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1型强直性肌营养不良症中的眼球运动障碍

Eye movement disorders in myotonic dystrophy type 1.

作者信息

Osanai Ryuichi, Kinoshita Masanobu, Hirose Kazuhiko

机构信息

Department of Otolaryngology, Kashiwa Kousei General Hospital, 3-8-20 Akebono, Kashiwa, Chiba, Japan.

出版信息

Acta Otolaryngol Suppl. 2007 Dec(559):78-84. doi: 10.1080/03655230701597192.

DOI:10.1080/03655230701597192
PMID:18340575
Abstract

CONCLUSIONS

No definite sign was found of central oculomotor system disorders being independent of saccadic slowing because (1) diminished maximum slow phase velocity of the optokinetic nystagmus (OKNspv) was closely related to saccadic slowing (p<0.01, r=0.59), (2) maximum frequency of optokinetic nystagmus (OKNfq) was normal, (3) visual suppression (VS) change was mild, and (4) the diminished maximum slow phase velocity of the caloric nystagmus (CNspv) seen in some patients is explained by both peripheral and central vestibular involvement. These findings support the extraocular muscle hypothesis.

OBJECTIVE

To assess whether eye movement disorders seen in patients with myotonic dystrophy type 1 (DM1) are caused by central oculomotor system involvement or extraocular muscle damage.

PATIENTS AND METHODS

Oculomotor functions and their correlation with (CTG)n length were studied in 29 DM1 patients and 12 age-matched controls.

RESULTS

Values for saccadic velocity (p<0.005), maximum OKNspv (p<0.005), and maximum CNspv (p<0.01) in the patient group were markedly lower than in the control group. VS of caloric nystagmus in the patient group was slightly lower than that in the controls. No significant difference was found between the two groups in the maximum OKNfq. Patients with greater (CTG)n lengths had lower saccadic velocities (p<0.01, r=0.71).

摘要

结论

未发现中枢动眼系统障碍独立于扫视减慢的明确迹象,原因如下:(1)视动性眼震(OKN)最大慢相速度(OKNspv)降低与扫视减慢密切相关(p<0.01,r=0.59);(2)视动性眼震(OKN)最大频率(OKNfq)正常;(3)视觉抑制(VS)变化轻微;(4)部分患者出现的冷热性眼震(CN)最大慢相速度(CNspv)降低可由外周和中枢前庭受累共同解释。这些发现支持眼外肌假说。

目的

评估1型强直性肌营养不良(DM1)患者出现的眼动障碍是由中枢动眼系统受累还是眼外肌损伤所致。

患者与方法

对29例DM1患者和12例年龄匹配的对照者的动眼功能及其与(CTG)n长度的相关性进行了研究。

结果

患者组的扫视速度(p<

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