Case Report:Pure word deafness associated with extrapontine myelinolysis  

作　　者：Ren-jing ZHU Zhi-su LV Chun-lei SHAN Ming-wei XU Ben-yan LUO 

机构地区：[1]Department of Neurology, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310029, China [2]Department of Neurology, Zhongshan Hospital, Xiamen University, Xiamen 361004, China [3]Department of Ultrasonography, Children's Hospital, School of Medicine, Zhejiang University, Hangzhou 310029, China [4]Laboratory for Higher Brain Function, Institute of Psychology, Chinese Academy of Sciences, Beijing 100101, China [5]Department of Rehabilitation Medicine, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China

出　　处：《Journal of Zhejiang University-Science B(Biomedicine & Biotechnology)》2010年第11期842-847,共6页浙江大学学报（英文版）B辑（生物医学与生物技术）

基　　金：Project supported by the Science Research Foundation of the Ministry of Health of China(No.WKJ2010-2-009);the Science and Technology Department of Zhejiang Province(No.2007C33007);the Key Project of Education Department of Zhejiang Province(No.Z2008 05185);the Natural Science Foundation of Zhejiang Province(No. Y2080132),China

摘　　要：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.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.

关 键 词：Pure word deafness Auditory agnosia Osmotic demyelination syndrome Extrapontine myelinolysis Cortical deafness 

分 类 号：R74[医药卫生—神经病学与精神病学]