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Léa Dormegny,1,2 Reana Velizarova,1 Carmen M Schroder,1,3 Ulker Kilic-Huck,1,3 Henri Comtet,1,3 Hélène Dollfus,4 Patrice Bourgin,1,3 Elisabeth Ruppert1,3 1Centre des Troubles du Sommeil - CIRCSom, Hôpital Civil, University of Strasbourg, Strasbourg, 67091, France; 2Department of Ophthalmology, Hôpital Civil, University of Strasbourg, Strasbourg, 67091, France; 3Institute for Cellular and Integrative Neurosciences, CNRS - UPR 3212, Strasbourg, 67000, France; 4Centre des Affections Rares en Génétique Ophtalmologique (CARGO), University of Strasbourg, Strasbourg, 67091, FranceCorrespondence: Elisabeth RuppertCentre des Troubles du Sommeil - CIRCSom - Hôpital Civil, 1, place de l’Hôpital, BP 426, Cedex, Strasbourg, 67091, FranceTel +33 3 88 11 64 30Fax +33 3 88 11 51 53Email elisabeth.ruppert@chru-strasbourg.frObjective/Background: Bardet–Biedl syndrome (BBS) is a rare but well-recognized ciliopathy with high genetic and phenotypic heterogeneity. Cardinal features include obesity, diabetes and high blood pressure (HBP), which are often associated with sleep-disordered breathing. Also, the high prevalence of blindness due to retinal dystrophy could affect circadian sleep–wake rhythms. We characterized in this cohort of adult BBS patients sleep-disordered breathing, sleep quality, daytime sleepiness and chronotype.Patients and Methods: Thirty-two patients with genetically confirmed BBS were included in this observational single center study. Overnight respiratory polygraphy was performed for sleep apnea syndrome (SAS) in 30 patients. Quality of sleep, daytime sleepiness, fatigue and chronotype were assessed in 25 patients using Pittsburgh sleep quality index (PSQI), 14-day sleep diary (SD), Epworth sleepiness scale (ESS), Pichot fatigue scale (PFS) and Horne and Ostberg morningness-eveningness questionnaire (MEQ).Results: Patients’ mean age was 32± 11 years and mean BMI 32.6± 7.7 kg/m2. Eleven (35%) patients had HBP and 7 (22%) diabetes. Moderate to severe sleep apnea syndrome (SAS) was present in 5 (17%) and was not associated with altered sleep, daytime sleepiness or fatigue. Most of the patients (63%) evaluated their sleep as of good quality (PSQI ≤ 5). Median scores of sleep quality, daytime sleepiness and fatigue were normal (PSQI of 3.0 [2.0– 6.0], ESS of 9.0 [6.0– 13.0] and PFS of 8.0 [3.0– 13.0], respectively). Predominant chronotypes according to MEQ were either “intermediate” (57%) or “moderate morning” (29%). None had a free running sleep–wake cycle. 14-day SD revealed overall few awakenings at night and low daytime napping.Conclusions: Given the cardiovascular risk factors, systematic screening for SAS should be considered in BBS patients, regardless of sleep and daytime vigilance complaints. None of these highly visually impaired patients had a circadian sleep–wake rhythm disorder. Further objective assessments are needed to better characterize sleep and circadian rhythms in BBS patients.Keywords: Bardet–Biedl syndrome, ciliopathy, sleep disordered breathing, sleep apnea syndrome, chronotype, circadian rhythm sleep wake disorder

Elisabeth Ruppert1–31Sleep Disorders Center - CIRCSom, Department of Neurology, Hôpital Civil, 67091 Strasbourg, France; 2Faculty of Medicine, University of Strasbourg, 67000 Strasbourg, France; 3Institute for Cellular and Integrative Neurosciences, CNRS - UPR 3212, University of Strasbourg, 67000 Strasbourg, FranceAbstract: 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.Keywords: RLS variant, RLS equivalent, restless upper limb, restlessness of upper extremities, Willis-Ekbom disease, periodic arm movements

A mathematical model is used to calculate the flux density vector B produced by the current circulating in a resistive single-phase superconducting current limiter (RSCL). The mathematical calculations and results are discussed.

The helicoidal method is used to calculate the flux density produced by the current in single-phase superconductor electrical current limiter (RSCL) coils. Three different coils made with copper wire are used to calculate electromagnetic properties and test results are discussed.