ABSTRACT
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.