可逆性脑部血管收缩症候群是雷击头痛的另一个原因


  September 23, 2009 (宾州费城) —可逆性脑部血管收缩症候群(RCVS)是雷击头痛(thunderclap headache)的一个原因,在这些急性发作头痛的不同诊断中应将其纳入考量。
  
  为了清楚定义与RCVS有关的诱发原因、症状以及诊断发现,华盛顿大学医学院神经科助理教授Todd Schwedt医师等人回顾了文献中的RCVS案例,发表于第14届国际头痛研讨会中。
  
  研究者报告指出,RCVS主要影响妇女,且大约三分之一的案例与暂时性神经缺损有关,但有10%的案例是持续性缺损。症状一般发生在产后,或者由某些药物引起。病患有颅内出血、缺血性中风、脑水肿等风险。血管收缩通常在12周内恢复。
  
  德州Baylor医学院神经科临床教授Medscape Neurology科学咨询委员会委员、未参与本研究的Randolph Evans医师表示,RCVS是一种罕见状况,有许多神经科专家未曾听过此病。1988年,有作者在Stroke期刊中描述此病,之后,该病也被称为「Call-Fleming syndrome」。
  
  Evans医师向Medscape Neurology表示,不过,因为症状依旧未被确认,可能不如我们所认为的这么罕见。
  
  他建议,我们的当务之急在于确认它,特别是有雷击头痛者发生突发的严重头痛时。
  
  【RCVS的系统性回顾】
  发表的这个系统性回顾中,纳入新发生的头痛或多处颅内动脉血管收缩案例,或者血管收缩在发生12周内缓解的案例,或者没有动脉瘤蛛网膜下出血的案例。
  
  80篇发表的文献中,有250名RCVS病患符合此一准则。女性和男性的比率为6比1。病患年纪在13至70岁之间(平均43岁)。发生的状况包括产后(18%的案例)或有偏头痛病史(27%),其它活动包括洗澡、费力活动/ Valsalva氏现象,以及血管创伤。RCVS病患有44%使用各种药物或非法药物。几乎所有(92%)RCVS病患都出现雷击头痛。脑脊液参数为正常或接近正常,有27% RCVS病患的蛋白质略为升高,且19% RCVS病患白血球计数大于5 cells/mm3。
  
  29%的RCVS病患发生暂时性神经缺损,10%是持续性缺损。RCVS病患皮质蛛网膜下出血、脑内出血、缺血性中风、脑水肿的发生比率分别是17%、9%、24%以及22%。
  
  研究者在摘要中写道,血管收缩在前循环中最常见,特别是中脑动脉。他们发现,在他们查找的文献中,91%的RCVS病患有中脑动脉血管收缩,60%有前脑动脉收缩、56%为后脑动脉收缩,19%发生在基底动脉,10%发生在内颈动脉。
  
  【罕见状况】
  Evans医师表示,神经科专家对于怀孕末期病患、产后、或高血压病患应提高怀疑。引起RCVS的药物,例如古柯碱或迷幻药或一些处方药,如选择性血清素再吸收抑制剂,或者完全没有诱发原因。
  
  Evans医师指出,这通常是有偏头痛病史的年轻妇女会发生的疾病。他表示,影像检查可以发现发生收缩的动脉,但是,他也指出,没有可以确认诊断的单一种检测方式。其它疾病也可能会引起血管收缩,例如血管炎,必须加以排除。
  
  需要就治疗方面进行更多研究,但是有些医师曾经使用nimodipine用于预防和急性治疗,其它钙通道阻断剂如verapamil也有用。
  
  除了Schwedt医师等人提出的诱发状况之外,Evans医师指出,性行为、排便、突发的情绪、排尿、咳嗽、打喷嚏或弯腰也会。约有20%的人在休息时、没有前述行为时发生雷击头痛。
  
  Evans医师表示,病患可能有出血1周且接著发生缺血性事件。动脉X光正常、显示没有血管收缩,并无法排除RCVS。再者,有些病患后来完全恢复。
  
  许多RCVS病患一开始会前往急诊,但是如果他们还有雷击头痛,或许会找神经科医师就医。Evans医师对神经科医师的建议是,将RCVS视为反覆雷击头痛的不同诊断的一部份。
  
  Schwedt医师宣告接受GlaxoSmithKline、AGA以及Allergan的临床研究费用、奖金与研究资金,以及担任VersusMed之顾问或咨询委员的酬劳。Evans医师宣告担任Merck、Ortho-McNeil、Pfizer、GlaxoSmithKline、Lilly、Teva以及UCB等之建言者或顾问、接受临床研究支持、接受奖助金。他也是Medscape Neurology的无给职咨询委员。
  
  第14届国际头痛研讨会:墙报PO373。展示于2009年9月11-12日。
  

Reversible Cerebral Vasoconstriction Syndromes Another Cause of Thunderclap Headache

By Daniel M. Keller, PhD
Medscape Medical News

September 23, 2009 (Philadelphia, Pennsylvania) — Reversible cerebral vasoconstriction syndromes (RCVS) are a cause of thunderclap headache and should be considered in the differential diagnosis of these acute-onset headaches.

To better define the triggers, symptoms, and diagnostic findings associated with RCVS, Todd Schwedt, MD, assistant professor of neurology, and colleagues at Washington University School of Medicine in St. Louis, Missouri, reviewed RCVS cases in the literature and presented their findings here at the 14th International Headache Congress.

The researchers report that RCVS primarily affects women and is associated with transient neurological deficits in about one third of cases, but that these deficits may persist in 10% of cases. The syndromes commonly occur in the postpartum period or may be brought on by certain drugs. Patients are at risk for intracranial bleeds, ischemic stroke, and cerebral edema. Vasoconstriction usually reverses within 12 weeks of onset.

Randolph Evans, MD, clinical professor of neurology at Baylor College of Medicine in Houston, Texas, and a member of the Medscape Neurology scientific advisory board, who was not involved in the study, said RCVS is a rare condition and that many neurologists have never heard of it. It has been referred to as Call-Fleming syndrome after the authors who described it in Stroke in 1988. As the syndrome is still underrecognized, however, "it may not be as rare as we think," Dr. Evans told Medscape Neurology.

"The first thing is for us to start recognizing it, particularly when people have thunderclap headaches — when they have sudden, severe headaches," he advised.

Systematic Review on RCVS

In the systematic review presented here, cases were included if they had new-onset headache or multifocal intracranial artery vasoconstriction, if the vasoconstriction resolved within 12 weeks of onset and if there was no aneurysmal subarachnoid hemorrhage.

Eighty publications containing 250 patients who had RCVS met these criteria. Women outnumbered men by 6 to1. Patients ranged in age from 13 to 70 years (mean, 43 years). Predisposing conditions included being postpartum (18% of cases) or having a history of migraine (27%), and activities included bathing, physical exertion/Valsalva, and vascular trauma. Exposure to various medications or illicit drugs occurred in 44% of patients with RCVS. Almost all patients with RCVS (92%) presented with a thunderclap headache. Cerebrospinal fluid parameters were normal or nearly so, with mildly elevated protein in 27% of patients with RCVS and white blood cell count greater than 5 cells/mm3 in 19% of patients with RCVS.

Transient neurologic deficits occurred in 29% of patients with RCVS, and 10% experienced persistent deficits. Cortical subarachnoid hemorrhage, intraparenchymal hemorrhage, ischemic stroke, and cerebral edema occurred in 17%, 9%, 24%, and 22% of patients with RCVS, respectively.

The researchers write in their abstract that "vasoconstriction is most commonly identified in the anterior circulation, specifically the middle cerebral artery." They found in their literature search vasoconstriction of the middle cerebral artery in 91% of patients with RCVS, anterior cerebral artery in 60%, posterior cerebral artery in 56%, basilar artery in 19%, and internal carotid in 10%.

A Rare Condition

Dr. Evans said that neurologists' suspicions should be raised for patients in late pregnancy, during postpartum, or with hypertension. RCVS can be triggered by drugs such as cocaine or ecstasy or some prescription drugs such as selective serotonin reuptake inhibitors, or there may be no trigger at all.

"It's usually a disease of young women who may have a history of migraine," Dr. Evans noted. He said imaging may reveal constriction of affected arteries, but added that "there is no single test to confirm the diagnosis." Other diseases that can also cause vasoconstriction, such as vasculitis, need to be ruled out.

More research into treatments is needed, but some clinicians have been using nimodipine for prevention and acute treatment, and other calcium channel blockers such as verapamil may also work.

In addition to the triggers mentioned by Dr. Schwedt and colleagues, Dr. Evans added sexual intercourse, bowel movement, sudden emotion, urination, coughing, sneezing, or bending over. About 20% of people have a thunderclap headache at rest with no predisposing activities.

Dr. Evans said that patients may have a hemorrhage 1 week and go on to have an ischemic event the next. A normal arteriogram showing no vasoconstriction cannot rule out RCVS. Furthermore, some patients go on to recover completely.

Many RCVS patients will be seen initially in the emergency department, but if they have additional thunderclap headaches, they will probably see a neurologist. Dr. Evans' advice is for neurologists to consider RCVS as part of the differential diagnosis of recurrent thunderclap headaches.

Dr. Schwedt has disclosed receiving payments for conducting clinical research, honoraria, research grants (from GlaxoSmithKline, AGA, and Allergan), and compensation as a consultant or advisory board member for VersusMed. Dr. Evans has disclosed that he has served as an advisor or consultant to, received clinical research support from, or received honoraria from Merck, Ortho-McNeil, Pfizer, GlaxoSmithKline, Lilly, Teva, and UCB. He is also an uncompensated advisory board member for Medscape Neurology.

14th International Headache Congress: Poster PO373. Displayed September 11 and 12, 2009.

    



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