Shrestha Suraj, Kharel Sanjeev, Gautam Sandesh, Poddar Elisha, Adhikari Sugat, Acharya Suman, Pant Samriddha Raj, Jha Anamika, Ojha Rajeev
Maharajgunj Medical Campus, Tribhuvan University Institute of Medicine, Kathmandu.
Shreegaun Primary Health Care Center, Dang.
Ann Med Surg (Lond). 2023 Feb 17;85(3):477-480. doi: 10.1097/MS9.0000000000000230. eCollection 2023 Mar.
Osmotic demyelination syndrome (ODS) as a result of the hyperosmolar hyperglycemic state is rare and can present with variable neurological manifestation due to lysis of myelin sheath.
A 44-year diabetic male presented with complaints of sudden onset, progressive bilateral weakness in lower limbs, and slurring of speech for the past 1.5 months. Cerebellar examination showed a bilaterally impaired finger nose test, dysdiadochokinesia, impaired heel shin test, and an impaired tandem gait. MRI brain (T2 and fluid-attenuated inversion recovery sequences) showed high signal intensity in the central pons and bilateral cerebellum. With a diagnosis of ODS with poorly controlled diabetes, he was treated with insulin, metformin, and supportive measures following which his symptoms subsided gradually.
A rapid correction of hyponatremia is considered the most common cause of ODS. Variations in plasma glucose levels, a rare cause of ODS, can cause an abrupt osmolality change causing pontine and extrapontine myelinolysis. Prevention of rapid correction of hyponatremia and rapid changes in plasma osmolality in vulnerable patients is the mainstay of treatment.
Clinical features, imaging studies, and monitoring of serum osmolality, serum glucose, and electrolytes aid in diagnosis and favorable outcomes for the patient.
高渗高血糖状态导致的渗透性脱髓鞘综合征(ODS)较为罕见,可因髓鞘溶解而出现多种神经学表现。
一名44岁的糖尿病男性患者,主诉在过去1.5个月内突然出现、进行性加重的双下肢无力及言语不清。小脑检查显示双侧指鼻试验受损、轮替运动障碍、跟膝胫试验受损及串联步态障碍。脑部MRI(T2加权像及液体衰减反转恢复序列)显示脑桥中部及双侧小脑高信号。诊断为ODS合并糖尿病控制不佳,给予胰岛素、二甲双胍及支持治疗,随后症状逐渐缓解。
低钠血症的快速纠正被认为是ODS最常见的原因。血浆葡萄糖水平的变化是ODS的罕见原因,可导致渗透压突然改变,引起脑桥和脑桥外髓鞘溶解。预防易患患者低钠血症的快速纠正及血浆渗透压的快速变化是治疗的关键。
临床特征、影像学检查以及血清渗透压、血糖和电解质的监测有助于诊断并为患者带来良好预后。