原发性中枢神经系统血管炎的临床特点

目的探讨原发性中枢神经系统血管炎(PACNS)的临床特点。方法对27例PACNS患者的临床资料进行分析。结果PACNS好发于中青年,呈急性或亚急性起病,临床表现以头痛、大脑皮质功能减退、局灶性神经功能缺损及癫疒间样发作为主;头颅MRI示病灶处呈长T1、长T2改变,多有强化;脑组织病理检查示软脑膜血管炎性改变。经糖皮质激素以及抗血小板聚集、清除自由基、降低颅内压、改善微循环等综合治疗,病情明显改善。结论PACNS临床表现多样,综合临床表现、影像学及脑组织活检可作出诊断;应用糖皮质激素治疗可显著提高疗效。

Clinical features of primary angiitis of the central nervous system

Objective To investigate the clinical features of primary angiitis of the central nervous system (PACNS). Methods The clinical data of 27 patients with PACNS were analyzed. Results PACNS commonly occured in young and middli-aged adult and with acute or subacute onset. The clinical manifestations were mainly including headache,decrudescence of cortical functions,focal neurological deficits and seizures. The hypointensity signals on T1WI and T2WI were showed and could be enhanced at lesions by skull MRI. The pia vessels inflammatory lesions were revealed in brain by pathological examination. The treated with glucocorticoid and combined therapy such as anti platelet aggregation,scavenging free radicals,reducing intracranial pressure,improving microcirculation and promoting neuronic metabolism,the condition get better.Conclusions The clinical manifestation of PACNS is various. The diagnosis can be make by combining the data of clinical features,imaging and brain biopsy.The treatment with glucocorticoid can obtain better efficacy.