Ferilli Michela Ada Noris, Valeriani Massimiliano, Papi Claudia, Papetti Laura, Ruscitto Claudia, Figà Talamanca Lorenzo, Ursitti Fabiana, Moavero Romina, Vigevano Federico, Iorio Raffaele
Neuroscience Department, Bambino Gesù Children's Hospital IRCCS, Rome 00165, Italy.
Neuroscience Department, Bambino Gesù Children's Hospital IRCCS, Rome 00165, Italy; Center for Sensory-Motor Interaction, Aalborg University, Aalborg DK-9220, Denmark.
Mult Scler Relat Disord. 2021 May;50:102837. doi: 10.1016/j.msard.2021.102837. Epub 2021 Feb 10.
Background Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been recently reevaluated as a biomarker of acquired demyelinating syndromes (ADS) of the central nervous system (CNS). Here, we describe the clinical and neuroimaging features, and the long-term outcome of children with ADS of the CNS associated with MOG-IgG. Methods All patients underwent brain and spinal cord magnetic resonance imaging (MRI), lumbar puncture for cerebrospinal fluid (CSF) analysis and MOG-IgG and aquaporin-4 IgG (AQP4-IgG) testing. Results Forty-eight pediatric patients were recruited. MOG-IgG were detected in 11/48 (25%) patients with the following clinical presentations: encephalomyelitis (EM), 8/11 (73%); optic neuritis (ON), 2/11 (18%); transverse myelitis (TM), 1/11 (9%). Patients negative for MOG-IgG were diagnosed with Multiple Sclerosis (MS) (n=15), EM (n=7), ON (n=7), neuromyelitis optica spectrum disorders (NMOSD) (n=5), TM (n=2) and encephalitis (n=1). MOG-IgG positive patients were younger at disease onset and they more frequently experienced encephalopathy and epileptic seizures compared with negative patients. EM and inflammatory lesions involving optic nerves on MRI imaging were more frequent in MOG-IgG positive patients. None of the patients with MOG-IgG became persistently seronegative during the follow-up, although a decrease in MOG-IgG titer was observed. Patients with MOG-IgG showed a good response to therapy and only two patients presented relapses during follow-up. Conclusion This study supports the distinction of MOG autoimmune oligodendrocytopathy as a unique disease entity, with clinical features different from those of MS and AQP4-IgG-positive NMOSD.
背景 髓鞘少突胶质细胞糖蛋白抗体（MOG-IgG）最近被重新评估为中枢神经系统（CNS）获得性脱髓鞘综合征（ADS）的生物标志物。在此，我们描述了与MOG-IgG相关的CNS-ADS患儿的临床和神经影像学特征以及长期预后。方法 所有患者均接受脑和脊髓磁共振成像（MRI）、腰椎穿刺进行脑脊液（CSF）分析以及MOG-IgG和水通道蛋白4 IgG（AQP4-IgG）检测。结果 招募了48例儿科患者。11/48（25%）例患者检测到MOG-IgG，临床表现如下：脑脊髓炎（EM），8/11（73%）；视神经炎（ON），2/11（18%）；横贯性脊髓炎（TM），1/11（9%）。MOG-IgG阴性患者被诊断为多发性硬化（MS）（n = 15）、EM（n = 7）、ON（n = 7）、视神经脊髓炎谱系障碍（NMOSD）（n = 5）、TM（n = 2）和脑炎（n = 1）。与阴性患者相比，MOG-IgG阳性患者发病年龄更小，更频繁地出现脑病和癫痫发作。MOG-IgG阳性患者中，MRI成像上EM和累及视神经的炎性病变更常见。随访期间，没有MOG-IgG阳性患者转为持续血清阴性，尽管观察到MOG-IgG滴度下降。MOG-IgG阳性患者对治疗反应良好，随访期间仅有2例患者复发。结论 本研究支持将MOG自身免疫性少突胶质细胞病作为一种独特的疾病实体进行区分，其临床特征不同于MS和AQP4-IgG阳性的NMOSD。
