Headache Group-Department of Neurology, University of California, San Francisco, CA 94115, USA.
J Neurol Neurosurg Psychiatry. 2012 Jun;83(6):663-5. doi: 10.1136/jnnp-2011-302124. Epub 2012 Apr 6.
Harlequin syndrome (HS) is a rare autonomic disorder characterised by unilateral diminished sweating and flushing of the face in response to heat or exercise. Some patients with HS complain of headache.
We present three new cases to characterise their headache phenotype and pharmacology and review the literature of cases where headache was described. RESULTS Two out of the three patients presented with episodes of unilateral headache associated with exercise: in one case the headache had migrainous features and was contralateral to the side where the flushing occurred, whereas the second patient, who had had migraine attacks in the past, had a brief throbbing headache, with no associated symptoms, ipsilateral to the facial flushing. The third woman had migraine but the attacks were not associated with HS. Pharmacological characterisation suggested the HS and migraine were biologically distinct. HS was not triggered by nitroglycerin and was unaffected by sumatriptan, dihydroergotamine and ergotamine. HS and migraine did not occur together. In the literature, we found six patients with both HS and headache, five of whom had migraine.
These data do not show any correlation between the phenotypic expression of migraine and HS suggesting the syndromes are pathogenetically independent.
亨氏综合征（HS）是一种罕见的自主神经紊乱，其特征是单侧出汗减少和面部潮红，对热或运动有反应。一些 HS 患者会出现头痛。
我们介绍了三个新病例，以描述其头痛表型和药理学，并回顾了文献中描述头痛的病例。
三个患者中有两个出现与运动相关的单侧头痛发作：一个病例的头痛具有偏头痛特征，并且与潮红发生的对侧相对应，而第二个患者过去有偏头痛发作，出现短暂的搏动性头痛，同侧面部潮红，没有伴随症状。第三位女性患有偏头痛，但头痛发作与 HS 无关。药物学特征表明 HS 和偏头痛在生物学上是不同的。HS 不会被硝化甘油触发，也不受舒马曲坦、二氢麦角胺和麦角胺的影响。HS 和偏头痛不会同时发生。在文献中，我们发现了六名同时患有 HS 和头痛的患者，其中五名患有偏头痛。
这些数据没有显示偏头痛和 HS 的表型表达之间有任何相关性，表明这两种综合征在发病机制上是独立的。
