Abraham S, Rajeev E, Girija A S
Department of Neurology, Medical College Hospital, Calicut, Kerala, S. India.
J Assoc Physicians India. 2000 Aug;48(8):838-41.
Behçet's disease (BD) is a multifocal disorder with an immunogenetic basis, which persists over many years. Initial descriptions mentioned oral and genital ulcers with uveitis. Later a number of other manifestations were added, like skin, joint and neurological. The involvement of nervous system (Neuro-Behçet's) is reportedly uncommon. We hereby report four cases of Neuro-Behçet's, i.e.; two cases of strokes involving multiple areas of the central nervous system and two cases had features of benign intracranial hypertension. All cases had mucocutaneous lesions or other system involvement. Cases satisfied the international study group criteria for diagnosis of BD. All cases were pathergy test positive. In comparison with the literature from Turkey and Greece, which reports a high pathergy positivity, reports from India have shown only few cases to be positive. The prognosis of Neuro-Behçet used to be poor but has recently been improved with reduced mortality, although whether this can be attributed to treatment with steroids and/or cytotoxic agents remains uncertain.
白塞病（BD）是一种具有免疫遗传基础的多灶性疾病，病程可持续多年。最初的描述提到口腔溃疡、生殖器溃疡伴葡萄膜炎。后来又增加了许多其他表现，如皮肤、关节和神经方面的表现。据报道，神经系统受累（神经白塞病）并不常见。我们在此报告4例神经白塞病，即2例累及中枢神经系统多个区域的中风病例和2例具有良性颅内高压特征的病例。所有病例均有黏膜皮肤病变或其他系统受累。病例均符合国际研究组的白塞病诊断标准。所有病例的针刺反应试验均为阳性。与土耳其和希腊文献报道的高针刺反应阳性率相比，印度的报道显示只有少数病例为阳性。神经白塞病的预后过去很差，但最近死亡率有所降低，预后有所改善，不过这是否归因于类固醇和/或细胞毒性药物治疗仍不确定。
