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Background Painful tonic spasm (PTS) is a common symptom in patients with neuromyelitis optica spectrum disorders (NMOSD). This study aimed to obtain further insights into the prevalence, characteristics, and treatment of PTS in patients with NMOSD, and to systematically investigate and compare the clinical features and prognosis of NMOSD with and without PTS. Methods We reviewed the medical records and prospectively interviewed patients with NMOSD who attended the West China Hospital of Sichuan University in Chengdu, China between September 2014 and December 2016. Results In total, 52 of the 230 patients with NMOSD experienced PTS (22.61%). Patients with NMOSD and PTS were characterized by a higher age at onset ( P = 0.017), higher annual relapse rate (ARR) ( P = 0.003), higher ARR of myelitis ( P = 0.011), and a tendency to experience pruritus ( P = 0.025). Sodium channel blocking antiepileptic drugs (carbamazepine or oxcarbazepine) had higher efficacy than gabapentin in the treatment of PTS ( P = 0.001). Although the progression index was higher in patients with PTS, this difference did not reach statistical significance ( P = 0.05). Conclusions Our study suggested that immunosuppressors for the prevention of relapse should be administered without delay in patients with NMOSD and PTS. Owing to the side effects of carbamazepine, we recommend oxcarbazepine as the first-line of treatment for PTS in patients with NMOSD. Whether PTS is a marker of disease severity in NMOSD remains to be determined, requiring a long-term prospective observational study. [ABSTRACT FROM AUTHOR]

• The left temporal lobe and adjacent regions showed cortical changes in the W-NP group. • White matter tracts started from ROIs showed decreased MD and RD in the W-NP group. • MD and RD decreased in extensive white matter skeleton in the W-NP group. • Over time, lower mean reduction rates of LGI of ROIs was found in the W-NP group. • Different longitudinal changes related to NP was found in the extensive white matter. Neuropathic pain (NP) is a highly disturbing sensory experience in patients with neuromyelitis optica spectrum disorders (NMOSD). However, the brain changes in NMOSD patients with NP have rarely been studied. The aim of the cross-sectional and follow-up longitudinal study was to investigate the brain changes in NMOSD patients with NP. In the cross-sectional study, comparisons were performed between groups with NP (W-NP) and without NP (Wo-NP), and age, sex and years of education were adjusted. We compared the voxel-wise whole-brain gray matter (GM) volume, cortical thickness (CT), cortical surface area (CSA) and local gyrification index (LGI). Probabilistic tractography started from regions with significant between-group differences in GM volume, CT, CSA and LGI. We also compared fractional anisotropy (FA), mean diffusivity (MD), radial diffusivity (RD) and axial diffusivity (AD) of the white matter (WM) skeleton using Tract-Based Spatial Statistics (TBSS). In the longitudinal study, the patients were followed for 2.0±0.0 years and underwent the same imaging scanning as the cross-sectional study. Changes of the CT, CSA, LGI and WM were obtained. Patients in the W-NP group were older than those in the Wo-NP group and showed significantly reduced LGI of the left temporal lobe and adjacent regions(regions of interest, ROIs), which participated in neuropathic pain processing, possibly by emotion and attention control. Probabilistic tractography started from ROIs, and the generated WM tracts showed decreased MD and RD in the W-NP group compared to the Wo-NP group. Using TBSS, both MD and RD decreased in extensive WM skeleton in the right hemisphere of the patients in the W-NP group. Additionally, in the follow-up longitudinal study, compared with patients in the Wo-NP group, patients in the W-NP group showed lower mean reduction rates of LGI of ROIs, and less increase of FA and more increases of MD, AD and RD in the extensive WM skeleton. These findings support the hypothesis that brain changes might correlate with NP in NMOSD patients and predict the changes related to NP over time. [ABSTRACT FROM AUTHOR]

• Providing structural MRI markers to unveil the pathophysiology underlying female NMO patients. • The first structural morphology study of NMO patients with pain. • Provide new insights for pain mechanism in NMO patients. Neuromyelitis optica (NMO) is a disease characterised by severe relapses of optic neuritis and longitudinally extensive transverse myelitis and it has a strong female predilection. Pain is one of the most typical symptom in NMO. However, few studies have been conducted to examine the neuropathic pain mechanism of NMO patients or gender-specific effects using magnetic resonance imaging technique. A total of 38 female patients with NMO, 28 with pain (NMOWP) and 10 without pain (NMOWoP), were classified using the Brief Pain Inventory (BPI); 22 healthy females were also recruited. We used the FSL Image Registration and Segmentation Toolbox (FIRST) for subcortical region volumes quantifications, and voxel-based morphometry analysis for cortical gray matter (GM) volume, to examine the brain morphology in NMOWP patients. In addition, correlation test between structural measurements of NMO patients and clinical indexes was also performed. The results showed: 1) no significant differences in cortical GM density between the NMOWP and NMOWoP groups; 2) significantly smaller hippocampus and pallidum volumes in the NMOWP group compared with the NMOWoP group; 3) significant negative correlation between the average BPI and volumes of the accumbens nucleus and thalamus in NMO patients. These results revealed that structural abnormality exists in NMO female patients who have pain, with significant implications for our understanding of the brain morphology in NMO patients with pain. [ABSTRACT FROM AUTHOR]