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一名酒精中毒患者出现韦尼克-科尔萨科夫综合征并发亚急性脚气病性神经病。

Wernicke-Korsakoff syndrome complicated by subacute beriberi neuropathy in an alcoholic patient.

作者信息

Di Marco Salvatore, Pilati Laura, Brighina Filippo, Fierro Brigida, Cosentino Giuseppe

机构信息

Department of Experimental Biomedicine and Clinical Neurosciences (BioNeC), University of Palermo, Italy.

Department of Experimental Biomedicine and Clinical Neurosciences (BioNeC), University of Palermo, Italy.

出版信息

Clin Neurol Neurosurg. 2018 Jan;164:1-4. doi: 10.1016/j.clineuro.2017.11.006. Epub 2017 Nov 8.

Abstract

Thiamine (vitamin B1) deficiency is a common condition in alcohol abusers, which can lead to damage of both the peripheral and the central nervous systems. Here we describe the case of an alcoholic patient who presented with acute onset of ataxia, severe weakness of the four limbs, and hypoesthesia and dysesthesia of the distal portion of the upper and lower extremities. The clinical picture also included mental confusion and amnesia. A diagnosis of Wernicke-Korsakoff syndrome was made based on clinical symptoms and brain RMI findings. Electromyography and electroneurography revealed signs of subacute axonal sensory-motor polyneuropathy that were compatible with a rare acute presentation of beriberi. Patient immediately received parenteral thiamine administration, which resulted in rapid clinical amelioration of ataxia and confusion and also in a significant improvement of motor and sensory deficits. The association between Wernicke-Korsakoff syndrome and acute axonal polyneuropathy is a very rare condition that could make less recognizable the clinical picture of a thiamine deficiency. However, the diagnosis of thiamine deficiency should be suspected in every alcoholic patient presenting with acute onset symptoms of central and/or peripheral nervous system involvement. This because the immediate replacement treatment can be life-saving and reverse the clinical symptoms.

摘要

硫胺素(维生素B1)缺乏是酗酒者的常见病症,可导致周围神经系统和中枢神经系统受损。在此,我们描述一例酒精性患者的病例,该患者出现急性共济失调、四肢严重无力以及上肢和下肢远端感觉减退和感觉异常。临床表现还包括精神错乱和失忆。根据临床症状和脑部磁共振成像(MRI)结果诊断为韦尼克 - 科萨科夫综合征。肌电图和神经电图显示亚急性轴索性感觉运动性多发性神经病的体征,这与一种罕见的急性脚气病表现相符。患者立即接受了肠胃外硫胺素给药,这使得共济失调和精神错乱迅速得到临床改善,运动和感觉功能障碍也有显著改善。韦尼克 - 科萨科夫综合征与急性轴索性多发性神经病之间的关联是一种非常罕见的情况,这可能会使硫胺素缺乏的临床症状不那么容易识别。然而,对于每一位出现中枢和/或周围神经系统受累急性发作症状的酒精性患者,都应怀疑硫胺素缺乏的诊断。这是因为立即进行替代治疗可能会挽救生命并逆转临床症状。

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