Department of Neurology, The University of Utah, Salt Lake City, Utah, USA.
Department of Internal Medicine, Saint Mary's Hospital, Waterbury, Connecticut, USA.
Headache. 2024 Jun;64(6):674-684. doi: 10.1111/head.14728. Epub 2024 May 23.
Hemicrania continua is a primary unilateral headache characterized by ipsilateral parasympathetic and sympathetic autonomic features. A key diagnostic criterion is its dramatic response to indomethacin treatment; however, various vascular or structural abnormalities have been reported to cause secondary hemicrania continua, presenting with clinical features similar to those of the primary headache presentation.
We reviewed the literature to compile secondary hemicrania continua cases, highlighting the importance of imaging during the evaluation. Additionally, we also contributed our three cases to the existing studies.
We conducted a review of articles from the PubMed and EMBASE databases that described reported cases of secondary hemicrania continua, covering the period from 1993 to 2021. Our review included detailed patient information, signs, and symptoms of hemicrania continua, as well as information on indomethacin usage and headache resolution (if pertinent).
Secondary hemicrania continua can result from a remarkably diverse range of structural and vascular lesions, yet clinical reports on long-term follow-up are lacking. Notably, cases may exhibit a classical response to indomethacin, emphasizing the importance of neuroimaging in excluding secondary cases. Our search yielded 41 cases meeting our criteria. We excluded six cases that were not treated with indomethacin or were unresponsive to it. Additionally, we present three cases that highlight the necessity of neuroimaging in evaluating hemicrania continua, along with short- and long-term clinical outcomes following indomethacin and lesion-directed treatments. Case 1 presented with daily right-sided headaches and cranial autonomic symptoms. Her pain completely resolved with indomethacin use. Neuroimaging of the brain revealed a laterally directed saccular aneurysm of the right internal carotid artery. Case 2 presented with continuous left-sided unilateral headaches with superimposed exacerbations. She complained of left-sided photophobia with a dull sensation in the left ear. Her symptoms decreased after 2 weeks of indomethacin use. Neuroimaging of the head indicated a benign tumor with mass effect into the left lateral medulla and inferior cerebellar peduncle. Case 3 presented with a right side-locked headache with daily, severe superimposed exacerbations. She had photophobia in the right eye and a right-sided Horner's syndrome, along with tearing during her exacerbations. Neuroimaging of the brain revealed a pituitary tumor and her pain completely resolved with indomethacin.
Hemicrania continua is a rare headache disorder that can be either primary or secondary. Importantly, response to indomethacin can still occur in secondary hemicrania continua. Thus, neuroimaging should be considered to rule out underlying structural etiology in all cases, regardless of their clinical responsiveness to indomethacin therapy.
丛集性头痛是一种原发性单侧头痛，其特征为同侧副交感神经和交感神经自主功能紊乱。一个关键的诊断标准是其对吲哚美辛治疗的显著反应；然而，各种血管或结构异常已被报道可引起继发性丛集性头痛，其临床表现与原发性头痛相似。
我们复习文献以汇编继发性丛集性头痛病例，强调在评估过程中影像学的重要性。此外，我们还将我们的三个病例纳入现有研究。
我们对 1993 年至 2021 年间发表的描述继发性丛集性头痛病例的 PubMed 和 EMBASE 数据库中的文章进行了综述。我们的综述包括详细的患者信息、丛集性头痛的体征和症状，以及吲哚美辛使用情况和头痛缓解情况（如果相关）。
继发性丛集性头痛可由多种结构和血管病变引起，但缺乏长期随访的临床报告。值得注意的是，某些病例可能对吲哚美辛表现出典型的反应，这强调了神经影像学在排除继发性病例中的重要性。我们的搜索结果得到了 41 例符合我们标准的病例。我们排除了 6 例未用吲哚美辛治疗或对其无反应的病例。此外，我们还介绍了 3 个病例，这些病例突出了在评估丛集性头痛时进行神经影像学检查的必要性，以及吲哚美辛和针对病变的治疗后的短期和长期临床结果。病例 1 表现为右侧持续性头痛和颅自主神经症状。她的疼痛完全缓解吲哚美辛的使用。脑影像学检查显示右侧颈内动脉外侧指向的囊状动脉瘤。病例 2表现为左侧持续性单侧头痛，伴有发作性加重。她抱怨左眼畏光，左耳钝痛。她的症状在使用吲哚美辛 2 周后减轻。头部影像学检查显示一个良性肿瘤，有向左侧延髓和小脑下脚外侧的肿块效应。病例 3表现为右侧锁定性头痛，每日剧烈发作。她右眼畏光，右侧 Horner 综合征，发作时流泪。脑影像学检查显示垂体瘤，她的疼痛完全缓解吲哚美辛。
丛集性头痛是一种罕见的头痛疾病，可分为原发性或继发性。重要的是，继发性丛集性头痛对吲哚美辛仍可能有反应。因此，无论其对吲哚美辛治疗的临床反应如何，都应考虑进行神经影像学检查以排除潜在的结构性病因。
