Procainamide for dyspnea in myotonic dystrophy

Details

Serval ID
serval:BIB_611EE6013BB7
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Procainamide for dyspnea in myotonic dystrophy
Journal
American Review of Respiratory Disease
Author(s)
Fitting  J. W., Leuenberger  P.
ISSN
1073-449X
0003-0805 (Print)
Publication state
Published
Issued date
11/1989
Volume
140
Number
5
Pages
1442-5
Notes
Case Reports
Journal Article --- Old month value: Nov
Abstract
We report the case of a patient with myotonic dystrophy who developed tachypnea and severe dyspnea without respiratory failure. Myotonia of inspiratory muscles was diagnosed on the grounds of marked prolongation of transdiaphragmatic pressure (Pdi) decay during sniffs. In view of the recognized sensory role of inspiratory muscles in dyspnea, it was hypothesized that antimyotonic therapy might relieve dyspnea in this patient. Procainamide therapy induced a decrease in half relaxation time of Pdi during sniffs and yielded a striking clinical improvement with cessation of tachypnea and dyspnea. Later, this beneficial effect was maintained by tocainide after procainamide was stopped because of a lupus syndrome. We conclude that myotonia of respiratory muscles can cause severe dyspnea that can be improved by antimyotonic therapy.
Keywords
Diaphragm/physiopathology Dyspnea/*drug therapy/etiology Female Humans Middle Aged Muscle Relaxation Myotonic Dystrophy/*complications/physiopathology Procainamide/*therapeutic use Respiration Respiratory Function Tests *Respiratory Muscles Rest
Pubmed
Web of science
Create date
25/01/2008 9:43
Last modification date
20/08/2019 14:18
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