Mpateni Siviwe S, Sihlali Naye C, Gardiner Emma C, Gigi Nkululo
Department of Diagnostic Radiology, Livingstone Tertiary Hospital, Port Elizabeth, South Africa.
Rheumatology Department, Livingstone Tertiary Hospital, Port Elizabeth, South Africa.
SA J Radiol. 2018 Aug 30;22(1):1306. doi: 10.4102/sajr.v22i1.1306. eCollection 2018.
We present the case of a 23-year-old female with a subacute history of complex additive neurology which consisted of progressive unilateral visual impairment and subsequent blindness of the right eye, in conjunction with distal lower motor neuron symptoms of weakness and sensory loss from T4 level down. Special investigations performed, included serology and an urgent magnetic resonance imaging (MRI) of the brain and spinal cord, which exhibited a diffuse demyelinating disease of the brain and spinal cord without the typical features of multiple sclerosis (MS) and laboratory findings, which were positive for the AQP-4 antibody, confirming the diagnosis of neuromyelitis optica (NMO). Pulsed methylprednisolone was initiated urgently with good effect and immunosuppression with cyclophosphamide was added after the exclusion of additional pathology. She experienced a complete resolution of her weakness and sensory impairment upon discharge; however, her unilateral visual loss remained. The recent advances in the identification of autoimmune biomarkers and the widening spectrum of imaging findings in NMO necessitate that the clinician and radiologist keep abreast of the current diagnostic tools and criteria that distinguish NMO from other demyelinating conditions.
我们报告一例23岁女性病例，其患有复杂的亚急性附加神经病，表现为进行性单侧视力损害，随后右眼失明，同时伴有T4水平以下远端下运动神经元症状，即无力和感觉丧失。所进行的特殊检查包括血清学检查以及脑部和脊髓的紧急磁共振成像（MRI），结果显示脑部和脊髓存在弥漫性脱髓鞘疾病，但无多发性硬化（MS）的典型特征，实验室检查结果显示水通道蛋白4（AQP-4）抗体呈阳性，从而确诊为视神经脊髓炎（NMO）。紧急开始使用脉冲甲基强的松龙治疗，效果良好，在排除其他病变后加用环磷酰胺进行免疫抑制治疗。出院时，她的无力和感觉障碍完全缓解；然而，她的单侧视力丧失仍然存在。NMO自身免疫生物标志物识别方面的最新进展以及其影像学表现范围的扩大，要求临床医生和放射科医生及时了解将NMO与其他脱髓鞘疾病区分开来的当前诊断工具和标准。
