Marrodan Mariano, Gaitán María I, Correale Jorge
Neurology Department, Fleni, C1428AQK Buenos Aires, Argentina.
Biomedicines. 2020 May 22;8(5):130. doi: 10.3390/biomedicines8050130.
Diagnostic accuracy is poor in demyelinating myelopathies, and therefore a challenge for neurologists in daily practice, mainly because of the multiple underlying pathophysiologic mechanisms involved in each subtype. A systematic diagnostic approach combining data from the clinical setting and presentation with magnetic resonance imaging (MRI) lesion patterns, cerebrospinal fluid (CSF) findings, and autoantibody markers can help to better distinguish between subtypes. In this review, we describe spinal cord involvement, and summarize clinical findings, MRI and diagnostic characteristics, as well as treatment options and prognostic implications in different demyelinating disorders including: multiple sclerosis (MS), neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, anti-myelin oligodendrocyte glycoprotein antibody-associated disease, and glial fibrillary acidic protein IgG-associated disease. Thorough understanding of individual case etiology is crucial, not only to provide valuable prognostic information on whether the disorder is likely to relapse, but also to make therapeutic decision-making easier and reduce treatment failures which may lead to new relapses and long-term disability. Identifying patients with monophasic disease who may only require acute management, symptomatic treatment, and subsequent rehabilitation, rather than immunosuppression, is also important.
脱髓鞘性脊髓病的诊断准确性较差,因此在日常临床实践中给神经科医生带来了挑战,主要原因是每种亚型都涉及多种潜在的病理生理机制。将临床症状和表现的数据与磁共振成像(MRI)病变模式、脑脊液(CSF)检查结果以及自身抗体标志物相结合的系统诊断方法,有助于更好地区分不同亚型。在这篇综述中,我们描述了脊髓受累情况,总结了不同脱髓鞘性疾病(包括多发性硬化症(MS)、视神经脊髓炎谱系障碍、急性播散性脑脊髓炎、抗髓鞘少突胶质细胞糖蛋白抗体相关疾病以及胶质纤维酸性蛋白IgG相关疾病)的临床发现、MRI表现及诊断特征,以及治疗选择和预后影响。深入了解个体病例的病因至关重要,这不仅能为疾病是否可能复发提供有价值的预后信息,还能使治疗决策更容易,并减少可能导致新的复发和长期残疾的治疗失败情况。识别出可能仅需急性处理、对症治疗及后续康复而非免疫抑制的单相病程疾病患者也很重要。