Sivaroja Yellaturi, Sowmini P R, Muralidharan K, Reddy P G Pavan Kumar, Mugundhan K
Department of Neurology, Government Stanley Medical College Hospital, Chennai, Tamil Nadu, India.
J Neurosci Rural Pract. 2024 Apr-Jun;15(2):313-319. doi: 10.25259/JNRP_603_2023. Epub 2024 Mar 21.
Demyelinating diseases of central nervous system (CNS) are a broad spectrum of conditions with autoimmune process against myelin. In a resource limited country like India, it is imperative to perform proper clinical evaluation, neuroimaging to differentiate among various categories of CNS demyelinating diseases to decide regarding further workup and treatment. The objective of our study was to determine clinical presentation, imaging findings, serology results, diagnosis, and treatment outcome of primary demyelinating disorders of CNS.
In this prospective study, a total of 44 patients were enrolled over a period of 1 year. After proper evaluation, patients were categorized into different groups applying newer diagnostic criteria. Patients were treated with steroids, appropriate immunomodulatory therapy, and outcomes were analyzed.
The majority of cases were of neuromyelitis optica spectrum disorder (NMOSD) (45.5%) with an overall female-to-male ratio of 3.4:1 and mean age of presentation was 30.5 ± 11.15. Myelitis (52.3%) followed by optic neuritis (45.5%) was the most common initial presentation. The most common site of involvement on magnetic resonance imaging was the spinal cord (particularly the cervicodorsal cord). The majority showed good response to therapy (77.27%) and two patients did not survive.
Higher disability observed among seropositive NMOSD patients warrants aggressive treatment during the first attack itself. It is important to suspect myelin oligodendrocyte glycoprotein antibody disease in patients with preceding viral infection. A good outcome in the majority is likely due to the availability of serological assays and aggressive immunomodulatory therapy.
中枢神经系统（CNS）脱髓鞘疾病是一系列针对髓鞘的自身免疫性疾病。在像印度这样资源有限的国家，进行恰当的临床评估和神经影像学检查以区分各类中枢神经系统脱髓鞘疾病，对于决定进一步的检查和治疗至关重要。我们研究的目的是确定中枢神经系统原发性脱髓鞘疾病的临床表现、影像学表现、血清学结果、诊断及治疗结果。
在这项前瞻性研究中，1年内共纳入44例患者。经过恰当评估后，根据新的诊断标准将患者分为不同组。患者接受类固醇、适当的免疫调节治疗，并对结果进行分析。
大多数病例为视神经脊髓炎谱系障碍（NMOSD）（45.5%），总体男女比例为3.4:1，平均发病年龄为30.5±11.15岁。脊髓炎（52.3%）继以视神经炎（45.5%）是最常见的首发表现。磁共振成像最常受累的部位是脊髓（尤其是颈胸段脊髓）。大多数患者对治疗反应良好（77.27%），2例患者死亡。
血清学阳性的NMOSD患者中观察到的较高残疾率，使得在首次发作时就需要积极治疗。对于先前有病毒感染的患者，怀疑髓鞘少突胶质细胞糖蛋白抗体病很重要。大多数患者预后良好可能归因于血清学检测方法的可用性和积极的免疫调节治疗。
