Bonkowsky Joshua L, Filloux Francis M
Department of Pediatrics, University of Utah School of Medicine, Salt Lake City 84132, USA.
J Child Neurol. 2003 Feb;18(2):144-7. doi: 10.1177/08830738030180021201.
Central pontine and extrapontine myelinolysis are characterized by symmetric demyelination following rapid shifts in serum osmolality, although in extrapontine myelinolysis, demyelination is confined to the supratentorial compartment. We present a case of extrapontine myelinolysis in a 17-year-old female that occurred in the setting of diabetic ketoacidosis, cerebral edema, mannitol therapy, and meningitis. The rate of correction of this patient's glucose and electrolyte levels was within well-accepted limits. Extrapontine myelinolysis is rare in pediatric patients: there are only 12 reports of extrapontine myelinolysis in children under age 20 years and no pediatric cases of extrapontine myelinolysis or central pontine myelinolysis associated with diabetic ketoacidosis. We review the published cases of extrapontine myelinolysis and examine the underlying etiologies and electrolyte disturbances that characterize these cases. This case expands the list of conditions in which extrapontine myelinolysis occurs to include pediatric patients with complicated diabetic ketoacidosis, emphasizing the importance of sudden osmolar shifts in the genesis of this disorder.
脑桥中央髓鞘溶解症和脑桥外髓鞘溶解症的特征是血清渗透压快速变化后出现对称性脱髓鞘，不过在脑桥外髓鞘溶解症中，脱髓鞘局限于幕上腔隙。我们报告一例17岁女性发生的脑桥外髓鞘溶解症，该病例发生于糖尿病酮症酸中毒、脑水肿、甘露醇治疗及脑膜炎的背景下。该患者血糖和电解质水平的纠正速度在公认的范围内。脑桥外髓鞘溶解症在儿科患者中罕见：20岁以下儿童仅有12例脑桥外髓鞘溶解症报告，且无与糖尿病酮症酸中毒相关的儿科脑桥外髓鞘溶解症或脑桥中央髓鞘溶解症病例。我们回顾已发表的脑桥外髓鞘溶解症病例，并研究这些病例的潜在病因及电解质紊乱情况。该病例扩大了发生脑桥外髓鞘溶解症的疾病范围，包括患有复杂糖尿病酮症酸中毒的儿科患者，强调了突然的渗透压变化在该疾病发生中的重要性。
