ABSTRACT
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.