van Vliet J A, Eekers P J E, Haan J, Ferrari M D
Department of Neurology, Leiden University Medical Centre, 2300 RC Leiden, Netherlands.
J Neurol Neurosurg Psychiatry. 2003 Aug;74(8):1123-5. doi: 10.1136/jnnp.74.8.1123.
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.
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.
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.
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.
丛集性头痛（CH）是一种相对罕见但极为严重的原发性头痛。尽管有明确且可识别的诊断标准，但诊断往往被漏诊或延误。此外，虽然确诊病例有充分且基于证据的治疗方法，但CH的管理似乎欠佳。作者对CH患者进行了一项全国性调查，并寻找与诊断延误相关的因素。
作者向荷兰所有全科医生（约5800名）和神经科医生（约560名）进行了全国范围的邮件调查，邀请他们推荐已确诊或疑似丛集性头痛的患者。患者也可通过荷兰头痛患者协会申请。通过问卷评估了各种临床特征。具体而言，询问了患者首次发作与诊断之间的时间。
2001名受访者中有1429人符合CH的国际头痛协会（IHS）标准，其中1163人填写了详细问卷。男女比例为3.7:1。发病的平均年龄为32（标准差14）岁。73%为发作性CH，21%为慢性CH，6%的周期性未确定。首次发作与诊断之间的时间跨度为1周至48年（中位数3年）：34%在确诊前咨询过牙医，33%咨询过耳鼻喉科专家。增加诊断延误的因素包括畏光或畏声、恶心、发作性发作模式和低发病年龄（p<0.01）。发作期间的性别或烦躁不安情况不影响诊断延误。
在许多情况下，CH多年来仍未被识别或误诊。畏光或畏声以及恶心在一定程度上导致了这种延误，应被视为CH临床症状的一部分。许多患者因CH发作首先就诊于牙医或耳鼻喉科专家，因此应更加重视培训一线医生识别CH，改善诊断流程，从而使患者能更早且更好地接受CH治疗。
