Shakir R A, Sulaiman K, Kahn R A, Rudwan M
Department of Neurology, Kuwait University, Ibn Sina Hospital.
Eur Neurol. 1990;30(5):249-53. doi: 10.1159/000117356.
In an attempt to categorize the diverse neurological manifestations of Behçet's syndrome, a group of 10 patients was prospectively studied; the diagnosis of Behçet's syndrome was not known in any of them prior to their neurological presentation. A reasonably clear distinction can be made to divide the manifestations into three general categories with some overlap. Category I includes patients with increased intracranial pressure with or without cerebral venous sinus thrombosis. Category II are those presenting with a stroke. Category III are those patients who present with spinal cord involvement and CSF pleocytosis. Brainstem involvement was noted independently of the group. In general, patients with Neuro-Behçet's syndrome showed little ocular involvement. The course of the illness both in morbidity and mortality tended to be better in category I, and mortality was only seen in category II patients. In populations susceptible to the disease, the clinical categorisation proposed may alert the clinician to the possibility of neuro-Behçet's syndrome whenever patients with pseudotumour cerebri, dural sinus thrombosis, stroke in the young, unexplained myelitis or possible multiple sclerosis are encountered.
为了对贝赫切特综合征的各种神经表现进行分类，我们前瞻性地研究了一组10例患者；在他们出现神经症状之前，均未确诊为贝赫切特综合征。可以做出合理明确的区分，将这些表现分为三大类，且存在一些重叠。第一类包括有或无脑静脉窦血栓形成的颅内压升高患者。第二类是出现中风的患者。第三类是出现脊髓受累和脑脊液淋巴细胞增多的患者。脑干受累情况独立于这些类别被记录。总体而言，神经贝赫切特综合征患者眼部受累较少。在发病率和死亡率方面，第一类患者的病情发展往往较好，只有第二类患者出现了死亡。在易患该疾病的人群中，每当遇到假性脑瘤、硬脑膜窦血栓形成、年轻人中风、不明原因的脊髓炎或可能的多发性硬化症患者时，所提出的临床分类可能会提醒临床医生注意神经贝赫切特综合征的可能性。
