Department of Neurology, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.
J Zhejiang Univ Sci B. 2010 Nov;11(11):842-7. doi: 10.1631/jzus.B1000200.
Extrapontine myelinolysis and pure word deafness are very uncommon disorders. Here, we report a case of a 19-year-old woman who suffered from osmotic demyelination syndrome with coincidence of typical pure word deafness. As a consequence of rapid correction of hyponatremia, the patient demonstrated an initial onset of cortical deafness, and then progressed to generalized auditory agnosia, which eventually developed into confined verbal auditory agnosia (pure word deafness). Bilateral extrapontine myelinolysis was confirmed using brain magnetic resonance imaging. This case suggests that verbal and nonverbal stimuli may involve separate thalamocortical pathways.
桥脑外髓鞘溶解症和纯词聋非常罕见。在此，我们报告一例 19 岁女性，患有渗透性脱髓鞘综合征，同时合并典型的纯词聋。由于低钠血症的快速纠正，患者表现出皮质性耳聋的初始发作，随后进展为全面性听觉失认，最终发展为局限的言语听觉失认（纯词聋）。磁共振成像证实存在双侧桥脑外髓鞘溶解症。该病例提示言语和非言语刺激可能涉及不同的丘脑-皮质通路。
