Video head impulse test findings in patients with peripheral myelin protein 22 related neuropathies

IF 3.7 3区医学 Q1 CLINICAL NEUROLOGY

Clinical Neurophysiology Pub Date : 2025-04-19 DOI:10.1016/j.clinph.2025.03.046

Zeljka Calic , Bogdan Bjelica , Stojan Peric , Milorad Vujnic , Ivo Bozovic , Vidosava Rakocevic-Stojanovic , Andrew Bradshaw , Miriam S Welgampola

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Abstract

Objective

Vestibular impairment may be present in and contribute to imbalance in patients with hereditary neuropathies. We examined the vestibulo-ocular reflex (VOR) characteristics in peripheral myelin protein 22 neuropathies using the video head-impulse test (vHIT).

Methods

23 patients with Charcot-Marie-Tooth disease 1A (CMT1A) and 17 with hereditary neuropathy with liability to pressure palsies (HNPP) were recruited. Three-dimensional vHIT was performed. VOR-gain and latency, refixation-saccade prevalence and first-saccade amplitude, onset-latency, peak-velocity and duration were examined and compared against age-matched controls.

Results

In CMT1A and HNPP gait imbalance was reported in 78.3 % and 58.8 % of patients, resulting in recurrent falls in 65.2 % and 23.5 %. Reduced VOR-gain affecting the posterior-canals (PCs) was found in 47.8 % of CMT1A and 11.7 % of HNPP patients. First saccade amplitude and peak-velocities higher in horizontal-canal (HC) and PC in the CMT1A group compared to controls (p < 0.05). In HNPP, first saccades were larger in HC and anterior-canal (AC) planes; saccade peak-velocity was higher in AC and PC planes compared to controls (p < 0.05). In CMT1A, VOR-gain impairment was associated with higher Charcot-Marie-Tooth Examination Score, longer disease duration, and higher total Overall Neuropathy Limitation Scale score (p < 0.05) and VOR-gain was lower for PC in patients with a history of recurrent falls (p < 0.05). VOR-latency was significantly longer in HC and PCs in CMT1A compared to controls (p < 0.05).

Conclusions

VOR impairment and slowing of the VOR-latency is found in CMT1A but not the HNPP cohort. These findings may relate to demyelinating processes affecting the vestibular nerves and thus the VOR pathways. Significance: VHIT allows detection of VOR impairment which could be an additional contributor to imbalance and falls in patients with CMT1A.

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外周髓鞘蛋白22相关神经病患者的视频头脉冲试验结果

目的：遗传性神经病变患者可能存在前庭功能障碍，并可能导致神经失衡。我们使用视频头冲试验（vHIT）检测了外周髓鞘蛋白22神经病变的前庭眼反射（VOR）特征。方法选取1A型夏-玛丽-图斯病（CMT1A）患者23例，遗传性神经病变伴压迫性麻痹（HNPP）患者17例。进行三维vHIT。检查了vor增益和潜伏期、固定扫视率和首次扫视幅度、发病潜伏期、峰值速度和持续时间，并与年龄匹配的对照组进行了比较。结果CMT1A和HNPP步态失衡发生率分别为78.3%和58.8%，复发跌倒发生率分别为65.2%和23.5%。在47.8%的CMT1A和11.7%的HNPP患者中发现影响后管（PCs）的vor增益降低。与对照组相比，CMT1A组水平管（HC）和PC的第一次扫视幅度和峰值速度更高(p <；0.05)。在HNPP中，第一次扫视在HC和AC平面较大；与对照组相比，AC和PC平面的眼跳峰值速度更高(p <；0.05)。在CMT1A中，vor增益障碍与较高的Charcot-Marie-Tooth检查评分、较长的疾病持续时间和较高的总体神经病变限制量表总分相关(p <；有反复跌倒史的PC患者的vor增益较低(p <；0.05)。与对照组相比，CMT1A中HC和pc的vor潜伏期明显更长(p <；0.05)。结论在CMT1A组中发现了svor损伤和vor潜伏期减慢，而在HNPP组中没有。这些发现可能与影响前庭神经和VOR通路的脱髓鞘过程有关。意义：VHIT允许检测VOR损伤，这可能是CMT1A患者失衡和跌倒的另一个因素。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical Neurophysiology 医学-临床神经学

CiteScore

8.70

自引率

6.40%

发文量

932

审稿时长

59 days

期刊介绍： As of January 1999, The journal Electroencephalography and Clinical Neurophysiology, and its two sections Electromyography and Motor Control and Evoked Potentials have amalgamated to become this journal - Clinical Neurophysiology. Clinical Neurophysiology is the official journal of the International Federation of Clinical Neurophysiology, the Brazilian Society of Clinical Neurophysiology, the Czech Society of Clinical Neurophysiology, the Italian Clinical Neurophysiology Society and the International Society of Intraoperative Neurophysiology.The journal is dedicated to fostering research and disseminating information on all aspects of both normal and abnormal functioning of the nervous system. The key aim of the publication is to disseminate scholarly reports on the pathophysiology underlying diseases of the central and peripheral nervous system of human patients. Clinical trials that use neurophysiological measures to document change are encouraged, as are manuscripts reporting data on integrated neuroimaging of central nervous function including, but not limited to, functional MRI, MEG, EEG, PET and other neuroimaging modalities.