Dilated Cardiomyopathy in Kugelberg-Welander Disease: Coexisting Sleep Disordered Breathing and Its Treatment with Continuous Positive Airway Pressure
2004; The Japanese Society of Internal Medicine; Volume: 43; Issue: 10 Linguagem: Inglês
10.2169/internalmedicine.43.951
ISSN1349-7235
AutoresFumihiko Yasuma, Satoshi Kuru, Masaaki Konagaya,
Tópico(s)Amyotrophic Lateral Sclerosis Research
ResumoA 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.
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