■视神经脊髓炎谱系障碍(NMOSD)是与针对水通道蛋白4(AQP4-IgG)的IgG抗体相关的中枢神经系统(CNS)的自身免疫性疾病。来自撒哈拉以南非洲(SSA)的NMOSD流行病学几乎没有发表。
■我们回顾性整理了我们三级区域神经科中心收治的NMOSD病例。
■我们确定了11例(10例女性,平均年龄30岁)。64%(7/11)的AQP4-IgG血清阳性,使用间接免疫荧光测量。剩下的案件要么负担不起测试,或者有病理放射学特征。57%(4/7)的血清阳性病例并发/近期CNS感染。所有患者均接受大剂量静脉注射甲泼尼龙(IVMP)治疗,36%(4/11)也进行了血浆置换。只有55%(6/11)的患者在就诊时被神经科医生看到:他们的复发较少(1.3vs2.4),诊断延迟较少(2.3个月比7.4个月),在我们审查期结束时,残疾程度较低。10例长期免疫抑制:60%的霉酚酸酯,30%硫唑嘌呤,还有一个是利妥昔单抗.
■我们的研究是东非地区最大的NMOSD病例系列。患者面临着获得适当和负担得起的检测的挑战,以及在发病时及时提供神经科医生,这对他们的功能结果有影响。大多数血清阳性病例近期/并发中枢神经系统感染,提示触发了自身免疫.
UNASSIGNED: Neuromyelitis optica spectrum disorder (NMOSD) is an auto-immune disease of the central nervous system (CNS) associated with the IgG-antibody against aquaporin-4 (AQP4-IgG). There is little published epidemiology of NMOSD from sub-Saharan Africa (SSA).
UNASSIGNED: We retrospectively collated NMOSD cases admitted to our tertiary regional neurology centre.
UNASSIGNED: We identified 11 cases (10 female, average age 30 years). 64% (7/11) were seropositive for AQP4-IgG, measured using indirect immunofluorescence. The remaining cases could either not afford tests, or had pathognomonic radiological features. 57% (4/7) of seropositive cases had concurrent/recent CNS infection. All patients were treated with high-dose intravenous methylprednisolone (IVMP), and 36% (4/11) also had plasma exchange. Only 55% (6/11) of the patients were seen by a neurologist at presentation: they had less relapses (1.3 vs 2.4), less diagnostic delay (2.3 vs 7.4 months), and were less disabled at the end of our review period. 10 cases were immunosuppressed long-term: 60% on mycophenolate, 30% azathioprine, and one on rituximab.
UNASSIGNED: Our study is the largest case series of NMOSD from the East Africa region. Patients faced challenges of access to appropriate and affordable testing, and timely availability of a neurologist at onset, which had impacts on their functional outcomes. The majority of the seropositive cases had recent/concurrent CNS infections, suggesting triggered auto-immunity.