Video head impulse gain is impaired in myotonic dystrophy types 1 and 2

IF 4.5 2区医学 Q1 CLINICAL NEUROLOGY

European Journal of Neurology Pub Date : 2024-10-15 DOI:10.1111/ene.16513

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

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Abstract

Background and Purpose

This study was undertaken to examine vestibulo-ocular reflex (VOR) characteristics in myotonic dystrophy type 1 (DM1) and type 2 (DM2) using video head impulse testing (vHIT).

Methods

VOR gain, refixation saccade prevalence, first saccade amplitude, onset latency, peak velocity, and duration were compared in DM1, DM2, age-matched normal controls, and patients with peripheral and central vestibulopathies.

Results

Fifty percent of DM1 and 37.5% of DM2 patients demonstrated reduced VOR gain. Refixation saccade prevalence for horizontal canal (HC) and posterior canal (PC) was significantly higher in DM1 (101 ± 42%, 82 ± 47%) and DM2 (70 ± 45%, 61 ± 38%) compared to controls (40 ± 28% and 43 ± 33%, p < 0.05). The first saccade amplitudes and peak velocities were higher in HC and PC planes in DM1 and DM2 compared to controls (p < 0.05). HC slow phase eye velocity profiles in DM1 showed delayed peaks. The asymmetry ratio, which represents the percentage difference between the first and second halves of the slow phase eye velocity response, was therefore negative (−22.5 ± 17%, −2.3 ± 16%, and − 4.7 ± 8% in DM1, DM2, and controls). HC VOR gains were lower and gain asymmetry ratio was larger and negative in patients with DM1 with moderate to severe ptosis and a history of imbalance and falls compared to the remaining DM1 patients (p < 0.05). In peripheral vestibulopathies, saccade amplitude was larger, peak velocity was higher, and onset latency was shorter (p < 0.05) than in DM1. In central vestibulopathy (posterior circulation strokes), saccade peak velocity was higher, but amplitude and onset latency were not significantly different from DM1.

Conclusions

VOR impairment is common in DM1 and DM2. In DM1, refixation saccade characteristics are closer to central than peripheral vestibulopathies. Delayed peaks in the vHIT eye velocity profile observed in patients with DM1 may reflect extraocular muscle weakness. VOR impairment and VOR asymmetry in DM1 are associated with imbalance and falls.

Abstract Image

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肌张力营养不良症 1 型和 2 型的视频头脉冲增益受损。

背景和目的：本研究采用视频头脉冲测试（vHIT）方法，研究肌营养不良症1型（DM1）和2型（DM2）患者的前庭眼反射（VOR）特征：方法：比较DM1、DM2、年龄匹配的正常对照组以及外周性和中枢性前庭疾病患者的VOR增益、复位囊回发生率、首次囊回振幅、起始潜伏期、峰值速度和持续时间：50%的DM1和37.5%的DM2患者表现出VOR增益降低。与对照组（40±28%和43±33%，P）相比，DM1（101±42%和82±47%）和DM2（70±45%和61±38%）患者水平管（HC）和后管（PC）的复位囊回发生率明显更高：VOR损伤在DM1和DM2中很常见。在 DM1 中，复位囊回的特征更接近于中枢性前庭疾病，而非外周性前庭疾病。在DM1患者中观察到的vHIT眼速曲线延迟峰可能反映了眼外肌无力。DM1 患者的 VOR 损伤和 VOR 不对称与失衡和跌倒有关。

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

European Journal of Neurology 医学-临床神经学

CiteScore

9.70

自引率

2.00%

发文量

418

审稿时长

1 months

期刊介绍： The European Journal of Neurology is the official journal of the European Academy of Neurology and covers all areas of clinical and basic research in neurology, including pre-clinical research of immediate translational value for new potential treatments. Emphasis is placed on major diseases of large clinical and socio-economic importance (dementia, stroke, epilepsy, headache, multiple sclerosis, movement disorders, and infectious diseases).