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Dilated cardiomyopathy in Kugelberg-Welander disease: coexisting sleep disordered breathing and its treatment with continuous positive airway pressure.

作者信息

Yasuma Fumihiko, Kuru Satoshi, Konagaya Masaaki

机构信息

Department of Internal Medicine, Suzuka National Hospital, 3-2-1 Kasado, Suzuka, Mie 513-8501.

出版信息

Intern Med. 2004 Oct;43(10):951-4. doi: 10.2169/internalmedicine.43.951.

DOI:10.2169/internalmedicine.43.951
PMID:15575246
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.

摘要

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J Neurol Neurosurg Psychiatry. 2006 May;77(5):671-3. doi: 10.1136/jnnp.2005.078600.