神经白塞氏病1例临床报道并文献回顾

Clinical report of a case on neuro-Behcet’s disease and review of relevant literature

白塞氏病是一种病因未明的以细小血管炎为病理基础的慢性、复发性、全身性、免疫性疾病。在临床上当白塞氏病出现神经系统病变时,则称为神经白塞氏病,是白塞氏病较为少见且最为严重的表现形式之一。神经白塞氏病根据累及中枢神经系统或周围神经系统的不同分为中枢型神经白塞氏病与周围型神经白塞氏病。神经白塞氏病发生中枢神经系统受累时依据损害部位又可分为实质型神经白塞氏病和非实质型神经白塞氏病。本文报道1例以认知功能障碍及神经功能缺损为主要临床表现的实质型神经白塞氏病,并对相关文献进行回顾。

Behcet’s disease is a chronic,recurrent,systemic,immune disease with unknown etiology and with microvascular inflammation as the pathologic basis.Clinically,when neurological lesions are present in Behcet’s disease,it is called neuro-Behcet’s disease,which is one of the less common and most serious manifestations of Behcet’s disease.Neuro-Behcet’s disease is divided into central neuro-Behcet’s disease and peripheral neuro-Behcet’s disease depending on the central nervous system or peripheral nervous system it involves.Central neuro-Behcet’s disease can be further divided into parenchymal neuro-Behcet’s disease and non-parenchymal neuro-Behcet’s disease depending on the site of damage when it involves the central nervous system.This paper reports a case of parenchymal neuro-Behcet’s disease with cognitive impairment and neurological deficits as the main clinical manifestations,and reviews relevant literature.