Clinical Neuroimmunology Unit, Department of Neurology, CHU Liège, University Hospital of Liège, Liège, Belgium.
GIGA-CRC in Vivo Imaging, University of Liège, Liège, Belgium.
Acta Neurol Belg. 2022 Feb;122(1):135-144. doi: 10.1007/s13760-021-01712-3. Epub 2021 Jun 7.
To emphasize physio-pathological, clinical and prognosis differences between conditions causing serious and sometimes very similar clinical manifestations: anti-aquaporin-4 (AQP4) and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies related diseases, and seronegative NMOSD (neuromyelitis optica spectrum disorders).
Based on Wingerchuk et al. (Neurology 85:177-189, 2015) criteria for NMOSD and on those more recently proposed by Jarius et al. (J Neuroinflammation 15:134, 2018) for MOGAD (MOG associated disorders), we retrospectively surveyed 10 AQP4-NMOSD, 8 MOGAD and 2 seronegative NMOSD, followed at the specialized neuroimmunology unit of the CHU Liège.
Female predominance was only observed in AQP4 group. Age at onset was 37.8 and 27.7 years old for AQP4-NMOSD and MOGAD respectively. In both groups, the first clinical event most often consisted of optic neuritis (ON), followed by isolated myelitis. Fifteen of our 20 patients encountered a relapsing course with 90% relapses in AQP4-NMOSD, 62.5% in MOGAD and 50% in seronegative group, and a mean period between first and second clinical event of 7.1 and 4.8 months for AQP4-NMOSD and MOGAD, respectively. In total we counted 54 ON, with more ON per patient in MOGAD. MOG-associated ON mainly affected the anterior part of the optic nerve with a papilledema in 79.2% of cases. Despite a fairly good visual outcome after MOG-associated ON, retinal nerve fibre layer (RNFL) thickness decreased, suggesting a fragility of the optic nerve toward further attacks.
As observed in larger cohorts, our MOGAD and AQP4-NMOSD cases differ by clinical and prognostic features. A better understanding of these diseases should encourage prompt biological screening and hasten proper diagnosis and treatment.
强调导致严重且有时非常相似临床表现的疾病之间的生理病理、临床和预后差异：抗水通道蛋白-4（AQP4）和抗髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病，以及血清阴性 NMOSD（视神经脊髓炎谱系疾病）。
根据 Wingerchuk 等人（神经病学 85:177-189, 2015）制定的 NMOSD 标准，以及 Jarius 等人（神经炎症 15:134, 2018）最近为 MOGAD（MOG 相关疾病）制定的标准，我们回顾性调查了在列日 CHU 专门的神经免疫科就诊的 10 例 AQP4-NMOSD、8 例 MOGAD 和 2 例血清阴性 NMOSD 患者。
仅在 AQP4 组观察到女性优势。AQP4-NMOSD 和 MOGAD 的发病年龄分别为 37.8 岁和 27.7 岁。在这两组中，首次临床事件最常见的是视神经炎（ON），其次是孤立性脊髓炎。我们的 20 名患者中有 15 名经历了复发病程，AQP4-NMOSD 的复发率为 90%，MOGAD 的复发率为 62.5%，血清阴性组的复发率为 50%，AQP4-NMOSD 和 MOGAD 的首次和第二次临床事件之间的平均间隔时间分别为 7.1 个月和 4.8 个月。我们共记录了 54 例 ON，MOGAD 中每位患者的 ON 更多。MOG 相关的 ON 主要影响视神经的前部，79.2%的病例伴有视乳头水肿。尽管 MOG 相关的 ON 后视力恢复良好，但视网膜神经纤维层（RNFL）厚度减少，这表明视神经对进一步攻击较为脆弱。
与较大的队列观察结果一致，我们的 MOGAD 和 AQP4-NMOSD 病例在临床和预后特征上存在差异。更好地了解这些疾病应鼓励及时进行生物学筛查，并加快正确的诊断和治疗。
