Department of Anatomical, Histological, Forensic and Orthopedic Sciences, "Sapienza" University of Rome, Rome, 00185, Italy.
Department of Medicine, Surgery and Dentistry, University of Salerno, Baronissi, 84081, Italy.
BMC Neurol. 2024 Nov 5;24(1):428. doi: 10.1186/s12883-024-03934-3.
Osmotic Demyelination Syndrome (ODS) encompasses Central Pontine Myelinolysis and Extrapontine Myelinolysis, both of which are serious neurological conditions linked to the overly rapid correction of hyponatremia. Despite growing evidence, the exact etiology of ODS remains incompletely understood. The present paper describes two case studies, aiming to provide a comprehensive overview of the pathological findings and clinical outcomes associated with ODS.
Case #1. A 74-year-old woman was admitted to the emergency department following a head trauma caused by a loss of consciousness. Initial laboratory tests revealed severe hyponatremia (sodium level of 101 mmol/L) and hypokalemia (potassium level of 2.9 mmol/L). The patient underwent corrective therapy with saline and potassium chloride. Despite the correction of electrolyte imbalances, the patient developed a hyperintense lesion in the median portion of the pons on T2-fluid-attenuated inversion recovery (FLAIR) MRI sequence 14 days post-treatment, consistent with ODS. The patient's condition deteriorated, leading to irreversible coma and status epilepticus, culminating in death 32 days after admission. Case #2. An 81-year-old woman with a medical history of hypothyroidism, hypertension, major depression, and stage 3 chronic kidney disease presented with mild gait disturbances. Subsequent testing revealed severe hyponatremia (sodium level of 100 mmol/L). Following an initial clinical improvement due to sodium correction, the patient's condition worsened, with symptoms progressing to confusion, lethargy, and eventually, ODS. Dermatological manifestations, including blistering lesions and facial edema, appeared as the condition advanced. The patient succumbed to irreversible coma 47 days after admission.
ODS traditionally carried a poor prognosis, with high mortality rates and diagnoses often made postmortem. However, recent advances in understanding the pathophysiology, along with improvements in diagnostic techniques such as MRI and intensive care treatments, have led to earlier identification, treatment, and recognition of milder forms of the syndrome. Despite these advancements, ODS remains a critical condition with significant risks, particularly following the rapid correction of severe hyponatremia.
渗透性脱髓鞘综合征（ODS）包括脑桥中央髓鞘溶解症和脑桥外髓鞘溶解症，这两种都是与低钠血症过快纠正相关的严重神经系统疾病。尽管有越来越多的证据，但 ODS 的确切病因仍不完全清楚。本文描述了两个病例研究，旨在提供与 ODS 相关的病理发现和临床结果的全面概述。
病例 1. 一名 74 岁女性因意识丧失导致头部外伤后被收入急诊病房。初步实验室检查显示严重低钠血症（钠水平为 101mmol/L）和低钾血症（钾水平为 2.9mmol/L）。患者接受了生理盐水和氯化钾的纠正治疗。尽管电解质失衡得到了纠正，但患者在治疗后 14 天的 T2 液体衰减反转恢复（FLAIR）MRI 序列中出现了桥中部高信号病变，符合 ODS 的表现。患者的病情恶化，导致不可逆的昏迷和癫痫持续状态，最终在入院后 32 天死亡。病例 2. 一名 81 岁女性，有甲状腺功能减退症、高血压、重度抑郁症和 3 期慢性肾脏病病史，表现为轻度步态障碍。随后的检查显示严重低钠血症（钠水平为 100mmol/L）。在由于钠纠正而出现初始临床改善后，患者的病情恶化，出现意识混乱、昏睡，最终发展为 ODS。随着病情的进展，出现了包括水疱性病变和面部水肿在内的皮肤表现。患者在入院后 47 天死于不可逆的昏迷。
ODS 传统上预后较差，死亡率较高，且通常在死后诊断。然而，随着对病理生理学的理解的最新进展，以及 MRI 和重症监护治疗等诊断技术的改进，已经能够更早地识别、治疗和认识到该综合征的较轻形式。尽管取得了这些进展，但 ODS 仍然是一种具有重大风险的危急情况，尤其是在严重低钠血症过快纠正后。
