Restless legs syndrome in spinocerebellar ataxia types 1, 2, and 3
Restless legs syndrome in spinocerebellar ataxia types 1, 2, and 3
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DOI：
10.1007/s004150170206
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发表时间：
2001-04-01
影响因子：
6
通讯作者：
Klockgether, T
中科院分区：
文献类型：
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作者：
Abele, M;Bürk, K;Klockgether, T
为了确定脊髓小脑性共济失调（SCA）患者中不宁腿综合征（RLS）的患病率和决定因素，我们研究了58例SCA1、SCA2和SCA3分子诊断患者。通过标准化问卷收集RLS症状的数据，当患者符合国际研究小组最近定义的综合征的四个最低标准时，诊断为RLS。此外，我们还研究了RLS与年龄、共济失调发病年龄、CAG重复序列长度、神经传导和诱发电位数据的关系。SCA患者的RLS发生率显著高于对照组（28% vs. 10%）。SCA中RLS发作时的年龄为49.0 ± 10.9岁。RLS与非RLS SCA患者之间的神经传导或诱发电位无显著差异。发生RLS的可能性随年龄增加而增加，但与CAG重复长度或共济失调发病年龄无关。这些数据提供的证据表明，SCAI，SCA2和SCA3患者本身比未受影响的个体更容易发生RLS。发生RLS的可能性主要与CAG重复序列突变发挥作用的时间有关，而与CAG重复序列长度或共济失调发作的年龄无关。
To identify the prevalence and determinants of restless legs syndrome (RLS) in spinocerebellar ataxia (SCA) we studied 58 patients with a molecular diagnosis of SCA1, SCA2 and SCA3. Data on the symptoms of RLS were collected by a standardized questionnaire, and RLS was diagnosed when patients met the four minimal criteria of the syndrome as recently defined by an international study group. In addition, we studied the relationship between RLS and age, age at ataxia onset, CAG repeat length, and nerve conduction and evoked potentials data. RLS was significantly more frequent in SCA patients than in controls (28% vs. 10%). Age at RLS onset in SCA was 49.0 +/- 10.9 years. There were no significant differences in nerve conduction or evoked potentials between RLS and non-RLS SCA patients. The probability of developing RLS increased with age but not with CAG repeat length or higher age of ataxia onset. The data provide evidence that patients with SCAI, SCA2 and SCA3 are per se more susceptible to RLS than non-affected individuals. The probability of developing RLS is related principally to the period over which the CAG repeat mutation exerts its effect and not to CAG repeat length or age of ataxia onset.