Burgetova A, Vaneckova M, Seidl Z, Dolezal O
Department of Radiology, First Faculty of Medicine, Charles University in Prague; Czech Republic -
Neuroradiol J. 2008 Oct 1;21(4):521-6. doi: 10.1177/197140090802100409.
Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are disorders frequently associated with serum osmotic imbalance. The prognosis is very variable from complete regression of clinical symptomatology to signs of significant quadruparesis, a vegetative state and death. We report the case of a 25-year-old man with a diagnosis of osmotic demyelination syndrome. The patient was young healthy man with no history of chronic alcoholism or malnutrition. He underwent head trauma associated with consumption of alcohol, being diagnosed with cerebral commotion. Four days later he presented with generalised epileptic convulsions with unconsciousness. Laboratory findings showed significant hyponatremia and hypochlorinemia. Following the rapid correction of osmotic conditions of serum, spastic quadruparesis and coma developed. MRI of the brain showed finding of CPM and EPM, cortical laminar necrosis (CLN) and coagulative necrosis in the putamina. Our case is suggestive in the rare MRI appearance of myelinolysis in addition to CLN and coagulative necrosis in the basal ganglia following the rapid correction of serum osmolarity. We suggest that this finding is prognostically very unfavourable. In the reported patient clinically initial neurological deficit progressed to a vegetative state within one month.
中央桥脑髓鞘溶解症(CPM)和脑桥外髓鞘溶解症(EPM)是常与血清渗透压失衡相关的疾病。其预后差异很大,从临床症状完全消退到出现明显的四肢瘫痪、植物状态和死亡等体征。我们报告一例诊断为渗透性脱髓鞘综合征的25岁男性病例。该患者为年轻健康男性,无慢性酒精中毒或营养不良病史。他因饮酒后头部外伤,被诊断为脑震荡。四天后,他出现全身性癫痫惊厥并昏迷。实验室检查结果显示严重低钠血症和低氯血症。在血清渗透压状况迅速纠正后,出现了痉挛性四肢瘫痪和昏迷。脑部MRI显示有CPM和EPM、皮质层状坏死(CLN)以及壳核的凝固性坏死。我们的病例提示,在血清渗透压迅速纠正后,除了基底节区的CLN和凝固性坏死外,髓鞘溶解症在MRI上有罕见表现。我们认为这一发现预后非常不利。在所报告的患者中,临床最初的神经功能缺损在一个月内进展为植物状态。