Riva M, Brioschi A M, Ferrante E, Marazzi R
Dipartimento di Scienze Neurologiche, Ospedale Niguarda Ca' Granda di Milano.
Ann Ital Med Int. 1996 Apr-Jun;11(2):144-6.
Central pontine myelinolysis (CPM), a rare condition first observed by Adams et al. in 1959 in a group of malnourished chronic alcoholic subjects, has subsequently been seen in patients treated with thiazide diuretics, patients hyperhydrated postoperatively, and in other clinical situations. it is characterized by quadriplegia and pseudobulbar palsy which sometimes evolves into a locked-in syndrome. The rapid correction of severe hyponatremia (> 12 mmol/L/24 h) seems to be the causal factor, with consequent osmotic edema in the richly vascularized white matter of the pons as the proposed pathogenetic mechanism. We describe the case of a chronic psychotic man with nutritional disorders and inappropriate water intake who came to our attention for a clinical picture of CPM. Neuroradiological findings and postmortem studies revealed a slow-growing cerebellar astrocytoma in addition to the typical features of CPM. We discuss the hypothesis that damage to the nervous pathways and centers involved in water and electrolyte regulation could be the causal factor of CPM pathogenesis in this case.
中央桥脑髓鞘溶解症（CPM）是一种罕见病症，1959年由亚当斯等人首次在一组营养不良的慢性酒精中毒患者中观察到，随后在接受噻嗪类利尿剂治疗的患者、术后过度补液的患者以及其他临床情况下也有发现。其特征为四肢瘫痪和假性球麻痹，有时会发展为闭锁综合征。严重低钠血症的快速纠正（>12 mmol/L/24小时）似乎是致病因素，推测的发病机制是脑桥血管丰富的白质中随之出现的渗透性水肿。我们描述了一例患有营养障碍和不适当饮水的慢性精神病男性患者，其因中央桥脑髓鞘溶解症的临床表现而引起我们的关注。神经放射学检查结果和尸检研究显示，除了中央桥脑髓鞘溶解症的典型特征外，还存在一个生长缓慢的小脑星形细胞瘤。我们讨论了这样一种假说，即在这种情况下，参与水和电解质调节的神经通路和中枢受损可能是中央桥脑髓鞘溶解症发病机制的致病因素。
