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可逆性脑血管收缩综合征

Reversible cerebral vasoconstriction syndrome.

作者信息

Ducros Anne, Bousser Marie-Germaine

机构信息

Emergency Headache Centre, Assistance Publique des Hôpitaux de Paris, Head and Neck Centre, Lariboisière Hospital, 2 rue Ambroise Paré, Paris Cedex 10, France.

出版信息

Pract Neurol. 2009 Oct;9(5):256-67. doi: 10.1136/jnnp.2009.187856.

Abstract

Reversible cerebral vasoconstriction syndrome is characterised by severe headaches with or without seizures and focal neurological deficits, and constriction of cerebral arteries which resolves spontaneously in 1-3 months. It affects females slightly more than males, and mean age of onset is around 45 years. Approximately 60% of cases are secondary, mainly postpartum and after exposure to vasoactive substances. The major complications are localised cortical subarachnoid haemorrhage (22%) and parenchymal ischaemic or haemorrhagic strokes (7%) which may leave permanent sequelae. Diagnosis requires the demonstration of the "string of beads" appearance of cerebral arteries on angiography, with complete or almost complete resolution on repeat angiography 12 weeks after onset. Nimodipine seems to reduce thunderclap headaches within 48 h but has no definite effect on the haemorrhagic and ischaemic complications.

摘要

可逆性脑血管收缩综合征的特征是伴有或不伴有癫痫发作及局灶性神经功能缺损的严重头痛,以及脑动脉收缩,这种收缩在1至3个月内可自发缓解。女性受影响略多于男性,平均发病年龄约为45岁。约60%的病例为继发性,主要发生在产后及接触血管活性物质后。主要并发症为局限性皮质蛛网膜下腔出血(22%)和实质性缺血性或出血性卒中(7%),这些并发症可能会留下永久性后遗症。诊断需要在血管造影上显示脑动脉呈“串珠样”外观,发病12周后复查血管造影时完全或几乎完全缓解。尼莫地平似乎能在48小时内减轻霹雳样头痛,但对出血性和缺血性并发症没有确切疗效。

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