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硫胺素缺乏时前庭和眼球运动异常的谱系

The Spectrum of Vestibular and Ocular Motor Abnormalities in Thiamine Deficiency.

作者信息

Kattah Jorge C

机构信息

Illinois Neurologic Institute, University of Illinois College of Medicine, Peoria, IL, USA.

出版信息

Curr Neurol Neurosci Rep. 2017 May;17(5):40. doi: 10.1007/s11910-017-0747-9.

Abstract

PURPOSE

The first aim of this review is to summarize recent ocular motor signs in pre-encephalopathy patients with nutritional deficiency at risk of thiamine deficiency. Timely recognition of thiamine depletion in these patients, who may have a normal brain MRI, could lead to appropriate management and prevention of Wernicke's encephalopathy (WE) with full recovery. The second aim is to incorporate recent diagnostic testing on the revised WE diagnostic criteria and the identification of patients who may show slow, partial, or no response to treatment.

RECENT FINDINGS

Selective vulnerability of periventricular gray neurons in thiamine deficiency is well known. Involvement of the vestibular and abducens nuclei may precede encephalopathy. Studies have shown mild ophthalmoparesis and bilateral symmetric vestibular loss in thiamine deficiency. Moreover, quantitative data has shown decreased horizontal vestibulo-ocular reflex (VOR) gain and nystagmus, with a favorable response to timely treatment. Ophthalmoparesis, horizontal nystagmus, and decreased gain of the horizontal VOR, sparing the vertical VOR, may be present in the early pre-encephalopathy stage of thiamine deficiency. Rapid response to a loading dose of parenteral thiamine might be seen in some cases and normalization, albeit slower in others. In contrast, analogous to the Korsakoff's syndrome, ocular motor and vestibular abnormalities may show only partial improvement. Future studies in larger populations at risk are needed to confirm the results of these preliminary observations.

摘要

目的

本综述的首要目的是总结近期在有硫胺素缺乏风险的营养缺乏性脑病前期患者中出现的眼动体征。及时识别这些脑部磁共振成像(MRI)可能正常的患者体内的硫胺素耗竭情况,有助于进行恰当的管理,并预防韦尼克脑病(WE),实现完全康复。第二个目的是纳入近期关于修订后的WE诊断标准的诊断测试,以及识别那些对治疗反应缓慢、部分反应或无反应的患者。

最新发现

硫胺素缺乏时脑室周围灰质神经元的选择性易损性是众所周知的。前庭核和展神经核受累可能先于脑病出现。研究表明,硫胺素缺乏时会出现轻度眼球运动麻痹和双侧对称性前庭功能丧失。此外,定量数据显示水平前庭眼反射(VOR)增益和眼球震颤降低,及时治疗后反应良好。在硫胺素缺乏的脑病前期早期,可能会出现眼球运动麻痹、水平眼球震颤以及水平VOR增益降低,而垂直VOR不受影响。在某些情况下,对静脉注射硫胺素负荷剂量可能会有快速反应,尽管在其他情况下恢复正常的速度较慢。相比之下,类似于科萨科夫综合征,眼球运动和前庭异常可能仅部分改善。需要在更多有风险的人群中开展进一步研究以证实这些初步观察结果。

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