Abstract：
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.
CONCLUSIONS: 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.
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.
CONCLUSIONS: 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.
摘要：
背景：视神经脊髓炎谱系障碍（NMOSD）是一种罕见的神经炎性疾病，其特征是复发性复发。最常见的体征是脊髓炎和视神经炎。它也可以表现为脑或脑干综合征。其诊断和治疗仍面临诸多挑战,并且需要长期的随访研究来观察疾病随时间的发展过程。
方法：我们从2015年10月开始在卡沙尼医院建立了NMOSD患者的电子注册系统，伊斯法罕,伊朗。记录每个可疑患者并将其纳入随访系统以调查其病程。通过基于细胞的测定方法检查所有的抗水通道蛋白4(AQP4)抗体。记录所有信息，例如人口统计学和临床数据以及实验室和MRI检查结果。参与者接受了任何复发的随访，新的临床旁试验和药物变化。本研究基于明确的NMOSD病例（根据2015年标准）特征和7年注册期间的临床过程。
结果：本研究纳入173例NMOSD病例，56例AQP4Ab血清阳性。他们的平均年龄为40.02±11.11岁（血清阳性组45.78岁）。发病时的平均年龄约为30.16岁。我们的注册系统平均随访时间为55.84±18.94个月（血清阳性的为54.82个月）。年复发率估计为0.47±0.36。77例(44.5%)患者的基线MRI中存在长延伸性脊髓炎(LETM),而其中32例没有表现出任何相关的临床症状。124例患者首次脑部MRI显示异常。27人患有甲状腺功能减退症是最常见的共病。这种疾病似乎在伊斯法罕省的西部和西南部地区更为普遍。
结论：平均发病年龄高于多发性硬化（MS）患者，但也有值得注意的儿科病例。还应当注意的是,宫颈LETM最初可以是无症状的。经常观察到脑部MRI异常。该疾病在显示高MS患病率的地理区域中更为普遍。
方法：我们从2015年10月开始在卡沙尼医院建立了NMOSD患者的电子注册系统，伊斯法罕,伊朗。记录每个可疑患者并将其纳入随访系统以调查其病程。通过基于细胞的测定方法检查所有的抗水通道蛋白4(AQP4)抗体。记录所有信息，例如人口统计学和临床数据以及实验室和MRI检查结果。参与者接受了任何复发的随访，新的临床旁试验和药物变化。本研究基于明确的NMOSD病例（根据2015年标准）特征和7年注册期间的临床过程。
结果：本研究纳入173例NMOSD病例，56例AQP4Ab血清阳性。他们的平均年龄为40.02±11.11岁（血清阳性组45.78岁）。发病时的平均年龄约为30.16岁。我们的注册系统平均随访时间为55.84±18.94个月（血清阳性的为54.82个月）。年复发率估计为0.47±0.36。77例(44.5%)患者的基线MRI中存在长延伸性脊髓炎(LETM),而其中32例没有表现出任何相关的临床症状。124例患者首次脑部MRI显示异常。27人患有甲状腺功能减退症是最常见的共病。这种疾病似乎在伊斯法罕省的西部和西南部地区更为普遍。
结论：平均发病年龄高于多发性硬化（MS）患者，但也有值得注意的儿科病例。还应当注意的是,宫颈LETM最初可以是无症状的。经常观察到脑部MRI异常。该疾病在显示高MS患病率的地理区域中更为普遍。
