Goadsby P J, Lipton R B
Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, UK.
Brain. 1997 Jan;120 ( Pt 1):193-209. doi: 10.1093/brain/120.1.193.
The short-lasting primary headache syndromes may be conveniently divided into those exhibiting marked autonomic activation and those without autonomic activation. The former group comprise chronic and episodic paroxysmal hemicrania, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) and cluster headache. These headache syndromes are compared with other short-lasting headache disorders, such as hypnic headache, and persistent headache with milder autonomic features such as hemicrania continua. Cluster headache is included with the shorter-lasting headaches to attempt a nosological analysis of these syndromes. The paroxysmal hemicranias are characterized by frequent short-lasting attacks of unilateral pain usually in the orbital, supraorbital or temporal region that typically last minutes. The attack frequency usually ranges from 5 to 40 attacks per day. The pain is severe and associated with autonomic symptoms such as conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, ptosis or eyelid oedema. Almost all reported cases respond to treatment with indomethacin, but respond poorly to other treatments including other nonsteroidal anti-inflammatory drugs. A recent case study demonstrated the release of both trigeminal and parasympathetic neuropeptides during a bout of pain in the same pattern previously described in cluster headache. The SUNCT syndrome is a distinctive rare condition characterized by less severe pain but marked autonomic activation during attacks. Consistent with previous reports, the present case of SUNCT syndrome was intractable to therapy. The similarites of these syndromes suggests a considerable shared pathophysiology. It is suggested that the syndromes are sufficiently well established for inclusion in the International Headache Society Classification system and that trigeminal-autonomic cephalalgias should be classified as a group together. A proposed re-classification is presented.
短期原发性头痛综合征可方便地分为表现出明显自主神经激活的和无自主神经激活的两类。前一组包括慢性发作性偏侧头痛、伴有结膜充血和流泪的短暂性单侧神经痛样头痛（SUNCT综合征）以及丛集性头痛。将这些头痛综合征与其他短期头痛疾病进行比较，如睡眠性头痛，以及具有较轻自主神经特征的持续性头痛，如连续性偏侧头痛。丛集性头痛与短期头痛一起纳入，以尝试对这些综合征进行疾病分类分析。发作性偏侧头痛的特点是频繁出现单侧疼痛的短暂发作，通常位于眼眶、眶上或颞部区域，通常持续数分钟。发作频率通常为每天5至40次。疼痛剧烈，并伴有自主神经症状，如结膜充血、流泪、鼻塞、流涕、上睑下垂或眼睑水肿。几乎所有报道的病例对吲哚美辛治疗有反应，但对包括其他非甾体抗炎药在内的其他治疗反应不佳。最近的一项病例研究表明，在一次疼痛发作期间，三叉神经和副交感神经肽的释放模式与先前在丛集性头痛中描述的相同。SUNCT综合征是一种独特的罕见疾病，其特点是发作时疼痛较轻，但自主神经激活明显。与先前的报道一致，本病例的SUNCT综合征治疗棘手。这些综合征的相似性表明存在相当多共同的病理生理学机制。有人认为，这些综合征已足够明确，可以纳入国际头痛协会分类系统，并且三叉神经自主性头痛应归为一组。本文提出了一种拟议的重新分类。
