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儿童期起病的不宁腿综合征:22个家庭的临床和遗传特征

Childhood-onset restless legs syndrome: clinical and genetic features of 22 families.

作者信息

Muhle Hiltrud, Neumann Anja, Lohmann-Hedrich Katja, Lohnau Thora, Lu Yang, Winkler Susen, Waltz Stephan, Fischenbeck Anke, Kramer Patricia L, Klein Christine, Stephani Ulrich

机构信息

Department of Neuropediatrics, University of Kiel, Kiel, Germany.

出版信息

Mov Disord. 2008 Jun 15;23(8):1113-21; quiz 1203. doi: 10.1002/mds.22016.

Abstract

Restless legs syndrome (RLS) is a sensory-motor disorder that is underdiagnosed in children and often misclassified as attention deficit hyperactivity disorder. Five different gene loci (RLS1-5) and three susceptibility loci have been identified in adult-onset RLS. We included 23 children with RLS (age at onset < or =14 years) from 22 families. In 14 families, we performed linkage and genotype analyses. Of the 23 RLS patients, only seven (30.4%) were admitted for a suspected diagnosis of RLS. Five patients had a retrospectively established onset at an age as early as 1 year. The most frequent complaint in patients were sleep problems (21 of 23; 91%) resulting in fatigue in 14 children (60.9%). Twelve of the 19 tested cases (63.2%) exhibited an index of periodic limb movements in sleep greater than 5. Dopaminergic therapy was successful in 12 of 14 treated patients (85.7%). Family history for RLS was positive in 20 of 23 children (87.0%) and compatible with an autosomal dominant inheritance pattern. Linkage analysis excluded all five loci in two families. A trend for an association at two of the three reported susceptibility regions was observed. RLS symptoms can occur in early childhood. The positive family history suggests a genetic cause in most families with at least one additional RLS gene locus.

摘要

不宁腿综合征(RLS)是一种感觉运动障碍,在儿童中诊断不足,常被误诊为注意力缺陷多动障碍。在成人起病的RLS中已鉴定出五个不同的基因位点(RLS1 - 5)和三个易感位点。我们纳入了来自22个家庭的23名RLS儿童(发病年龄≤14岁)。在14个家庭中,我们进行了连锁和基因型分析。在这23名RLS患者中,只有7名(30.4%)因疑似RLS诊断而入院。5名患者回顾性确定的发病年龄早至1岁。患者最常见的主诉是睡眠问题(23例中的21例;91%),14名儿童(60.9%)出现疲劳。19例检测病例中有12例(63.2%)睡眠期周期性肢体运动指数大于5。14例接受治疗的患者中有12例(85.7%)多巴胺能治疗成功。23名儿童中有20名(87.0%)RLS家族史呈阳性,符合常染色体显性遗传模式。连锁分析在两个家庭中排除了所有五个位点。在三个报道的易感区域中的两个区域观察到关联趋势。RLS症状可在儿童早期出现。阳性家族史表明,在大多数至少有一个额外RLS基因位点的家庭中存在遗传原因。

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