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脑部肉芽肿性血管炎。一种病因多样的炎症反应。

Granulomatous angiitis of the brain. An inflammatory reaction of diverse etiology.

作者信息

Younger D S, Hays A P, Brust J C, Rowland L P

机构信息

Department of Neurology, Columbia University College of Physicians and Surgeons, New York, NY.

出版信息

Arch Neurol. 1988 May;45(5):514-8. doi: 10.1001/archneur.1988.00520290042012.

Abstract

Granulomatous angiitis of the brain (GAB) is defined histologically by granulomatous inflammation of intracranial blood vessels. We have studied four patients with autopsy-proved GAB who had, respectively, Hodgkin's lymphoma, herpes zoster, neurosarcoidosis, and no associated illness. Headache, fever, or mental change was followed by hemiparesis or quadriparesis, coma, and death in all four patients. There were no diagnostic findings from cerebral computed tomograms, cerebrospinal fluid, or cerebral angiograms; the diagnosis was established only by postmortem examination. Vasculitis was limited to the brain in all four patients, and involved large arteries, small arteries and veins, or both large and small vessels. Differences in etiology and different particulars of the pathologic conditions imply that GAB is a nonspecific reaction, not a unique disease. The diagnosis, moreover, cannot be proved without histologic confirmation. A biopsy specimen is the only way to ascertain the diagnosis in living patients.

摘要

脑肉芽肿性血管炎(GAB)在组织学上定义为颅内血管的肉芽肿性炎症。我们研究了4例经尸检证实为GAB的患者,他们分别患有霍奇金淋巴瘤、带状疱疹、神经结节病,且无相关疾病。所有4例患者均先出现头痛、发热或精神改变,随后出现偏瘫或四肢瘫、昏迷及死亡。头颅计算机断层扫描、脑脊液或脑血管造影均无诊断性发现;仅通过尸检确诊。所有4例患者的血管炎均局限于脑部,累及大动脉、小动脉和静脉,或大小血管均受累。病因差异及病理状况的不同细节表明,GAB是一种非特异性反应,而非独特的疾病。此外,未经组织学证实无法确诊。活检标本是在活体患者中确定诊断的唯一方法。

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