Background and purpose: This study is to report the clinical, neuroimaging and pathological characteristics of patients with primary angiitis of the central nervous system (PACNS) from Southern China.
Methods: Eight patients with PACNS admitted between August 1995 and April 2006 were retrospectively studied. Records of clinical features, neuroimaging, brain biopsy and therapy were analyzed.
Results: Primary angiitis of the central nervous system occurred predominantly in youth and middle-aged adults. Headache, hemiplegia and speech disturbance were the most predominant manifestations. The cerebrospinal fluid (CSF) was slightly abnormal in only one case, MRI was abnormal in seven, magnetic resonance angiography in seven, diffusion-weighted imaging in five and digital subtraction angiography in four. Brain biopsy in four cases revealed lymphocytic angiitis. All cases had good outcome with the treatment of single corticoid or cyclophosphamide.
Conclusion: We report eight cases of PACNS from Southern China associated with neurological and neuroimaging abnormalities; these patients presented a mild to moderate inflammatory disease that was correlated with few CSF abnormalities and good response to single steroid or cyclophosphamide treatment without relapses. Although brain biopsy represents the gold standard for diagnosis of PACNS, considering the difficulty and challenge of identification of this disease, combination with other examinations might be necessary to arrive at an early and definitive diagnosis.