Lilje Christian G, Heinen Florian, Laubenberger Jörg, Krug Isolde, Brandis Matthias
Department of Pediatrics; University of Freiburg;, Freiburg, Germany.
Pediatr Neurol. 2002 Aug;27(2):132-5. doi: 10.1016/s0887-8994(02)00411-3.
Central pontine myelinolysis is a rare neurologic disorder defined by symmetric demyelination of the central base of the pons. Until recently its outcome was considered invariably poor if not fatal. We report a 15-year-old female patient with severe anorexia nervosa who acutely developed a locked-in syndrome. Magnetic resonance imaging revealed a central pontine lesion. There was no serum sodium abnormality. The corticospinal and corticonuclear tracts were intact, as assessed by serial neurophysiologic studies. Finally, the patient recovered completely both clinically and radiologically. This article discusses these observations in the light of recent reports on similar benign outcomes of central pontine myelinolysis. Although electrolyte imbalances could be overestimated and the clinical significance of the radiologic lesion is uncertain, the functional assessment of the corticospinal and corticonuclear fibers might have prognostic value.
桥脑中央髓鞘溶解症是一种罕见的神经系统疾病,其特征为脑桥基底部对称性脱髓鞘。直到最近,人们还认为其预后即使不致命也必然很差。我们报告了一名15岁患有严重神经性厌食症的女性患者,她急性发展为闭锁综合征。磁共振成像显示脑桥中央有病变。血清钠无异常。通过系列神经生理学研究评估,皮质脊髓束和皮质核束完整。最终,患者在临床和影像学上均完全康复。本文根据近期关于桥脑中央髓鞘溶解症类似良性预后的报道对这些观察结果进行了讨论。尽管电解质失衡可能被高估,且影像学病变的临床意义尚不确定,但皮质脊髓纤维和皮质核纤维的功能评估可能具有预后价值。