儿童原发性中枢神经系统血管炎五例临床分析

Clinical analysis of 5 cases of children with primary angiitis of the central nervous system

目的 探讨儿童原发性中枢神经系统血管炎（cPACNS）的临床表现及诊治特点,提高对该病的认识.方法 总结云南省第一人民医院2009年1月至2013年12月收治的5例cPACNS患儿的临床资料,对其临床表现及实验室检查结果进行分析总结.结果 cPACNS 5例首诊均误诊,从初次就诊到明确诊断平均（4±6）个月;临床表现5例均有不同程度的头痛,1例头痛剧烈,2例伴视力下降,1例听力下降,2例继发癫痫发作;5例均有轻度脑脊液异常;1例ESR增快、CRP升高,1例免疫球蛋白IgG升高;5例MRI检查均异常,呈双侧多发病灶,病变范围广泛,累及皮质和深部白质;4例磁共振血管造影（MRA）检查血管异常;治疗上单用糖皮质激素或与环磷酰胺联用均取得较好疗效.结论 cPACNS是一种罕见的主要累及中枢神经系统的自身免疫病,临床诊断困难,对以头痛为主要表现合并有视力下降、听力下降、癫痫发作等局灶性神经系统损害的患儿需警惕。

Objective The purpose of the study was to investigate clinical manifestation and the characters of diagnosis and treatment among children with primary angiitis of the central nervous system （cPACNS） in order to improve awareness of the disease.Methods Clinical data of 5 children with cPACNS in the First People＇s Hospital of Yunnan Province from January 2009 to December 2013 were collected,and the clinical manifestations and laboratory test results were analyzed and summarized.Results Five cases of children with cPACNS were misdiagnosed at the first clinic visit,and were confirmed a clear diagnosis on the average of （4±6） months; clinical manifestations of five cases of varying degrees of headache,one case with severe headache,2 patients with decreased visual acuity,a cases with hearing were loss,two cases with secondarily generalized seizures; five cases with mild abnormal cerebrospinal fluid examination; 1 case with elevated ESR and CRP level,1 case with elevated immunoglobulin IgG level; 5 cases with abnomal MRI examinations,which showed multiple bilateral lesions,diffuse,lesions,involving the cortex and deep white matter; 4 cases had vascular abnormalities on MRA,treated with corticosteroids alone or in combination with cyclophosphamide and achieved good results.Conclusion Children of primary central nervous system vasculitis is ar are autoimmmune disease primarily involving the central nervous system.It is difficult for the clinical diagnosis.Children need to be wary of the major manifestation of headache associated with vision loss,hearing loss,seizures and other focal neurological system damage.