title: Dilated cardiomyopathy in Kugelberg-Welander disease: coexisting sleep disordered breathing and its treatment with continuous positive airway pressure.
abstract: A rare complication of possible secondary dilated cardiomyopathy to Kugelberg-Welander disease was described in a 53-year-old patient with this inherent motor neuron disease, whom we diagnosed after a genetic analysis of the defective survival motor neuron gene. An association of sleep disordered breathing of Cheyne-Stokes respiration was diagnosed, which was virtually eliminated with continuous positive airway pressure via nasal mask. Considering the paucity of therapeutic options in most degenerative neuromuscular disorders, ameliorations in not only sleep quality but also cardiac function with continuous positive airway pressure have clinical implications.
authors.affiliation: Department of Internal Medicine, Suzuka National Hospital, 3-2-1 Kasado, Suzuka, Mie 513-8501.
authors.forename: Fumihiko
authors.initials: F
authors.lastname: Yasuma
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kuru
authors.forename: Masaaki
authors.initials: M
authors.lastname: Konagaya
mesh_terms: D001784:Blood Gas Analysis; D002311:Cardiomyopathy, Dilated / Q000150:complications*; D045422:Continuous Positive Airway Pressure; D004232:Diuretics / Q000627:therapeutic use; D004562:Electrocardiography; D004576:Electromyography; D006334:Heart Function Tests; D006801:Humans; D008297:Male; D008875:Middle Aged; D009132:Muscles / Q000473:pathology* / Q000503:physiopathology; D017286:Polysomnography; D012891:Sleep Apnea Syndromes / Q000150:complications* / Q000628:therapy; D014897:Spinal Muscular Atrophies of Childhood / Q000150:complications* / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D004232:Diuretics; D014665:Vasodilator Agents