The aims of this study were to report brain characteristic abnormalities and to evaluate the relationship of blood-brain barrier (BBB) disruption and brain lesions in Chinese patients with NMOSD.Brain magnetic resonance imaging characteristics and cerebrospinal fluid (CSF) laboratory tests of 121 patients with NMOSD at acute attack were reviewed retrospectively. Qalb (CSF albumin/serum albumin) was used for assessment of disruption of BBB.Brain MRI abnormalities were observed in 36.4% (44/121) of the NMOSD patients. Thirty patients (25%) showed typical-NMOSD abnormalities, including dorsal medulla lesions (n = 16, 13.2%), brainstem/cerebellum (n = 11, 9.1%), thalamus/hypothalamus (n = 3, 2.5%), periventricular white matter lesions (n = 4, 3.3%) hemispheric white matter (n = 4, 3.3%). Twenty-five patients (20.7%) had nonspecific lesions. Compared to the NMOSD patients without brain lesion, the proportion of patients who had abnormal BBB permeability was significantly higher in the abnormal brain MRI group (47.7% vs. 27.3%, P < 0.05). BBB permeability was not correlated to distribution of brain lesions or enhancement lesions. Qalb was associated with higher Expanded Disability Status Scale scores (r = 0.689, P < 0.05).Brain lesions are common in NMOSD patients. Marker of BBB permeability is associated with brain lesion and EDSS scores of NMOSD.