Ruppert Elisabeth
Sleep Disorders Center - CIRCSom, Department of Neurology, Hôpital Civil, 67091 Strasbourg, France.
Faculty of Medicine, University of Strasbourg, 67000 Strasbourg, France.
Neuropsychiatr Dis Treat. 2019 Jul 1;15:1737-1750. doi: 10.2147/NDT.S161583. eCollection 2019.
This literature review focuses on restless arms syndrome (RAS), an upper limb variant of restless legs syndrome (RLS). RLS, also known as Willis-Ekbom disease, is a frequently occurring neurological disorder characterized by an irresistible urge to move the lower limbs often accompanied by unpleasant sensations in the legs, worsened at rest and in the evening, improved by movement. Extension of leg restlessness to the upper limbs is frequently reported in typical patients who had RLS only in the legs and usually occurs later in the course of RLS, restlessness remaining most invalidating in the lower limbs. In RAS, the arms are predominantly affected with little or no involvement of the legs. Cases of restless shoulders syndrome or periodic arm movements without arm restlessness were not considered. A total of 9 articles with 10 cases were included and analyzed for the adherence to the five essential diagnostic criteria of the International RLS Study Group (IRLSSG) classification, as well as for the additional supportive features. All of the reported cases were classified as having definite RAS. The clinical history and disease evolution of two previously reported patients were completed and updated. Overall, the clinical picture of RAS does not differ from that of RLS, except for the symptoms localization on the upper limbs. Underlying mechanisms of the spread of RLS to upper limb restlessness and of RAS remain unknown. Whether RAS is a phenotypic variant of RLS or a separate entity needs further investigations. RAS likely remains underdiagnosed and according to IRLSSG diagnostic criteria RAS should be considered when RLS-like symptoms are present in one or both arms, especially when they have a circadian pattern and are improved by movement and dopaminergic therapy. Clinicians should be aware of this rare condition, especially as treatment using dopaminergic agonists proves to be very efficient.
本综述聚焦于不安臂综合征(RAS),它是不安腿综合征(RLS)的上肢变体。RLS,也称为 Willis-Ekbom 病,是一种常见的神经系统疾病,其特征为下肢有不可抑制的运动冲动,常伴有腿部不适感觉,在休息时和晚上加重,运动后改善。仅腿部患有 RLS 的典型患者经常报告腿部不安延伸至上肢,且通常发生在 RLS 病程后期,不安感在下肢最为严重。在 RAS 中,主要是手臂受到影响,而腿部很少或没有受累。不安肩综合征或无手臂不安的周期性手臂运动的病例未被考虑。共纳入 9 篇文章中的 10 例病例,并分析其对国际不安腿综合征研究组(IRLSSG)分类的五项基本诊断标准的符合情况以及其他支持性特征。所有报告的病例均被分类为确诊的 RAS。完成并更新了两名先前报告患者的临床病史和疾病演变情况。总体而言,RAS 的临床表现与 RLS 并无不同,只是症状定位于上肢。RLS 向上肢不安蔓延以及 RAS 的潜在机制尚不清楚。RAS 是 RLS 的表型变体还是一个独立的实体,需要进一步研究。RAS 可能仍未得到充分诊断,根据 IRLSSG 诊断标准,当一只或两只手臂出现类似 RLS 的症状时,尤其是当这些症状具有昼夜节律模式且通过运动和多巴胺能治疗得到改善时,应考虑 RAS。临床医生应了解这种罕见疾病,特别是因为使用多巴胺能激动剂治疗被证明非常有效。