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Effects of 1-month withdrawal of ventilatory support in hypercapnic myotonic dystrophy type 1.

Fergal J O'DonoghueJean-Christian BorelYves DauvilliersPatrick LevyRenaud TamisierJean-Louis Pépin
Published in: Respirology (Carlton, Vic.) (2017)
DM1 patients derive little benefit in symptoms or quality of life from NIV. Nocturnal and diurnal ventilatory functions deteriorate slightly off NIV for 1 month, but this does not appear to be due to changes in HCVR or respiratory function. HCVR changes may be of primary CNS origin given stability on or off NIV.
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