Epidemiologic and clinical characteristics of neuromyelitis optica spectrum disorder patients, A seven years follow-up study from Iran.

BACKGROUND: Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare neuroinflammatory disease characterized by recurrent relapses. The most common signs are myelitis and optic neuritis. It can also present by cerebral or brain stem syndromes. There are still many challenges in its diagnosis and treatment, and long-term follow-up studies are needed to see the disease course over time. METHODS: We established an electronic registration system of NMOSD patients starting from October 2015 in Kashani hospital, Isfahan, Iran. Every suspected patient was documented and included in the follow-up system to survey their disease course. Anti-aquaporine 4 (AQP4) antibody checked for all by cell-based assay method. All information such as demographic and clinical data and laboratory and MRI findings were documented. Participants were followed up for any relapses, new paraclinical tests and drug changes. This study is based on the definite NMOSD cases (according to the 2015 criteria) characteristics and clinical course during 7 years of registration. RESULTS: The study included 173 NMOSD cases and 56 ones were seropositive for AQP4 Ab. Their mean age was 40.02±11.11 years (45.78 in the seropositive group). The mean age at disease onset was about 30.16 years. The mean time of follow-up by our registration system is 55.84 ± 18.94 months (54.82 months in seropositive ones). The annual relapse rate is estimated as 0.47±0.36. Long extended transvers myelitis (LETM) was present in the baseline MRI of 77 patients (44.5%), while 32 of them did not show any related clinical symptoms. 124 patients revealed an abnormality in the first brain MRI. 27 individuals suffer hypothyroidism as the most common comorbid disease. The disease seems to be more prevalent in the west and southwest areas of Isfahan province. CONCLUSION: The mean age of onset is higher than Multiple Sclerosis (MS) patients, but there are notable pediatric cases too. It should also be noticed that cervical LETM can be asymptomatic at first. Brain MRI abnormalities are frequently observed. The disease is more prevalent in the geographical areas where showing high MS prevalence.

Pre-pregnancy, obstetric and delivery status in women with neuromyelitis optica spectrum disorder.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is a potentially disabling disease which affects predominantly women of reproductive age. OBJECTIVES: To evaluate the pre-pregnancy condition and to investigate the prevalence of obstetrical and neonatal complaints in NMOSD. METHODS: An observational retrospective study of 37 NMOSD patients positive for aquaporin-4-immunoglobulin-IgG. Age at menarche, menstrual cycle, gravidity, type of delivery obstetrical complications and perinatal problems were recorded. We assessed the annualized relapse rate (ARR). RESULTS: A total of 23 patients (62%) suffered from irregular menstruations and two from infertility after disease onset. 11 patients had 20 informative pregnancies with 14 deliveries (5 cesareans) and six abortions, four spontaneous, and two ectopic pregnancies after the first trimester. Additionally, three patients experienced threatening abortion and one preeclampsia. No stillbirth or premature birth was recorded. None of the patients experienced attacks during pregnancy, but postpartum relapses occurred after 10 deliveries (71,4%) with the highest ARR (0.937; p = 0.037) during the first three months postpartum compared to pre-pregnancy ARR (0.375). Four patients (10.8%) developed the initial symptoms of NMOSD postpartum. CONCLUSION: Irregular menstruation in more than half of NMOSD patients may reflect a hormonal imbalance. Pregnancies were associated with obstetrical complications and increased disease activity postpartum.

Disease course, progression and activity of neuromyelitis optica (NMOSD) in patients who were treated with Rituximab, 6 and 12 months after receiving the first dose of drug, in Isfahan city.

INTRODUCTION: Neuromyelitis optica spectrum disease (NMOSD) is a chronic inflammatory and usually relapsing demyelinating disease which mainly involves optic nerve and spinal cord and also other parts of the central nervous system. Rituximab has been used for some neurological disorders with a probable autoimmune basis. Early and aggressive immunosuppression therapy is necessary to prevent clinical relapses and permanent disability in NMOSD. Rituximab, a monoclonal antibody against B cells, has been found effective in several recent studies. The objective of this study is to evaluate the clinical efficacy of Rituximab as a newly introduced treatment to NMOSD in Iran. METHODS AND MATERIALS: This is an observational cohort study carried out,on 56 patients who have been diagnosed with NMOSD. The patients' information registered in Kashani Hospital MS clinic. They received the first dose of Rituximab for approximately 6 months before the beginning of study. In the first session, every patient was interviewed and examined based on necessary information for the study. Follow up visits were done as needed in a period of 12 months after the first dose. Patient's age, sex, age of onset, presenting symptom, annual relapse rate, acute attacks, EDSS (primary, 6 and 12 months) were recorded. Comparative aims achieved by within group analysis before and after treatment. AntiAQP4 antibody had been checked before the start of study. patients divided into two groups based on the antibody test results for subgroup analysis. Data were analyzed using the IBM SPSS23 - United States software. RESULTS: 56 known cases of NMOSD including 17.85% male and 82.15% female were under study. The Mean disease duration was 87.60±59.65 months and mean duration of treatment was 19.56±8.26. The mean EDSS score was primarily 4.83±1.87 and after treatment it decreased significantly (p-value<0.001) to 3.38±1.57 and 2.87±1.63 (6 months and 12 months later). There was a statistical difference between the annual mean relapse rate before and after treatment (p<0.001). The above mentioned index decreased from 1.43±1.107-0.147±0.27. Also, the mean number of attacks in one year before and after treatment were 1.97±0.57 and 0.28±0.45 with significant statistical difference (p<0.001). In the patients with positive anti AQP4 antibody,the mean EDSS decreased from 4.94±1.83-3.52±1.27 and 2.92±1.54. Annual mean relapse rate also decreased significantly from 1.35±0.85-0.10±0.19. Mean number of attacks in one year before and after treatment were 2.05±0.75-0.27±0.46 (p<0.001) respectively. CONCLUSION: In the current study, we found that Rituximab can significantly improve disability state and also can markedly reduce relapse rate in NMOSD patients.