导读：持续性偏头痛是一种主要的偏头痛类型，可以长期存在。它对生活质量往往产生了很多负面影响。临床表现为持续数月的单侧头痛，可出现间歇性加重并伴有相关自主神经症状。IntroductionHemicrania continua (HC) is a primary headache disorder with pathognomonic treatment response to indomethacin. It presents clinically with a baseline continuous unilateral headache for months that intermittently exacerbates with associated autonomic features. HC was first described in 1981 by Medina and Diamond as a cluster headache variant.[1] and the term “hemicrania continua” was first coined in 1984 by Sjaastad and Spierings.[2] 持续性偏头痛(HC)是一种原发性头痛，症状治疗一般选用吲哚美辛。临床表现为持续数月的单侧头痛，间歇性加重并伴有相关自主神经特征。HC在1981年被Medina和Diamond首次描述为一种丛集性头痛。1984年，Sjaastad和Spierings首次创造了“持续性偏头痛”这个词。 HC has been placed under the heading of trigeminal autonomic cephalalgias (TACs) in the third edition of the International Classification of Headache Disorder (ICHD-3). Other primary headache disorders included in TACs are cluster headache (CH), paroxysmal hemicrania (PH), short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), and short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing (SUNCT).[3] HC在第三版国际头痛疾病分类(ICHD-3)中被列为三叉自主神经性头痛(TACs)。TACs包括的其他原发性偏头痛，包括丛集性偏头痛(CH)、阵发性偏头痛(PH)、伴自主神经症状的短期单侧神经性头痛发作(SUNA)和伴流泪和短期撕裂样单侧神经性头痛发作(SUNCT) EtiologyHemicrania continua is a primary headache disorder without a secondary organic cause. Our understanding of TACs, including HC, is limited. Multiple theories have been proposed that include cavernous sinus inflammation, intracranial arterial vasodilation, upregulation of vasopeptides, particularly calcitonin gene-related peptide (CGRP) and vasoactive intestinal peptide (VIP), and trigeminal nerve autonomic dysregulation resulting in the increased cranial parasympathetic outflow. Other intracranial areas are also involved as pain is not always restricted to the area supplied by the trigeminal nerve. Secondly, the transection of the trigeminal nerve does not relieve symptoms in all the patients.[4] 持续性偏头痛是一种原发性头痛，没有继发器质性原因。我们对包括HC在内的很多类型偏头痛的理解是有限的。目前已有多种理论提出，包括海绵窦炎症、颅内动脉血管舒张、血管肽(特别是降钙素基因相关肽(CGRP)和血管活性肠肽(VIP)上调、三叉神经自主调节失调导致颅副交感神经兴奋性增加等。且疼痛并不总是局限于三叉神经供应的区域，也累及其他颅内区域。因此，截断三叉神经并不能缓解所有患者的症状。 EpidemiologyHemicrania continua was initially considered a rare headache disorder, and only 18 cases were reported in the literature in the first 7 years after the discovery and about 100 cases in the first 17 years (1984-2001).[5][6] Although large epidemiologic studies are lacking to outline the exact prevalence of this condition, it is estimated that HC cases constitute about 1% of total headache cases.[7][8] This number is still considered underreported and underdiagnosed. HC is more prevalent in young adults in their third and fourth decades with a mean age of 30 years. However, the range varies from the first to seventh decades. HC is more common in females than in males (2:1). 持续性偏头痛最初被认为是一种罕见的头痛疾病，发现后的前7年文献报道仅18例，前17年(1984-2001)报道约100例。虽然尚无大型流行病学研究来概括这种情况的确切流行情况，但据估计HC病例约占头痛病例总数的1%。这个数字仍然被认为是漏报和诊断不足。HC在三四十岁的年轻人中更为普遍，平均年龄为30岁。HC在女性中比在男性中更常见(2:1)。 History and PhysicalPatients with hemicrania continua, as the name indicates, present with a continuous unilateral headache that is present for months. It is mild-to-moderate in severity, dull in character, and often does not affect physical activity. It is commonly located in the first division of the trigeminal nerve involving the frontal and periorbital regions, but other extra-trigeminal areas may also be involved. The unilateral headache in HC is side-locked, i.e., occurs on the same side, with a slight preference for the right side; however, side-alternating attacks have also been reported in the literature.[5][9] A rare bilateral HC is also reported in the literature.[10][11][12] Although headache in HC is unremitting in nature, about 1 in 5 persons (20%) may experience pain-free periods lasting from one day to several weeks.[13] 持续性偏头痛患者，顾名思义，会出现持续的单侧头痛，持续数月。它的严重程度为轻中度，性质沉闷，通常不影响体力活动。它通常位于三叉神经第一分支累及额部和眶周区域，但也可累及其他三叉神经外区域。HC的单侧头痛是侧锁的，即发生在同侧，轻微倾向于右侧;然而，侧边交替攻击也有文献报道。[5][9]文献中也报道了罕见的双侧HC。虽然HC的头痛本质上是持续性的，但大约五分之一(20%)的人可能经历一天到几周的无痛期 This background headache often has superimposed fluctuating headache exacerbations, which may last for a few minutes to days. These exacerbations are also highly variable with a frequency ranging from more than 20 attacks daily to one attack in 4 months. About half of patients report one attack daily. These are moderate-to-very severe in intensity and throbbing or stabbing in character.[9] Some people may label these headache exacerbations as the worst headache of their lives and may also experience suicidal thoughts during these exacerbations. The patients may become restless, agitated, and have difficulty staying still. The migrainous features of photophobia, phonophobia, nausea, and/or vomiting may occur during exacerbations, but the aura is uncommon. Exacerbations are also triggered by stress, alcohol, irregular sleep patterns, and menstruation in some patients.[5][9] 这种背景头痛往往叠加波动性头痛恶化，可能持续几分钟至几天。这些恶化也高度可变，频率从每天20次以上发作到4个月一次发作不等。大约一半的患者报告每天发作一次。这些症状在强度上是中等到非常严重的，特点是悸动或刺痛一些人可能会将这些头痛恶化标记为他们生命中最严重的头痛，并可能在这些恶化期间经历自杀的想法。病人可能变得烦躁不安，难以保持静止。偏头痛的特点，畏光，声音恐怖，恶心，和/或呕吐可发生在恶化期间，但先兆不常见。在一些患者中，压力、酒精、睡眠不规律和月经也会引发病情恶化。 Like other TACs, HC is associated with ipsilateral cranial autonomic symptoms, especially during exacerbations, but these autonomic symptoms are generally less prominent than other TACs, including cluster headache (CH) and paroxysmal hemicrania (PH). The cranial autonomic features may include forehead sweating, lacrimation, conjunctival injection and swelling, ptosis, miosis, a feeling of foreign body sensation in the eye, nasal congestion, rhinorrhea, and/or aural fullness.[5][9] 与其他tac一样，HC与同侧脑神经自主症状相关，尤其是在病情加重期间，但这些自主症状通常不如其他tac突出，包括丛集性头痛(CH)和阵发性偏脑(PH)。颅自主神经特征可能包括额头出汗、流泪、结膜注射和肿胀、上睑下垂、瞳孔缩小、眼异物感、鼻塞、流涕和/或耳充盈 EvaluationICHD-3 has proposed the following diagnostic criteria for the diagnosis of hemicrania continua[3]:
评价 icd -3提出了以下连续性偏头痛的诊断标准:
The ICHD-3 diagnostic criterion focuses on three key features; (1) unilateral continuous pain for greater 3 months, (2) presence of either ipsilateral cranial autonomic symptoms or agitation during exacerbations, (3) a complete response to indomethacin, which is one of the pathognomonic features of HC. A complete response to indomethacin is usually noted within two hours of indomethacin injection. The headache reappears within 6 to 24 hours of stopping indomethacin. Since indomethacin provides a dramatic relief, an indomethacin trial is proposed by a few authors in all chronic unilateral headaches.[9] It should be noted that a response to indomethacin does not rule out secondary causes of HC.[14] icd -3诊断标准着重于三个关键特征;(1)单侧持续疼痛超过3个月，(2)加重期间出现同侧脑神经自主症状或躁动，(3)对消炎痛敏感，这是HC的症状特征之一。对消炎痛的敏感通常在注射消炎痛后两小时内被注意到。消炎痛停止后6至24小时内头痛再次出现。由于吲哚美辛能显著缓解头痛，一些作者提出了一项关于所有慢性单侧头痛的吲哚美辛试验值得注意的是，对吲哚美辛的反应并不排除HC中[14]的继发原因。 Secondary conditions that may mimic HC include posttraumatic headache, post-craniotomy headache, intracranial space-occupying lesion, post-stroke headache, internal carotid artery (ICA) dissection or aneurysm, idiopathic intracranial hypertension, venous malformation, cerebral venous sinus thrombosis, analgesic rebound headache, paraneoplastic, sinus pathologies, dental lesions, and temporomandibular joint pathologies.[9] 类似于HC的继发性症状包括外伤性头痛、开颅后头痛、颅内占位性病变、卒中后头痛、颈内动脉(ICA)剥离或动脉瘤、特发性颅内高血压、静脉畸形、脑静脉窦血栓形成、使用镇痛药物性头痛、副肿瘤、鼻窦炎，口腔病变，颞下颌关节紊乱。 Secondary causes of HC should be excluded by clinical features and appropriate investigations. A history of recent trauma should be elucidated from all patients. A magnetic resonance imaging (MRI) of the brain is recommended in all the patients presenting as HC-like headaches. An angiography (MRA, CTA or digital subtraction angiography) of head and neck should be advised if a vascular pathology (e.g. ICA dissection or aneurysm) is suspected. The clinical features suggesting a vascular pathology include the short-term duration of symptoms, frequent exacerbations, neck pain, neck tenderness, focal neurological symptoms, Horner syndrome, or a history of trauma.[9] HC的继发性原因应排除临床特征和适当的调查。所有患者都应阐明近期外伤史。所有表现为hc样头痛的患者都建议进行大脑磁共振成像(MRI)。如果怀疑头颈部血管病变(如ICA夹层或动脉瘤)，应建议进行头颈部血管造影(MRA、CTA或数字减影血管造影)。提示血管病理的临床特征包括症状持续时间短、频繁发作、颈部疼痛、颈部压痛、局灶性神经症状、霍纳综合征或外伤史。 Treatment / ManagementIndomethacin A complete response to indomethacin is one of the pathognomonic features of hemicrania continua. Indomethacin is a non-steroidal anti-inflammatory drug (NSAID) that reversibly inhibits prostaglandin-forming cyclooxygenase (COX) enzyme, similar to ibuprofen and naproxen. It is proposed that indomethacin is more effective than other NSAIDs, probably due to the highest central nervous system penetration, central serotonergic effects, and inhibition of nitrous oxide-dependent vasodilation.[9][15] 吲哚美辛 对吲哚美辛的敏感是持续偏头痛的症状特征之一。消炎痛是一种非甾体抗炎药(NSAID)，可逆地抑制前列腺素形成环氧化酶(COX)，类似于布洛芬和萘普生。有人认为吲哚美辛比其他非甾体抗炎药更有效，这可能是因为它具有最高的中枢神经系统渗透力，以及抑制氧化氮依赖性的血管舒张。 Indomethacin is started at a low dose of 25 mg three times a day with meals and titrated slowly depending upon the response. Patients usually respond within 24 hours, but some may take up to a week.[16] If no clinical response is seen within 48 to 72 hours, a dose is usually increased till either a complete response is achieved or a maximum dose of 300 mg per day is given. It is reasonable to keep the patient at a minimum long-term therapeutic dose to avoid the possible adverse effects, which include abdominal discomfort, heartburn, nausea, vomiting, life-threatening gastrointestinal hemorrhage, hypertension, renal failure, and liver failure. Most of the adverse effects of indomethacin are dose-dependent, and maintaining the lowest possible therapeutic dose is recommended. The treatment is often long-term, if not lifelong. Successful indomethacin tapering has been reported in multiple patients without headache recurrence.[14] 消炎痛一开始是小剂量的25毫克，每日三次，根据反应缓慢加量。患者通常在24小时内就会有反应，但有些可能需要长达一周。如果在48至72小时内没有临床反应，通常要增加剂量，直到达到完全改善或给予每天300毫克的最大剂量。合理的做法是将患者的长期治疗剂量保持在最低限度，以避免可能的副作用，包括腹部不适、胃灼热、恶心、呕吐、危及生命的胃肠出血、高血压、肾衰竭和肝功能衰竭。吲哚美辛的大多数副作用是剂量依赖性的，建议保持最低的治疗剂量。治疗通常是长期的，如果不是终身的。有报道称，有多例患者在吲哚美辛减量后头痛再发作。 Other Non-invasive Treatments If a patient cannot tolerate indomethacin due to side effects, other non-invasive treatment options should be tried. These include melatonin, topiramate, COX-2 inhibitors (rofecoxib and celecoxib), gabapentin, corticosteroids, lamotrigine, lithium, amitriptyline, valproate, and naproxen. These drugs are not as effective as indomethacin in the treatment of HC, but they should be tried before interventional options are considered. Melatonin has a structure similar to the indomethacin and can also be used in combination with indomethacin to lower the former’s dose to prevent side effects.[17] High-dose oxygen and sumatriptan are usually not effective in the management of HC. Vagus nerve stimulation (VNS) is a non-invasive neuromodulation technique that is utilized for the treatment of HC. Although available data is limited, a positive response has been reported in some studies.[18][19] 其他非侵入性治疗 如果患者因副作用不能忍受消炎痛，应尝试其他非侵入性治疗方案。这些药物包括褪黑激素、托吡酯、COX-2抑制剂(罗非昔布和塞来昔布)、加巴喷丁、皮质类固醇、拉莫三嗪、阿米替林、丙戊酸酯和萘普生。褪黑素的结构与消炎痛相似，可与消炎痛联合使用，降低褪黑素的剂量。 迷走神经刺激(VNS)是一种用于治疗HC的无创神经调节技术。虽然现有数据有限，但在一些研究中已报道了有效案例。 Invasive Treatments Botulinum toxin-A is a Food and Drug Administration (FDA) approved treatment for chronic migraines. Many clinicians also consider it for the treatment of HC when non-invasive treatment fails or cannot be tolerated.[17][20] Occipital nerve stimulation (ONS), like botulinum toxin, is primarily used for resistant cases of chronic migraine. It is currently being investigated for the treatment of HC with varying results.[21][22][23] Sphenopalatine ganglion (SPG) blockade is another invasive treatment for the management of HC. SPG cuts the parasympathetic outflow of the trigeminal nerve, which is an important pathophysiologic response in patients with HC.[17] Deep brain stimulation (DBS) is another invasive method that may be used in the treatment of medically refractory TAC, including HC. The posterior hypothalamus is the target in these patients.[24 侵入性治疗 a型肉毒杆菌毒素是美国食品和药物管理局(FDA)批准的一种治疗慢性偏头痛的药物。当非侵入性治疗失败或不能耐受时，许多临床医生也考虑用它来治疗HC。 枕神经刺激(ONS)，像肉毒杆菌毒素，主要用于耐药的慢性偏头痛病例。目前正在研究如何治疗HC，结果各不相同。 SPG阻滞是另一种有创治疗方法。SPG切断三叉神经副交感神经传出，这是HC.[17]患者重要的病理生理反应。 深部脑刺激(DBS)是另一种可用于治疗医学上难治性TAC(包括HC)的侵入性方法。下丘脑是这些患者的治疗靶点。 、 |
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