Imam Yahia Z, Saqqur Maher, Alhail Hassan, Deleu Dirk
Neurology Section, Department of Medicine, Hamad Medical Corporation, P.O. Box 3050, Doha, Qatar.
Case Rep Neurol Med. 2012;2012:327058. doi: 10.1155/2012/327058. Epub 2012 Dec 17.
Background. Extrapontine myelinolysis (EPM) has been well described in the presence of rapid correction of hyponatremia. It is seldom reported with adrenal insufficiency. We report a unique case where a patient developed EPM as a result of adrenal insufficiency where the brain MRI revealed symmetrical lesion in the basal ganglia with pallidal sparing. Case Report. A 30-year-old gentleman with panhypopituitarism developed adrenal crisis, hyponatremia, and hyponatremic encephalopathy. Seven days after the rapid correction of hyponatremia, he developed parkinsonism and neuropsychiatric symptoms. MRI showed extrapontine myelinolysis without central pontine myelinolysis. Conclusion. Extrapontine myelinolysis without central pontine myelinolysis is rare and should raise a concern of associated adrenal insufficiency in the right clinical setting. Rapid correction of hyponatremia particularly in steroid-deficient states should be avoided as it can predispose to extrapontine myelinolysis. Magnetic resonance imaging is very helpful in supporting the diagnosis of EPM.
背景。桥外髓鞘溶解症（EPM）在低钠血症快速纠正的情况下已有充分描述。肾上腺功能不全伴发此病的情况鲜有报道。我们报告了一例独特病例，一名患者因肾上腺功能不全而发生EPM，脑部磁共振成像（MRI）显示基底节区对称性病变，苍白球未受累。病例报告。一名患有全垂体功能减退症的30岁男性发生了肾上腺危象、低钠血症和低钠血性脑病。低钠血症快速纠正7天后，他出现了帕金森综合征和神经精神症状。MRI显示为桥外髓鞘溶解症，无桥脑中央髓鞘溶解症。结论。无桥脑中央髓鞘溶解症的桥外髓鞘溶解症很罕见，在合适的临床背景下应引起对相关肾上腺功能不全的关注。应避免低钠血症的快速纠正，尤其是在类固醇缺乏状态下，因为这可能易引发桥外髓鞘溶解症。磁共振成像对支持EPM的诊断非常有帮助。
