■在SLE患者中,并发NMOSD可表现为视神经炎和横贯性脊髓炎。AQP-4抗体阳性证实诊断。及时治疗对于控制急性症状和预防复发至关重要,正如一名年轻患者的视神经炎和广泛的脊髓病变所强调的那样。
■视神经脊髓炎谱系障碍(NMOSD)是一种罕见的中枢神经系统自身免疫性疾病,影响视神经和脊髓。它与针对水通道蛋白4(AQP-4)和/或髓鞘少突胶质细胞糖蛋白的自身抗体相关。它是根据临床诊断的,放射学,和血清学标准,并在急性期用免疫抑制剂治疗。长期免疫抑制对于预防潜在的复发至关重要。在这个案例报告中,我们介绍了一个19岁女性患者的系统性红斑狼疮(SLE),她的左眼出现模糊和视力丧失。光学相干断层扫描正常,但是钆增强的颈背MRI显示多个病变从脑干延伸到C7-T1交界处,提示纵向广泛的横贯性脊髓炎(LETM),其中最大的是颈脊髓交界处的囊性病变。造影剂注射也显示左视神经炎。脑脊液分析显示IgG和红细胞计数升高,但没有寡克隆带。患者AQP-4自身抗体检测呈阳性,确认NMOSD的诊断。静脉注射甲基强的松龙治疗导致部分改善,但是病人出现了严重的神经症状,包括四肢瘫痪和膀胱和肠功能障碍。此病例说明在鉴别诊断存在视神经炎和/或脊髓炎的SLE患者中考虑NMOSD的重要性。尤其是当MRI检查结果提示LETM时。早期诊断和坚持治疗对于防止进一步复发和有害后遗症至关重要。
UNASSIGNED: In patients with SLE, concurrent NMOSD can manifest with optic neuritis and transverse myelitis. AQP-4 antibody positivity confirms the diagnosis. Prompt treatment is critical to manage the acute symptoms and prevent relapses, as highlighted by a young patient\'s
case with optic neuritis and extensive spinal cord lesions.
UNASSIGNED: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder of the central nervous system that affects the optic nerve and spinal cord. It is associated with autoantibodies against aquaporin-4 (AQP-4) and/or myelin oligodendrocytes glycoproteins. It is diagnosed based on clinical, radiological, and serological criteria, and treated with immunosuppressants in the acute phase. Long-term immunosuppression is essential to prevent potential relapses. In this
case report, we present the
case of a 19-year-old female patient with systemic lupus erythematosus (SLE), who presented with blurriness and loss of vision in her left eye. Optical coherence tomography was normal, but a gadolinium-enhanced cervico-dorsal MRI showed multiple lesions extending from the brainstem to the C7-T1 junction suggestive of longitudinally extensive transverse myelitis (LETM), the largest of which was a cystic lesion at the cervico-spinal junction. A contrast injection also revealed left optic neuritis. Cerebrospinal fluid analysis showed elevated IgG and red blood cell count, but no oligoclonal bands. The patient tested positive for AQP-4 autoantibodies, confirming the diagnosis of NMOSD. Treatment with intravenous methylprednisolone led to partial improvement, but the patient experienced a relapse with severe neurological symptoms, including tetraplegia and bladder and bowel dysfunction. This
case illustrates the importance of considering NMOSD in the differential diagnosis of patients with SLE who present with optic neuritis and/or myelitis, especially when MRI findings are suggestive of LETM. Early diagnosis and adherence to treatment are crucial to prevent further relapses and deleterious sequelae.