻度・部位・持続時間・痛みの強さ、自律神経症状の有無を確認する。
  1. 頭痛は、一側性の重度~極めて重度の頭痛が、眼窩部、眼窩上部または側頭部のいずれか1つ以上の部位に生じる。
  1. 発作持続時間はほかの1次性頭痛との鑑別に重要であり、4時間以上であれば片頭痛、15分未満であれば発作性片側頭痛または結膜充血および流涙を伴う短時間持続性片側神経痛様頭痛発作(SUNCT)を疑う。
  1. 自律神経症状には、流涙、結膜充血、鼻閉、鼻漏、眼瞼浮腫、前頭部および顔面の発汗、縮瞳、眼瞼下垂がある。
 
群発頭痛発作時の様子

左眼に結膜充血、流涙、縮瞳、眼瞼下垂を認める。

出典

img1:  Diagnosis and treatment of headache in the ambulatory care setting: a review of classic presentations and new considerations in diagnosis and management.
 
 Med Clin North Am. 2014 May;98(3):505-27・・・
 
  1. 自律神経症状を認めない場合は、「落ち着きがない、あるいは興奮した様子」の有無を確認する。
病歴・診察のポイント  
  1. 群発頭痛は、人類最悪の痛みの1つといわれるほどの激痛が持続的に起こることを特徴している。このため「焼け火箸を目に突っ込まれたような」「眼球をえぐり取られるような」と表現される独特な訴えをすることが多い。

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文献 

著者: J A van Vliet, P J E Eekers, J Haan, M D Ferrari, Dutch RUSSH Study Group
雑誌名: J Neurol Neurosurg Psychiatry. 2003 Aug;74(8):1123-5.
Abstract/Text BACKGROUND: Cluster headache (CH) is a comparatively rare, very severe primary headache. Although circumscript and recognisable criteria are available, the diagnosis is often missed or delayed. Besides, while adequate and evidence based treatment is available in diagnosed cases, CH seems to be poorly managed. The authors performed a nationwide survey among CH patients, and looked for factors involved in the diagnostic delay.
METHODS: The authors performed a nationwide mailing to all Dutch general practitioners (about 5800), and neurologists (about 560) and invited them to refer patients in whom the diagnosis CH was made or considered. Patients could also apply via the Dutch Headache Patients Society. A variety of clinical characteristics were assessed by means of questionnaires. Specifically, patients were asked about the time between their first episode and the diagnosis.
RESULTS: The IHS criteria for CH were met by 1429 of 2001 responders, and 1163 of these filled in an extended questionnaire. The male to female ratio was 3.7:1. Mean age at onset was 32 (SD 14) years. Seventy three per cent had episodic CH, 21% had chronic CH, and in 6% the periodicity was undetermined. The time between the first episode and the diagnosis ranged from 1 week to 48 years (median 3 years): 34% had consulted a dentist and 33% an ENT specialist before the diagnosis was established. Among factors that increased the diagnostic delay were the presence of photophobia or phonophobia, nausea, an episodic attack pattern and a low age at onset (p<0.01). Sex or presence of restlessness during episodes did not influence the diagnostic delay.
CONCLUSION: CH remains unrecognised or misdiagnosed in many cases for many years. Photophobia or phonophobia and nausea were in part responsible for this delay, and should be recognised as part of the clinical spectrum of CH. Many patients were first seen by a dentist or ENT specialist for their CH episodes, so more attention should be paid to educate first line physicians to recognise CH, to improve the diagnostic process and so to expose patients to earlier and better treatment of CH.

PMID 12876249  J Neurol Neurosurg Psychiatry. 2003 Aug;74(8):1123-5.
著者: Noboru Imai, Nobuyasu Yagi, Ryou Kuroda, Takashi Konishi, Masahiro Serizawa, Masahiro Kobari
雑誌名: Cephalalgia. 2011 Apr;31(5):628-33. doi: 10.1177/0333102410391486. Epub 2011 Jan 28.
Abstract/Text INTRODUCTION: This study examined the clinical profile of cluster headaches in Japan and the potential presence of features peculiar to Japan. Most previous studies of cluster headaches have focused on Caucasian populations.
METHODS: Subjects comprised 86 consecutive new cluster headache patients (68 males, 18 females; mean age, 38.4 ± 12.2 years; range, 17-73 years). Mean age at onset was 31.0 years and the ratio of males to females was 3.8:1.
RESULTS: Chronic cluster headache was observed in 3.5% of patients. More than half of patients (68.9%) reported feelings of restlessness during headache episodes and 42.9% reported restless behaviour. Patients with uncoupling of feelings of restlessness and restless behaviour forced themselves to keep still. Similar findings were reported in a Taiwanese study.
CONCLUSION: Japanese patients in this study showed a relatively low prevalence of chronic cluster headaches, and uncoupling of a sense of restlessness and restless behaviour. These features of cluster headache may be more common in Japanese and Taiwanese patients than in Caucasian patients.

PMID 21278239  Cephalalgia. 2011 Apr;31(5):628-33. doi: 10.1177/033310・・・
著者: Headache Classification Subcommittee of the International Headache Society
雑誌名: Cephalalgia. 2004;24 Suppl 1:9-160.
Abstract/Text
PMID 14979299  Cephalalgia. 2004;24 Suppl 1:9-160.
著者: Simon Law, Sheena Derry, R Andrew Moore
雑誌名: Cochrane Database Syst Rev. 2010 Apr 14;(4):CD008042. doi: 10.1002/14651858.CD008042.pub2. Epub 2010 Apr 14.
Abstract/Text BACKGROUND: Cluster headache is an uncommon, but severely painful and disabling condition, with rapid onset. Validated treatment options are limited, and first-line therapy includes inhaled oxygen. Alternative therapies such as intranasal lignocaine and ergotamine are not as commonly used and are less well studied. Triptans are successfully used to treat migraine attacks and, because of this, they may also be useful for cluster headache.
OBJECTIVES: To determine the efficacy and tolerability of triptans for the acute treatment of cluster headaches.
SEARCH STRATEGY: We searched Cochrane CENTRAL, MEDLINE and EMBASE for studies through 22 January 2010.
SELECTION CRITERIA: Randomised, double-blind, placebo-controlled studies of triptans for acute treatment of cluster headache episodes.
DATA COLLECTION AND ANALYSIS: Two review authors independently assessed study quality and extracted data. Numbers of participants with different levels of pain relief, requiring rescue medication and experiencing adverse events and headache-associated symptoms in treatment and control groups were used to calculate relative risk and numbers needed to treat (NNT) and harm (NNH).
MAIN RESULTS: All six included studies used a single dose of triptan to treat an attack of moderate to severe pain intensity. In total 231 participants received zolmitriptan 5 mg, 223 received zolmitriptan 10 mg, 131 received sumatriptan 6 mg, 88 received sumatriptan 12 mg, and 326 received placebo. Zolmitriptan was administered either orally or intranasally, and sumatriptan either subcutaneously or intranasally.Overall, the triptans studied were better than placebo for headache relief and pain-free responses, with an NNT of 2.4 for 15 minute pain relief with subcutaneous sumatriptan 6 mg (75% with sumatriptan and 32% with placebo), and 2.8 for 30 minute pain relief with intranasal zolmitriptan 10 mg (62% with zolmitriptan and 26% with placebo). Fewer participants need rescue medication with triptan than with placebo, but more experienced adverse events.
AUTHORS' CONCLUSIONS: Zolmitriptan and sumatriptan are effective in the acute treatment of cluster headaches and may provide a useful treatment option, potentially offering convenience over oxygen therapy and a better safety and tolerability profile than ergotamine. Non-oral routes of administration are likely to provide better and more rapid responses.

PMID 20393964  Cochrane Database Syst Rev. 2010 Apr 14;(4):CD008042. d・・・
著者: George J Francis, Werner J Becker, Tamara M Pringsheim
雑誌名: Neurology. 2010 Aug 3;75(5):463-73. doi: 10.1212/WNL.0b013e3181eb58c8.
Abstract/Text Cluster headache (CH) is a rare and disabling primary headache disorder. CH attacks are unilateral, short, severe headaches associated with ipsilateral autonomic symptoms that occur in a periodic fashion. We provide a systematic review and meta-analysis of existing trials of pharmacotherapy for CH and evidence-based suggestions for acute abortive treatment and preventive therapy for cluster headache. Prospective, double-blind, randomized controlled trials of any pharmacologic agent for the symptomatic relief or prevention of CH were included in this evidence-based review. The main outcomes considered were headache response and pain-free response at 15 and 30 minutes for acute treatment trials, and the cessation of CH attacks within a specific time period or the number of days on which CH attacks occurred for preventive trials. Twenty-seven trials were included in the analysis. The American Academy of Neurology quality criteria were used to assess trial quality and to grade advisements. Based on the evidence, for acute treatment of CH, Level A advice can be given for subcutaneous sumatriptan 6 mg, zolmitriptan nasal spray 5 mg and 10 mg, and 100% oxygen 6-12 L/min. Level B advice can be given for sumatriptan nasal spray 20 mg and oral zolmitriptan 5 mg and 10 mg. For the prevention of CH, Level B advice can be given for intranasal civamide 100 microg daily and suboccipital steroid injections, and Level C advice can be given for verapamil 360 mg, lithium 900 mg, and melatonin 10 mg.

PMID 20679639  Neurology. 2010 Aug 3;75(5):463-73. doi: 10.1212/WNL.0b・・・
著者: A May, M Leone, J Afra, M Linde, P S Sándor, S Evers, P J Goadsby, EFNS Task Force
雑誌名: Eur J Neurol. 2006 Oct;13(10):1066-77. doi: 10.1111/j.1468-1331.2006.01566.x.
Abstract/Text Cluster headache and the other trigeminal-autonomic cephalalgias [paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome] are rare but very disabling conditions with a major impact on the patient's quality of life. The objective of this study was to give evidence-based recommendations for the treatment of these headache disorders based on a literature search and consensus amongst a panel of experts. All available medical reference systems were screened for any kind of studies on cluster headache, paroxysmal hemicrania and SUNCT syndrome. The findings in these studies were evaluated according to the recommendations of the European Federation of Neurological Societies resulting in level A, B or C recommendations and good practice points. For the acute treatment of cluster headache attacks, oxygen (100%) with a flow of at least 7 l/min over 15 min and 6 mg subcutaneous sumatriptan are drugs of first choice. Prophylaxis of cluster headache should be performed with verapamil at a daily dose of at least 240 mg (maximum dose depends on efficacy or tolerability). Although no class I or II trials are available, steroids are clearly effective in cluster headache. Therefore, the use of at least 100 mg methylprednisone (or equivalent corticosteroid) given orally or at up to 500 mg i.v. per day over 5 days (then tapering down) is recommended. Methysergide, lithium and topiramate are recommended as alternative treatments. Surgical procedures, although in part promising, require further scientific evaluation. For paroxysmal hemicranias, indomethacin at a daily dose of up to 225 mg is the drug of choice. For treatment of SUNCT syndrome, large series suggest that lamotrigine is the most effective preventive agent, with topiramate and gabapentin also being useful. Intravenous lidocaine may also be helpful as an acute therapy when patients are extremely distressed and disabled by frequent attacks.

PMID 16987158  Eur J Neurol. 2006 Oct;13(10):1066-77. doi: 10.1111/j.1・・・
著者: A Bahra, M J Gawel, J E Hardebo, D Millson, S A Breen, P J Goadsby
雑誌名: Neurology. 2000 May 9;54(9):1832-9.
Abstract/Text OBJECTIVE: To evaluate the efficacy and tolerability of oral zolmitriptan 5 mg and 10 mg and placebo in cluster headache.
METHODS: A multicenter, double-blind, randomized, three-period, crossover, outpatient study. Adult patients received placebo and zolmitriptan 5 mg and 10 mg orally for the acute treatment of episodic or chronic cluster headache. Headache intensity was rated by a five-point scale: none, mild, moderate, severe, or very severe. Patients only treated moderate to very severe headaches. The primary efficacy measure was headache response (two-point or greater reduction from baseline in the cluster headache rating scale) at 30 minutes. Secondary efficacy measures included proportion of patients with initial headache relief within 15 and 30 minutes, mild or no pain at 30 minutes, meaningful headache relief, and use of escape medication.
RESULTS: A total of 124 patients took at least one dose of study medication, with 73% having episodic and 27% chronic cluster headache. For the primary endpoint, there was a treatment-by-cluster-headache-type interaction (p = 0.0453). Therefore, results are presented separately for chronic and episodic cluster headache. In patients with episodic cluster headache, the difference between zolmitriptan 10 mg and placebo at 30 minutes reached significance (47% versus 29%; p = 0.02). Mild or no pain at 30 minutes was reported by 60%, 57%, and 42% patients treated with zolmitriptan 10 mg, zolmitriptan 5 mg, and placebo (both p CONCLUSION: Oral zolmitriptan is efficacious in episodic cluster headache.

PMID 10802793  Neurology. 2000 May 9;54(9):1832-9.

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