Sindhu Dodmalur Mallikarjuna, Holla Vikram V, Prasad Shweta, Kamble Nitish, Netravathi Manjunath, Yadav Ravi, Pal Pramod K
Department of Neurology National Institute of Mental health and Neurosciences Bengaluru India.
Department of Clinical Neurosciences National Institute of Mental health and Neurosciences Bengaluru India.
Mov Disord Clin Pract. 2021 Jun 4;8(6):875-884. doi: 10.1002/mdc3.13250. eCollection 2021 Aug.
Osmotic demyelination syndrome (ODS) can be a central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) based on the regions involved even though they share the same disease process, aetiopathogenesis and time course.
Present study aims to characterize the clinical, radiological features and the outcome of patients with ODS with movement disorders as the forthcoming manifestation.
Chart review of patients with ODS with movement disorders. Demographic, clinical and radiological details of the patients were reviewed.
Eleven patients (six females; mean age: 48.3 ± 17.6 years) were included in the study. Parkinsonism alone and parkinsonism with dystonia was noted in four patients each (36.4%) while dystonia alone was noted in the other 3 (27.3%). Five patients (45.5%) had postural tremors. While 5 patients had dystonia early in the course of illness (3-7 days), it was delayed (6-9 months) in the other 2. A triphasic course was noted in two patients. The first phase of hyponatremia induced neurological impairment was followed by a second phase of worsening due to the immediate effect of ODS and a third delayed phase of worsening due to delayed effect of ODS. MRI showed both EPM and CPM in eight patients, EPM alone in two patients and CPM alone in 1 patient. Nine patients had a good outcome with mRS < 3.
Parkinsonism and dystonia are important manifestations of ODS. Triphasic course with a delayed phase of worsening of movement disorders is probably due to the maladaptive neuronal repair. The concept of triphasic ODS is first being described in our series.
渗透性脱髓鞘综合征（ODS）可根据受累区域分为中央脑桥髓鞘溶解症（CPM）和脑桥外髓鞘溶解症（EPM），尽管它们具有相同的疾病过程、病因发病机制和病程。
本研究旨在描述以运动障碍为首发表现的ODS患者的临床、影像学特征及预后。
对有运动障碍的ODS患者进行病历回顾。回顾患者的人口统计学、临床和影像学细节。
本研究纳入11例患者（6例女性；平均年龄：48.3±17.6岁）。4例患者（36.4%）出现单纯帕金森综合征，4例患者（36.4%）出现帕金森综合征合并肌张力障碍，另外3例患者（27.3%）出现单纯肌张力障碍。5例患者（45.5%）有姿势性震颤。5例患者在病程早期（3 - 7天）出现肌张力障碍，另外2例患者出现较晚（6 - 9个月）。2例患者呈现三相病程。第一阶段为低钠血症引起的神经功能损害，第二阶段由于ODS的直接作用导致病情恶化，第三阶段由于ODS的延迟作用导致病情再次恶化。MRI显示8例患者同时存在EPM和CPM，2例患者仅存在EPM，1例患者仅存在CPM。9例患者预后良好，改良Rankin量表（mRS）评分<3分。
帕金森综合征和肌张力障碍是ODS的重要表现。运动障碍恶化的延迟期三相病程可能是由于神经元修复适应不良所致。三相ODS的概念首次在我们的系列研究中被描述。
