今日の臨床サポート

頭痛

著者: 竹島多賀夫 社会医療法人寿会富永病院

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:2021/03/31
参考ガイドライン:
  1. 日本頭痛学会日本神経学会:慢性頭痛の診療ガイドライン2013
患者向け説明資料

概要・推奨   

  1. 頭痛の分類と診断は、国際頭痛分類第3版International Classification of Headache Disorders 3rd Edition(ICHD-3)に準拠して診断する(推奨度1)
  1. 「人生最悪の頭痛」「これまでに経験のない頭痛」は、緊急性の高い二次性頭痛の可能性を念頭において、十分検索する必要がある(推奨度1)
  1. 片頭痛発作期急性期の特異的治療薬として、トリプタンが有効である(推奨度1)
  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。閲覧には
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:
竹島多賀夫 : 講演料(エーザイ,大塚,イーライ・リリー),研究費・助成金など(イーライ・リリー,エーザイ,大塚,アムジェン,アラガン)[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. 定期レビューを行い、新規治療薬について追記した。

病態・疫学・診察

疫学情報・病態・注意事項  
ポイント:
  1. 頭痛(英語:headache、cephalalgia)とは、頭部の一部あるいは全体の痛みの総称であり、後頭部と後頚部の境界、眼の奥の痛みも頭痛として扱う。頭皮の外傷や感染などによって生じる頭の一部の表面の痛みは通常は頭痛とはいわない。
  1. 頭痛は、熱や腹痛と同様に症状名であるが、慢性的に頭痛発作を繰り返す場合に、疾病(頭痛症)として取り扱う。
  1. 頭痛を来す原因疾患には、明白な器質的異常との関連がみられない一次性頭痛疾患と器質的異常によって頭痛が引き起こされている二次性頭痛疾患が存在する。
  1. 頭痛の診断は国際頭痛分類第3版の診断基準(ICHD-3)に沿って行う。ICHD-3は階層的な頭痛分類がなされている。プライマリケアでは、ICHD-3の頭痛グループ(2桁でコードされるレベル)、すなわち、片頭痛、緊張型頭痛、群発頭痛のレベルで診断できることが求められる。神経内科専門医、頭痛専門医は3桁のレベル、すなわち「1.2.1典型的前兆に片頭痛を伴うもの」、「1.2.2脳幹性前兆を伴う片頭痛」などの診断ができる必要がある。
  1. なお、頭痛のない頭痛性疾患も存在する。頭痛の訴えがなくとも頭痛性疾患の可能性を考慮して除外診断が必要なことがある。例えば、閃輝暗点のみで頭痛を伴わないものは、前兆のある片頭痛のサブタイプに診断される。また、小児周期性嘔吐症、腹部片頭痛、小児両性発作性めまいは、頭痛がなくとも片頭痛のサブタイプに分類される。また、脳底型片頭痛の一部では、意識障害が高度で患者は頭痛を訴えないこともある。
問診・診察のポイント  
 
問診:
  1. 頭痛の性状、部位、程度、持続時間、随伴症状(悪心、嘔吐、光過敏、音過敏、流涙、眼充血、鼻汁漏、鼻閉、眼瞼浮腫、眼瞼下垂)、前兆(閃輝暗点、感覚障害、言語障害、運動麻痺、めまい、失調など)について問診する。

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文献 

著者: P L Mayer, I A Awad, R Todor, K Harbaugh, G Varnavas, T A Lansen, P Dickey, R Harbaugh, L N Hopkins
雑誌名: Stroke. 1996 Sep;27(9):1558-63.
Abstract/Text BACKGROUND AND PURPOSE: It is not known what fraction of patients with symptomatic cerebral aneurysms are misdiagnosed at initial medical presentation. It is also not clear whether misdiagnosed patients more frequently deteriorate before definitive aneurysm diagnosis and therapy or achieve a poorer outcome than correctly diagnosed patients.
METHODS: We reviewed records of consecutive patients with symptomatic cerebral aneurysms managed by four tertiary-care neurosurgical services during a recent 19-month period. Clinical course and outcome were analyzed according to misdiagnosis or correct diagnosis at initial medical evaluation.
RESULTS: Fifty-four of 217 patients (25%) were misdiagnosed at initial medical evaluation, including 46 of 121 patients (38%) initially in good clinical condition (clinical grade 1 or 2). Forty-six of 54 patients (85%) in the misdiagnosis group were initially grade 1 or 2 compared with 75 of 163 patients (46%) with correct initial diagnosis (P < .01). Twenty-six of 54 misdiagnosed patients (48%) deteriorated or rebled before definitive aneurysm treatment compared with 4 of 165 correctly diagnosed patients (2%) (P < .001). Among patients initially presenting as clinical grade 1 or 2, overall good or excellent outcome was achieved in 91% of those with correct initial diagnosis and 53% of patients with initial misdiagnosis (P < .001). Deterioration before correct diagnosis accounted for 16 of 67 patients (24%) with poor or worse final outcome in this series.
CONCLUSIONS: Patients in good clinical condition with symptomatic cerebral aneurysms were commonly misdiagnosed. Misdiagnosed patients were more likely than correctly diagnosed patients to deteriorate clinically and had a worse overall outcome. Misdiagnosed cases accounted for a significant fraction of overall poor outcomes among consecutive cases of symptomatic aneurysms.

PMID 8784130  Stroke. 1996 Sep;27(9):1558-63.
著者: Marcelo E Bigal, Richard B Lipton
雑誌名: Headache. 2008 Jan;48(1):7-15. doi: 10.1111/j.1526-4610.2007.00969.x.
Abstract/Text Migraine is a chronic recurrent disorder with episodic manifestations that is progressive in some individuals. Migraine progresses clinically, physiologically, and anatomically. Progression may be a consequence of the mechanisms that generate the migraine attacks (eg, cortical spreading depression) or it may be a function of the activations generated by the attacks (eg, lesions in the periaqueductal gray area), a hypothesis supported by the increase in lesions with attack frequency. Progression may also be partially explained by common genetic or environmental risk factors. Finally, migraine with aura is associated with an elevated Framingham score and with risk factors for cardiovascular disease. Research on this issue is in its infancy and cautions are necessary before extrapolating this information into clinical practice.

PMID 18184280  Headache. 2008 Jan;48(1):7-15. doi: 10.1111/j.1526-4610・・・
著者: Ann Christine Lyngberg, Birthe Krogh Rasmussen, Torben Jørgensen, Rigmor Jensen
雑誌名: Neurology. 2005 Aug 23;65(4):580-5. doi: 10.1212/01.wnl.0000172918.74999.8a.
Abstract/Text OBJECTIVE: To determine the prognosis of migraine and tension-type headache and to identify prognostic factors.
METHODS: Of 740 persons (aged 25 to 64 years) examined in a 1989 Danish cross-sectional headache study, 673 were eligible for follow-up in 2001. All interviews at baseline and at follow-up were conducted by medical doctors and based on the 1988 IHS-criteria.
RESULTS: A total of 549 persons (81.6%) participated in the follow-up study. Of 64 migraineurs at baseline, 42% had experienced remission, 38% had low migraine frequency, and 20% had more than 14 migraine days per year (poor outcome) at follow-up. Poor outcome was associated with high migraine frequency at baseline and age at onset younger than 20 years. Among 146 subjects with frequent episodic tension-type headache and 15 with chronic tension-type headache at baseline, 45% experienced infrequent or no tension-type headache (remission), 39% had frequent episodic tension-type headache, and 16% experienced chronic tension-type headache (poor outcome) at follow-up. Poor outcome was associated with baseline chronic tension-type headache, coexisting migraine, not being married, and sleeping problems.
CONCLUSIONS: The prognosis of migraine, frequent episodic tension-type headache, and chronic tension-type headache was favorable.

PMID 16116119  Neurology. 2005 Aug 23;65(4):580-5. doi: 10.1212/01.wnl・・・
著者: S Kinze, M Clauss, U Reuter, T Wolf, J P Dreier, K M Einhäupl, G Arnold
雑誌名: Headache. 2001 Sep;41(8):774-8.
Abstract/Text OBJECTIVE: We evaluated the efficacy of prophylactic valproic acid treatment (6 months) on the frequency of migraine attacks and the number of migraine headache days with respect to serum levels.
BACKGROUND: Valproic acid, a GABAergic drug, has been shown to be effective for migraine prophylaxis. Results from several dose- and serum level-adjusted studies have recommended valproic acid doses within a range of 500 to 1500 mg per day for migraine prophylaxis.
DESIGN AND METHODS: In this prospective open-label study, 52 patients received valproic acid doses of 300 to 1200 mg per day; 45 patients were treated per protocol. Valproic acid serum levels increased linearly in relation to the valproic acid dose and were between 21 and 107 microg/mL at the end of the treatment period. Patients were divided into two groups: those with valproic acid serum levels less than 50 microg/mL (group 1) and those with serum levels greater than 50 microg/mL (group 2).
RESULTS: The frequency of migraine attacks was significantly reduced in group 1 from 3.5 +/- 0.9 to 2.0 +/- 0.9 attacks per month. Migraine headache days also decreased (6.4 +/- 3.5 to 4.6 +/- 2.9 days per month). In the high serum level group, a reduction of migraine attacks from 3.5 +/- 0.9 to 2.8 +/- 1.0 attacks per month and only a slight decrease in headache days (6.4 +/- 3.5 to 6.1 +/- 2.4 days per month) was observed. The outcome of group 1 (low serum level) was significantly better than that of group 2 with respect to both parameters (P<.05). Side effects were generally mild and temporary.
CONCLUSIONS: Due to the lack of additional benefit from higher valproic acid doses (more than 600 mg per day), we recommend daily valproic acid doses of 500 to 600 mg with a target serum level less than 50 microg/mL for the prophylactic treatment of migraine.

PMID 11576201  Headache. 2001 Sep;41(8):774-8.
著者: K Ghose, B Niven
雑誌名: Methods Find Exp Clin Pharmacol. 1998 May;20(4):353-9.
Abstract/Text We assessed the efficacy of sodium valproate as a prophylactic agent in migraine headache. A prospective randomized study was conducted in adult patients who previously derived no significant benefit from most conventional prophylactic therapy for migraine. Twenty-seven patients with a diagnosis of migraine with aura or migraine without aura from a headache clinic received low dose sodium valproate for 3 months. Response to therapy was defined as 50% or greater reduction in the frequency of headache. Plasma drug level monitoring helped to identify four noncompliers who were excluded from the study. Seventeen (71%) patients observed improvement within 4-6 weeks of medication and remained well for 12 weeks. They were further followed up for 12-24 months. Two patients for side effects and 1 for nondrug-related problems were withdrawn from follow-up study. Twelve patients (60%) maintained their response for 12 months or longer. Clinical improvement (percentage reduction in the frequency of migraine attacks) correlated inversely with the plasma drug levels at 13-24 months and daily dose of valproate, among the responders, suggestive of a possible therapeutic window. In other words, patients who do not respond to low dose valproate are unlikely to benefit from further increase in dosage.

PMID 9658386  Methods Find Exp Clin Pharmacol. 1998 May;20(4):353-9.
著者: Janneke Jentink, Maria A Loane, Helen Dolk, Ingeborg Barisic, Ester Garne, Joan K Morris, Lolkje T W de Jong-van den Berg, EUROCAT Antiepileptic Study Working Group
雑誌名: N Engl J Med. 2010 Jun 10;362(23):2185-93. doi: 10.1056/NEJMoa0907328.
Abstract/Text BACKGROUND: The use of valproic acid in the first trimester of pregnancy is associated with an increased risk of spina bifida, but data on the risks of other congenital malformations are limited.
METHODS: We first combined data from eight published cohort studies (1565 pregnancies in which the women were exposed to valproic acid, among which 118 major malformations were observed) and identified 14 malformations that were significantly more common among the offspring of women who had received valproic acid during the first trimester. We then assessed the associations between use of valproic acid during the first trimester and these 14 malformations by performing a case-control study with the use of the European Surveillance of Congenital Anomalies (EUROCAT) antiepileptic-study database, which is derived from population-based congenital-anomaly registries. Registrations (i.e., pregnancy outcomes with malformations included in EUROCAT) with any of these 14 malformations were compared with two control groups, one consisting of infants with malformations not previously linked to valproic acid use (control group 1), and one consisting of infants with chromosomal abnormalities (control group 2). The data set included 98,075 live births, stillbirths, or terminations with malformations among 3.8 million births in 14 European countries from 1995 through 2005.
RESULTS: Exposure to valproic acid monotherapy was recorded for a total of 180 registrations, with 122 registrations in the case group, 45 in control group 1, and 13 in control group 2. As compared with no use of an antiepileptic drug during the first trimester (control group 1), use of valproic acid monotherapy was associated with significantly increased risks for 6 of the 14 malformations under consideration; the adjusted odds ratios were as follows: spina bifida, 12.7 (95% confidence interval [CI], 7.7 to 20.7); atrial septal defect, 2.5 (95% CI, 1.4 to 4.4); cleft palate, 5.2 (95% CI, 2.8 to 9.9); hypospadias, 4.8 (95% CI, 2.9 to 8.1); polydactyly, 2.2 (95% CI, 1.0 to 4.5); and craniosynostosis, 6.8 (95% CI, 1.8 to 18.8). Results for exposure to valproic acid were similar to results for exposure to other antiepileptic drugs.
CONCLUSIONS: The use of valproic acid monotherapy in the first trimester was associated with significantly increased risks of several congenital malformations, as compared with no use of antiepileptic drugs or with use of other antiepileptic drugs.

Copyright 2010 Massachusetts Medical Society.
PMID 20558369  N Engl J Med. 2010 Jun 10;362(23):2185-93. doi: 10.1056・・・
著者: Headache Classification Committee of the International Headache Society (IHS)
雑誌名: Cephalalgia. 2013 Jul;33(9):629-808. doi: 10.1177/0333102413485658.
Abstract/Text
PMID 23771276  Cephalalgia. 2013 Jul;33(9):629-808. doi: 10.1177/03331・・・
著者: F H Linn, E F Wijdicks, Y van der Graaf, F A Weerdesteyn-van Vliet, A I Bartelds, J van Gijn
雑誌名: Lancet. 1994 Aug 27;344(8922):590-3.
Abstract/Text Retrospective surveys of patients with subarachnoid haemorrhage suggest that minor episodes with sudden headache (warning leaks) may precede rupture of an aneurysm, and that early recognition and surgery might lead to improved outcome. We studied 148 patients with sudden and severe headache (possible sentinel headache) seen by 252 general practitioners in a 5-year period in the Netherlands. Subarachnoid haemorrhage was the cause in 37 patients (25%) (proven aneurysm in 21, negative angiogram in 6, no angiogram done in 6, sudden headache followed by death in 4). 103 patients had headache as the only symptom, 12 of whom proved to have subarachnoid haemorrhage (6 with a ruptured aneurysm). Previous bouts of sudden headache had occurred in only 2. Other serious neurological conditions were diagnosed in 18. In the remaining 93, no underlying cause of headache was found; follow-up over 1 year showed no subsequent subarachnoid haemorrhage or sudden death. In this cohort, acute, severe headache in general practice indicated a serious neurological disorder in 37% (95% CI 29-45%), and subarachnoid haemorrhage in 25% (18-32%). 12% (5-18%) of those with headache as the only symptom. The notion of warning leaks as a less serious variant of subarachnoid haemorrhage is not supported by this study. Early recognition of subarachnoid haemorrhage is important but will probably have only limited impact on the outcome in the general population.

PMID 7914965  Lancet. 1994 Aug 27;344(8922):590-3.
著者: A-M Landtblom, S Fridriksson, J Boivie, J Hillman, G Johansson, I Johansson
雑誌名: Cephalalgia. 2002 Jun;22(5):354-60.
Abstract/Text Sudden onset headache is a common condition that sometimes indicates a life-threatening subarachnoid haemorrhage (SAH) but is mostly harmless. We have performed a prospective study of 137 consecutive patients with this kind of headache (thunderclap headache=TCH). The examination included a CT scan, CSF examination and follow-up of patients with no SAH during the period between 2 days and 12 months after the headache attack. The incidence was 43 per 100 000 inhabitants >18 years of age per year; 11.3% of the patients with TCH had SAH. Findings in other patients indicated cerebral infarction (five), intracerebral haematoma (three), aseptic meningitis (four), cerebral oedema (one) and sinus thrombosis (one). Thus no specific finding indicating the underlying cause of the TCH attack was found in the majority of the patients. A slightly increased prevalence of migraine was found in the non-SAH patients (28%). The attacks occurred in 11 cases (8%) during sexual activity and two of these had an SAH. Nausea, neck stiffness, occipital location and impaired consciousness were significantly more frequent with SAH but did not occur in all cases. Location in the temporal region and pressing headache quality were the only features that were more common in non-SAH patients. Recurrent attacks of TCH occurred in 24% of the non-SAH patients. No SAH occurred later in this group, nor in any of the other patients. It was concluded that attacks caused by a SAH cannot be distinguished from non-SAH attacks on clinical grounds. It is important that patients with their first TCH attack are investigated with CT and CSF examination to exclude SAH, meningitis or cerebral infarction. The results from this and previous studies indicate that it is not necessary to perform angiography in patients with a TCH attack, provided that no symptoms or signs indicate a possible brain lesion and a CT scan and CSF examination have not indicated SAH.

PMID 12110111  Cephalalgia. 2002 Jun;22(5):354-60.
著者: J A Edlow, L R Caplan
雑誌名: N Engl J Med. 2000 Jan 6;342(1):29-36. doi: 10.1056/NEJM200001063420106.
Abstract/Text
PMID 10620647  N Engl J Med. 2000 Jan 6;342(1):29-36. doi: 10.1056/NEJ・・・
著者: J van Gijn, K J van Dongen
雑誌名: Neuroradiology. 1982;23(3):153-6.
Abstract/Text We performed serial CT scans in a prospective series of 100 patients with a ruptured aneurysm who were first scanned within 2 days of the haemorrhage. In all patients the early CT scan showed evidence of extravasated blood. In 96 patients the source of bleeding was clearly at the base of the brain, and 32 of these had a haematoma. We estimated from the results of 139 repeat scans that the probability of recognizing an aneurysmal haemorrhage on CT is 85% after 5 days, 50% after 1 week, 30% after 2 weeks (mostly patients with haematomas), and almost nil after 3 weeks.

PMID 7088285  Neuroradiology. 1982;23(3):153-6.
著者: T Uchihara, H Tsukagoshi
雑誌名: Headache. 1991 Mar;31(3):167-71.
Abstract/Text We prospectively examined the clinical signs of 54 febrile patients associated with recent-onset headache. They underwent lumbar puncture (LP) on suspicion of meningitis. The relation of each sign to cerebrospinal fluid (CSF) pleocytosis was estimated. Among 34 patients with pleocytosis, 33 had jolt accentuation (sensitivity: 97.1%), while only 5 of them had neck stiffness or Kernig's sign. Among 20 patients without pleocytosis, 12 had no jolt accentuation (specificity: 60%). We found jolt accentuation to be the most sensitive sign of CSF pleocytosis. If jolt accentuation is noted in a febrile patient associated with recent onset headache, the CSF should be examined even in the absence of neck stiffness or Kernig's sign.

PMID 2071396  Headache. 1991 Mar;31(3):167-71.
著者: Jolene H Nakao, Farrukh N Jafri, Kaushal Shah, David H Newman
雑誌名: Am J Emerg Med. 2014 Jan;32(1):24-8. doi: 10.1016/j.ajem.2013.09.012. Epub 2013 Oct 16.
Abstract/Text Jolt accentuation or exacerbation of a baseline headache with horizontal rotation of the neck is a physical finding believed to assess for meningeal irritation. We conducted a prospective observational study of neurologically intact emergency department (ED) patients undergoing lumbar puncture in 2 inner city academic EDs to validate the sensitivity and specificity of jolt accentuation and to assess the sensitivity and specificity of Kernig sign, Brudzinski sign, and nuchal rigidity, in predicting cerebrospinal fluid (CSF) pleocytosis in individuals being assessed for meningitis. Adult patients 18 years and older undergoing lumbar puncture between 2006 and 2009 were approached for consent. Exclusions included inability to consent and altered mental status. Physicians were asked to answer a questionnaire of physical examination findings before receiving CSF results. The primary outcome was the presence or absence of pleocytosis, defined as greater than or equal to 5 cells/high-power field in the fourth CSF tube. We calculated descriptive statistics and tests of diagnostic accuracy. A total of 230 patients consented for participation and had CSF white blood cell counts recorded. Forty-seven individuals (20%) had pleocytosis. A total of 197 patients had headache and were, hence, eligible for jolt accentuation assessment. For pleocytosis, the sensitivity of jolt accentuation was 21%, Kernig sign was 2%, Brudzinski sign was 2%, and nuchal rigidity was 13%. The specificity of jolt accentuation was 82%, Kernig sign was 97%, Brudzinski sign was 98%, and nuchal rigidity was 80%. Jolt accentuation in our cohort was poorly predictive of pleocytosis and insensitive. The presence of Kernig sign, Brudzinski sign, or nuchal rigidity has moderate positive but no negative predictive value for pleocytosis.

© 2013.
PMID 24139448  Am J Emerg Med. 2014 Jan;32(1):24-8. doi: 10.1016/j.aje・・・
著者: Hidetaka Tamune, Hiroaki Takeya, Wakako Suzuki, Yasuaki Tagashira, Takaie Kuki, Mitsuhiro Nakamura
雑誌名: Am J Emerg Med. 2013 Nov;31(11):1601-4. doi: 10.1016/j.ajem.2013.08.028. Epub 2013 Sep 23.
Abstract/Text BACKGROUND: Meningitis is a common emergency disease. Signs and symptoms easily observed at the bedside are needed because early recognition of the possibility of meningitis is necessary for the decision to perform lumbar puncture. Jolt accentuation of headache has been reported to be the most sensitive diagnostic test; however, limited articles have reproduced its sensitivity.
METHODS: This is a single-center retrospective medical record review between 2007 and 2012. We diagnosed meningitis based on the criterion standard that cerebrospinal fluid total cells is more than 5/mm(3), in accordance with previous studies. All diagnostic and management decisions including Kernig sign, nuchal rigidity, and jolt accentuation of headache were at the physician's discretion. We calculated the sensitivity and specificity of well-known signs and symptoms of meningitis and, especially, compared the efficacy of jolt accentuation of headache with previous studies.
RESULTS: We investigated 531 adult patients who were suspected of meningitis and had lumbar puncture performed. Of these patients, 139 had meningitis. Background characteristics and vital signs were not clinically different between the 2 groups, although classic tetralogy of bacterial meningitis (fever, nuchal rigidity, mental disturbance, and headache) was worth investigated. The sensitivity and specificity of jolt accentuation of headache were 63.9% (95% confidence interval, 51.9%-76.0%) and 43.2% (34.7%-51.6%), respectively.
CONCLUSION: The absence of jolt accentuation of headache test cannot, on its own, accurately rule out meningitis in adults. Further studies are warranted to reproduce this result and to discover better bedside diagnostic tests.

© 2013.
PMID 24070978  Am J Emerg Med. 2013 Nov;31(11):1601-4. doi: 10.1016/j.・・・
著者: Miguel A Gonzalez-Gay, Maria J Lopez-Diaz, Sonia Barros, Carlos Garcia-Porrua, Amalia Sanchez-Andrade, Jose Paz-Carreira, Javier Martin, Javier Llorca
雑誌名: Medicine (Baltimore). 2005 Sep;84(5):277-90.
Abstract/Text The outcome of a patient with giant cell arteritis (GCA) is closely related to the development of severe ischemic manifestations. In the current study we analyzed the implications of routine laboratory tests obtained at the time of diagnosis in the clinical spectrum of a series of 240 consecutive patients with biopsy-proven GCA at the single hospital for a defined population. We also examined whether the laboratory markers of inflammation may be predictors of severe ischemic manifestations (visual ischemic events, cerebrovascular accidents, jaw claudication, or large-artery stenosis of the extremities of recent onset), and their potential correlation. Anemia (hemoglobin <12 g/dL) was observed in 131 (54.6%) and thrombocytosis in 117 (48.8%) patients. Sixty-eight (28.3%) patients had leukocytosis. The percentage of patients showing a significant increase of alkaline phosphatase and hypoalbuminemia was similar (25% and 27.8%, respectively). The mean values of erythrocyte sedimentation rate (ESR) and C-reactive protein were 93 +/- 23 mm/h and 94 +/- 63 mg/L, respectively. A strong correlation among most laboratory markers of inflammation was observed. Anemia was more commonly observed in patients without severe ischemic manifestations (61.5% versus 48.9% in those with severe ischemic manifestation; p = 0.05) and in patients with constitutional syndrome or fever (p < 0.001). Patients with ESR greater than 100 mm/h exhibited more commonly constitutional syndrome (p < 0.001) and had a statistically significant reduction in the incidence of visual ischemic events (p < 0.025). Only 7 (22.6%) of the 31 patients who suffered permanent visual loss had an ESR at the time of disease diagnosis greater than 100 mm/h. However, in a multivariate logistic regression analysis, only anemia was found to be a negative predictor for the development of severe ischemic manifestations of GCA (odds ratio, 0.53; 95% confidence intervals, 0.30-0.94; p = 0.03). In conclusion, our results suggest that some laboratory markers of inflammation, in particular the presence of anemia, may negatively predict the risk of severe ischemic complications in GCA patients.

PMID 16148728  Medicine (Baltimore). 2005 Sep;84(5):277-90.
著者: S Hall, S Persellin, J T Lie, P C O'Brien, L T Kurland, G G Hunder
雑誌名: Lancet. 1983 Nov 26;2(8361):1217-20.
Abstract/Text To evaluate the clinical usefulness of temporal artery biopsy in the diagnosis of giant-cell arteritis we followed up all 134 residents of Olmsted County, Minnesota, who had temporal artery biopsies between 1965 and 1980. Initial biopsies were positive for giant-cell arteritis in 46 cases and negative in 88. A history of jaw pain or claudication and the findings of a palpably abnormal temporal artery were significantly more common in the patients whose biopsy specimens showed giant-cell arteritis. Over a median follow-up period of 70 months (range 1-192) only 8 of the 88 biopsy-negative patients had clinical courses requiring long-term, high-dose corticosteroid therapy for giant-cell arteritis. In this population-based study temporal artery biopsy correctly predicted the subsequent need for corticosteroid therapy in 94% of cases: these findings indicate that biopsy should be done before patients are committed to long-term corticosteroid therapy.

PMID 6139569  Lancet. 1983 Nov 26;2(8361):1217-20.
著者: A Cifelli, L Vaithianathar
雑誌名: BMJ Case Rep. 2011 Mar 29;2011. doi: 10.1136/bcr.03.2010.2862. Epub 2011 Mar 29.
Abstract/Text A lady in her late 20s was admitted with a history of headaches and intermittent focal neurological symptoms which were greatly exacerbated by catheter angiography of the cerebral circulation. Cerebrospinal fluid analysis demonstrated a lymphocytic pleocytosis. She subsequently made a spontaneous recovery, without neurological sequelae. Her presentation fits the diagnostic criteria for the previously described syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL). HaNDL is a probably underdiagnosed nosological entity, characterised by often dramatic clinical manifestations but ultimately a good prognosis.

PMID 22700346  BMJ Case Rep. 2011 Mar 29;2011. doi: 10.1136/bcr.03.201・・・
著者: Leonard H Calabrese, David W Dodick, Todd J Schwedt, Aneesh B Singhal
雑誌名: Ann Intern Med. 2007 Jan 2;146(1):34-44.
Abstract/Text Reversible cerebral vasoconstriction syndromes (RCVS) comprise a group of diverse conditions, all characterized by reversible multifocal narrowing of the cerebral arteries heralded by sudden (thunderclap), severe headaches with or without associated neurologic deficits. Reversible cerebral vasoconstriction syndromes are clinically important because they affect young persons and can be complicated by ischemic or hemorrhagic strokes. The differential diagnosis of RCVS includes conditions associated with thunderclap headache and conditions that cause irreversible or progressive cerebral artery narrowing, such as intracranial atherosclerosis and cerebral vasculitis. Misdiagnosis as primary cerebral vasculitis and aneurysmal subarachnoid hemorrhage is common because of overlapping clinical and angiographic features. However, unlike these more ominous conditions, RCVS is usually self-limited: Resolution of headaches and vasoconstriction occurs over a period of days to weeks. In this review, we describe our current understanding of RCVS; summarize its key clinical, laboratory, and imaging features; and discuss strategies for diagnostic evaluation and treatment.

PMID 17200220  Ann Intern Med. 2007 Jan 2;146(1):34-44.
著者: Anne Ducros, Ursula Fiedler, Raphael Porcher, Monique Boukobza, Christian Stapf, Marie-Germaine Bousser
雑誌名: Stroke. 2010 Nov;41(11):2505-11. doi: 10.1161/STROKEAHA.109.572313. Epub 2010 Sep 30.
Abstract/Text BACKGROUND AND PURPOSE: Reversible cerebral vasoconstriction syndrome (RCVS), characterized by severe headaches and reversible constriction of cerebral arteries, may be associated with ischemic and hemorrhagic strokes. The aim of this study was to describe the frequency, patterns, and risk factors of intracranial hemorrhages in RCVS.
METHODS: We analyzed prospective data on 89 consecutive patients with RCVS, of which 8 were postpartum and 46 used vasoactive substances. Standard bivariate and multivariate statistical tests were applied to compare patients with and without hemorrhage.
RESULTS: Thirty patients (34%), of which 5 were postpartum and 12 used vasoactive substances, developed at least 1 type of intracranial hemorrhage, including cortical subarachnoid (n = 27), intracerebral (n = 11), and subdural hemorrhage (n=2). Patients with hemorrhage had an older age (46.6 versus 41.6 years, P = 0.049) and were more frequently females (90% versus 51%, P = 0.0017) or were migrainers (43% versus 19%, P = 0.022) than those without hemorrhage. Multivariate testing identified 2 independent risk factors of hemorrhage in RCVS: female gender (OR, 4.05; 95% CI, 1.46 to 11.2) and migraine (OR, 2.34; 95% CI, 1.06 to 5.18). Patients with hemorrhage had a greater risk of persistent focal deficits (30% versus 2%, P = 0.0002), cerebral infarction (13% versus 2%, P = 0.039), posterior reversible encephalopathy syndrome (17% versus 3%, P = 0.041) at the acute stage, and of inability to resume normal activities at 6 months (27% versus 0%, P < 0.0001).
CONCLUSIONS: In RCVS, women and migrainers seem to be at higher risk of intracranial hemorrhage. Overall, intracranial hemorrhages are frequent in RCVS and are associated with a more severe clinical spectrum.

PMID 20884871  Stroke. 2010 Nov;41(11):2505-11. doi: 10.1161/STROKEAHA・・・
著者: Anne Ducros, Rula A Hajj-Ali, Aneesh Bhim Singhal, Shuu-Jiun Wang
雑誌名: JAMA Neurol. 2014 Mar;71(3):368. doi: 10.1001/jamaneurol.2013.5875.
Abstract/Text
PMID 24614989  JAMA Neurol. 2014 Mar;71(3):368. doi: 10.1001/jamaneuro・・・
著者: Michael E Detsky, Devon R McDonald, Mark O Baerlocher, George A Tomlinson, Douglas C McCrory, Christopher M Booth
雑誌名: JAMA. 2006 Sep 13;296(10):1274-83. doi: 10.1001/jama.296.10.1274.
Abstract/Text CONTEXT: In assessing the patient with headache, clinicians are often faced with 2 important questions: Is this headache a migraine? Does this patient require neuroimaging? The diagnosis of migraine can direct therapy, and information obtained from the history and physical examination is used by physicians to determine which patients require neuroimaging.
OBJECTIVE: To determine the usefulness of the history and physical examination that distinguish patients with migraine from those with other headache types and that identify those patients who should undergo neuroimaging.
DATA SOURCES AND STUDY SELECTION: A systematic review was performed using articles from MEDLINE (1966-November 2005) that assessed the performance characteristics of screening questions in diagnosing migraine (with the International Headache Society diagnostic criteria as a gold standard) and addressed the accuracy of the clinical examination in predicting the presence of underlying intracranial pathology (with computed tomography/magnetic resonance imaging as the reference standard).
DATA EXTRACTION: Two authors independently reviewed each study to determine eligibility, abstract data, and classify methodological quality using predetermined criteria. Disagreement was resolved by consensus with a third author.
DATA SYNTHESIS: Four studies of screening questions for migraine (n = 1745 patients) and 11 neuroimaging studies (n = 3725 patients) met inclusion criteria. All 4 of the migraine studies illustrated high sensitivity and specificity if 3 or 4 criteria were met. The best predictors can be summarized by the mnemonic POUNDing (Pulsating, duration of 4-72 hOurs, Unilateral, Nausea, Disabling). If 4 of the 5 criteria are met, the likelihood ratio (LR) for definite or possible migraine is 24 (95% confidence interval [CI], 1.5-388); if 3 are met, the LR is 3.5 (95% CI, 1.3-9.2), and if 2 or fewer are met, the LR is 0.41 (95% CI, 0.32-0.52). For the neuroimaging question, several clinical features were found on pooled analysis to predict the presence of a serious intracranial abnormality: cluster-type headache (LR, 10.7; 95% CI, 2.2-52); abnormal findings on neurologic examination (LR, 5.3; 95% CI, 2.4-12); undefined headache (ie, not cluster-, migraine-, or tension-type) (LR, 3.8; 95% CI, 2.0-7.1); headache with aura (LR, 3.2; 95% CI, 1.6-6.6); headache aggravated by exertion or a valsalva-like maneuver (LR, 2.3; 95% CI, 1.4-3.8); and headache with vomiting (LR, 1.8; 95% CI, 1.2-2.6). No clinical features were useful in ruling out significant pathologic conditions.
CONCLUSIONS: The presence of 4 simple historical features can accurately diagnose migraine. Several individual clinical features were found to be associated with a significant intracranial abnormality, and patients with these features should undergo neuroimaging.

PMID 16968852  JAMA. 2006 Sep 13;296(10):1274-83. doi: 10.1001/jama.29・・・
著者: R B Lipton, D Dodick, R Sadovsky, K Kolodner, J Endicott, J Hettiarachchi, W Harrison, ID Migraine validation study
雑誌名: Neurology. 2003 Aug 12;61(3):375-82.
Abstract/Text BACKGROUND: Migraine is a highly prevalent and disabling illness that remains substantially undiagnosed in primary care. Because of the potential value of a screening tool, the current study was designed to establish the validity and reliability of a brief, self-administered migraine screener in patients with headache complaints in the primary care setting.
METHODS: A total of 563 patients presenting for routine primary care appointments and reporting headaches in the past 3 months completed a self-administered migraine screener. All patients were then referred for an independent diagnostic evaluation by a headache expert, of whom 451 (80%) completed a full evaluation. Migraine diagnosis was assigned based on International Headache Society criteria after completing a semi-structured diagnostic interview.
RESULTS: Of nine diagnostic screening questions, a three-item subset of disability, nausea, and sensitivity to light provided optimum performance, with a sensitivity of 0.81 (95% CI, 0.77 to 0.85), a specificity of 0.75 (95% CI, 0.64 to 0.84), and positive predictive value of 0.93 (95% CI, 89.9 to 95.8). Test-retest reliability was good, with a kappa of 0.68 (95% CI, 0.54 to 0.82). The sensitivity and specificity of the three-item migraine screener was similar regardless of sex, age, presence of other comorbid headaches, or previous diagnostic status.
CONCLUSIONS: The three-item ID Migraine migraine screener was found to be a valid and reliable screening instrument for migraine headaches. Its ease of use and operating characteristics suggest that it could significantly improve migraine recognition in primary care.

PMID 12913201  Neurology. 2003 Aug 12;61(3):375-82.

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