Abstract: Neuromyelitis optica spectrum disorder ofen co-exists with primary Sj?gren’s syndrome. We compared the clinical features of 16 neuromyelitis optica spectrum disorder patients with (n = 6) or without primary Sj?gren’s syndrome (n = 10). All patients underwent extensive clinical, laboratory, and MRI evaluations. Tere were no statistical di?erences in demographics or frst neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sj?gren’s syndrome. The laboratory findings of cerebrospinal ?uid oligoclonal banding, serum C-reactive protein, antinuclear autoantibody, anti-Sj?gren’s-syndrome-related antigen A antibodies, anti-Sj?gren’s-syndrome-related antigen B antibodies, and anti-Sm antibodies were signifcantly higher in patients with primary Sj? gren’s syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% (4/6) of patients with primary Sj?gren’s syndrome and in 60% (6/10) of patients without primary Sj?gren’s syndrome. More brain abnormalities were observed in patients without primary Sj?gren’s syndrome than in those with primary Sj?gren’s syndrome. Segments lesions (> 3 centrum) were noted in 50% (5/10) of patients without primary Sj?gren’s syndrome and in 67% (4/6) of patients with primary Sj?gren’s syndrome. Tese fndings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sj?gren’s syndrome are similar. However, neuromyelitis optica spectrum disorder patients without primary Sj?gren’s syndrome have a high frequency of brain abnormalities.

Key words: nerve regeneration, neuromyelitis optica, primary Sj?gren’s syndrome, neuromyelitis optica spectrum disorder, xerostomia, xerophthalmia, neurological involvements, magnetic resonance imaging, anti-aquaporin 4, neural regeneration