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      Neuropsychiatric Disease and Treatment (submit here)

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      Restless arms syndrome: prevalence, impact, and management strategies

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          Abstract

          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.

          Most cited references55

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          Restless legs syndrome: diagnostic criteria, special considerations, and epidemiology. A report from the restless legs syndrome diagnosis and epidemiology workshop at the National Institutes of Health.

          Restless legs syndrome is a common yet frequently undiagnosed sensorimotor disorder. In 1995, the International Restless Legs Syndrome Study Group developed standardized criteria for the diagnosis of restless legs syndrome. Since that time, additional scientific scrutiny and clinical experience have led to a better understanding of the condition. Modification of the criteria is now necessary to better reflect that increased body of knowledge, as well as to clarify slight confusion with the wording of the original criteria. The restless legs syndrome diagnostic criteria and epidemiology workshop at the National Institutes of Health. Members of the International Restless Legs Syndrome Study Group and authorities on epidemiology and the design of questionnaires and scales. To modify the current criteria for the diagnosis of restless legs syndrome, to develop new criteria for the diagnosis of restless legs syndrome in the cognitively impaired elderly and in children, to create standardized criteria for the identification of augmentation, and to establish consistent questions for use in epidemiology studies. The essential diagnostic criteria for restless legs syndrome were developed and approved by workshop participants and the executive committee of the International Restless Legs Syndrome Study Group. Criteria were also developed and approved for the additional aforementioned groups.
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            Restless legs syndrome associated with major diseases: A systematic review and new concept.

            Recent publications on both the genetics and environmental factors of restless legs syndrome (RLS) defined as a clinical disorder suggest that overlapping genetic risk factors may play a role in primary (idiopathic) and secondary (symptomatic) RLS. Following a systematic literature search of RLS associated with comorbidities, we identified an increased prevalence of RLS only in iron deficiency and kidney disease. In cardiovascular disease, arterial hypertension, diabetes, migraine, and Parkinson disease, the methodology of studies was poor, but an association might be possible. There is insufficient evidence for conditions such as anemia (without iron deficiency), chronic obstructive pulmonary disease, multiple sclerosis, headache, stroke, narcolepsy, and ataxias. Based on possible gene-microenvironmental interaction, the classifications primary and secondary RLS may suggest an inappropriate causal relation. We recognize that in some conditions, treatment of the underlying disease should be achieved as far as possible to reduce or eliminate RLS symptoms. RLS might be seen as a continuous spectrum with a major genetic contribution at one end and a major environmental or comorbid disease contribution at the other.
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              Altered brain iron homeostasis and dopaminergic function in Restless Legs Syndrome (Willis-Ekbom Disease).

              Restless legs syndrome (RLS), also known as Willis-Ekbom Disease (WED), is a sensorimotor disorder for which the exact pathophysiology remains unclear. Brain iron insufficiency and altered dopaminergic function appear to play important roles in the etiology of the disorder. This concept is based partly on extensive research studies using cerebrospinal fluid (CSF), autopsy material, and brain imaging indicating reduced regional brain iron and on the clinical efficacy of dopamine receptor agonists for alleviating RLS symptoms. Finding causal relations, linking low brain iron to altered dopaminergic function in RLS, has required however the use of animal models. These models have provided insights into how alterations in brain iron homeostasis and dopaminergic system may be involved in RLS. The results of animal models of RLS and biochemical, postmortem, and imaging studies in patients with the disease suggest that disruptions in brain iron trafficking lead to disturbances in striatal dopamine neurotransmission for at least some patients with RLS. This review examines the data supporting an iron deficiency-dopamine metabolic theory of RLS by relating the results from animal model investigations of the influence of brain iron deficiency on dopaminergic systems to data from clinical studies in patients with RLS.
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                Author and article information

                Journal
                Neuropsychiatr Dis Treat
                Neuropsychiatr Dis Treat
                NDT
                neurodist
                Neuropsychiatric Disease and Treatment
                Dove
                1176-6328
                1178-2021
                01 July 2019
                2019
                : 15
                : 1737-1750
                Affiliations
                [1 ] Sleep Disorders Center - CIRCSom, Department of Neurology, Hôpital Civil , 67091 Strasbourg, France
                [2 ] Faculty of Medicine, University of Strasbourg , 67000 Strasbourg, France
                [3 ] Institute for Cellular and Integrative Neurosciences, CNRS - UPR 3212, University of Strasbourg , 67000 Strasbourg, France
                Author notes
                Correspondence: Elisabeth Ruppert Centre des Troubles du Sommeil – CIRCSom, Hôpital Civil 1, Place de l’Hôpital, BP 426, Cedex, 67091Strasbourg, FranceTel +33 38 811 6430Fax +33 38 811 5153Email elisabeth.ruppert@ 123456chru-strasbourg.fr
                Article
                161583
                10.2147/NDT.S161583
                6612954
                8ddd0c59-1d00-4a71-9205-f86ec90436d5
                © 2019 Ruppert.

                This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms ( https://www.dovepress.com/terms.php).

                History
                : 10 January 2019
                : 28 May 2019
                Page count
                Figures: 1, Tables: 2, References: 60, Pages: 14
                Categories
                Review

                Neurology
                rls variant,rls equivalent,restless upper limb,restlessness of upper extremities,willis-ekbom disease,periodic arm movements

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