Khadka Bibek, Bhattarai Ayush Mohan, Dhakal Bishal, Karki Abinash, Acharya Apshara, Poudel Raju
Nepalese Army Institute of Health Sciences, Sanobharyang, Kathmandu, Nepal.
Department of Medicine, Shree Birendra Hospital, Chaunni, Kathmandu, Nepal.
Ann Med Surg (Lond). 2022 May 15;78:103757. doi: 10.1016/j.amsu.2022.103757. eCollection 2022 Jun.
Neuromyelitis Optica (NMO; Devic syndrome,1894) is a CNS demyelinating syndrome. Significant proportion of neuromyelitis optica spectrum disorder is associated with Anti AQ4 Ab. The revised diagnostic criteria for neuromyelitis optica spectrum disorder (2015) has been proposed on the basis of Anti AQ4 Ab status. Most of cases reported has been found in females. It presents with multiple remissions. Common features of acute myelitis and optic neuritis seems to be the usual presentation.
Herein we report a case of a 35-year-old male with longitudinally extending transverse myelitis and Optic Neuritis with confirmation of Anti AQ4 Ab negative status with presentation of bilateral below knee weakness and incontinence of bowel and bladder. It was confirmed by Magnetic Resonance Imaging.
Seronegative neuromyelitis optica spectrum disorder recently classified by 2015 diagnostic criteria associated with strict clinical presentations neuroimaging findings and exclusions of differentials. It presents with a poor prognosis particularly in relapsing course.
We report a case of seronegative neuromyelitis optica spectrum disorder. The prognosis of relapsing course is poor. Early diagnosis and immunomodulators are required to decrease chances of recurrence. Further development of diagnostic modalities in seronegative neuromyelitis optica spectrum disorder is required.
视神经脊髓炎（NMO；德维克综合征，1894年）是一种中枢神经系统脱髓鞘综合征。相当一部分视神经脊髓炎谱系障碍与抗AQ4抗体相关。基于抗AQ4抗体状态，提出了视神经脊髓炎谱系障碍的修订诊断标准（2015年）。已报道的大多数病例见于女性。它表现为多次缓解。急性脊髓炎和视神经炎的常见特征似乎是其常见表现。
在此，我们报告一例35岁男性，患有纵向延伸的横贯性脊髓炎和视神经炎，抗AQ4抗体阴性，表现为双侧膝下无力及大小便失禁。通过磁共振成像得以确诊。
血清阴性视神经脊髓炎谱系障碍最近根据2015年诊断标准进行分类，与严格的临床表现、神经影像学表现及鉴别诊断排除相关。其预后较差，尤其是在复发病程中。
我们报告一例血清阴性视神经脊髓炎谱系障碍病例。复发病程的预后较差。需要早期诊断和免疫调节剂来降低复发几率。血清阴性视神经脊髓炎谱系障碍的诊断方法需要进一步发展。
