D.P.2.03 Clinical follow up in a large cohort of patients affected by myotonic dystrophy type 1

2008 ◽  
Vol 18 (9-10) ◽  
pp. 743-744
Author(s):  
C. Harling ◽  
M. Guglieri ◽  
G. Bell ◽  
J. Bourke ◽  
M. McCallum ◽  
...  
Keyword(s):  
Myotonic Dystrophy ◽  
Large Cohort ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type

Clinical guide for the diagnosis and follow-up of myotonic dystrophy type 1, MD1 or Steinert's disease

2019 ◽  
Vol 153 (2) ◽  
pp. 82.e1-82.e17
Author(s):  
Gerardo Gutiérrez Gutiérrez ◽  
Jordi Díaz-Manera ◽  
Míriam Almendrote ◽  
Sharona Azriel ◽  
José Eulalio Bárcena ◽  
...  
Keyword(s):  
Myotonic Dystrophy ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
Steinert's Disease ◽  
Steinert’S Disease

scholarly journals Mexiletine in myotonic dystrophy type-1

Neurology ◽  
2020 ◽  
pp. 10.1212/WNL.0000000000011002
Author(s):  
Chad Heatwole ◽  
Elizabeth Luebbe ◽  
Spencer Rosero ◽  
Katy Eichinger ◽  
William Martens ◽  
...  
Keyword(s):  
Myotonic Dystrophy ◽  
Cardiac Conduction ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
Walk Distance ◽  
Hand Grip ◽  
Minute Walk Distance ◽  
Minute Walk

ObjectiveTo assess mexiletine's long-term safety and effect on 6 minute walk distance in a well-defined cohort of myotonic dystrophy type-1 (DM1) patients.MethodsWe performed a randomized, double-blind, placebo-controlled trial of mexiletine (150 mg 3 times daily) to evaluate its efficacy and safety in a homogenous cohort of adult ambulatory DM1 patients. The primary outcome was change in 6-minute walk distance at 6 months. Secondary outcomes included changes in hand grip myotonia, strength, swallowing, forced vital capacity, lean muscle mass, Myotonic Dystrophy Health Index scores, and 24-hour Holter and EKG results at 3 and 6 months.ResultsForty-two participants were randomized and 40 completed the 6-month follow-up (n = 20 in both groups). No significant effects of mexiletine were observed on 6-minute walk distance, but hand grip myotonia was improved with mexiletine treatment. There were no differences between the mexiletine and placebo groups with respect to the frequency or type of adverse events. Changes in PR, QRS, and QTc intervals were similar in mexiletine and placebo-treated participants.ConclusionsThere was no benefit of mexiletine on six-minute walk distance at 6 months. Although mexiletine had a sustained positive effect on objectively measured hand grip myotonia, this was not seen in measures reflecting participants' perceptions of their myotonia. No effects of mexiletine on cardiac conduction parameters were seen over the 6-month follow-up period.Classification of evidenceThis study provides Class I evidence that for ambulatory patients with DM1 mexiletine does not significantly change six-minute walk distance at 6 months.

scholarly journals Cardiac Conduction Disorders as Markers of Cardiac Events in Myotonic Dystrophy Type 1

2020 ◽  
Vol 9 (17) ◽  
Author(s):  
Hideki Itoh ◽  
Takashi Hisamatsu ◽  
Takuhisa Tamura ◽  
Kazuhiko Segawa ◽  
Toshiaki Takahashi ◽  
...  
Keyword(s):  
Sudden Death ◽  
Myotonic Dystrophy ◽  
Cardiac Conduction ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
Cardiac Events ◽  
Conduction Disorders ◽  
Qrs Duration

Background Myotonic dystrophy type 1 involves cardiac conduction disorders. Cardiac conduction disease can cause fatal arrhythmias or sudden death in patients with myotonic dystrophy type 1. Methods and Results This study enrolled 506 patients with myotonic dystrophy type 1 (aged ≥15 years; >50 cytosine‐thymine‐guanine repeats) and was treated in 9 Japanese hospitals for neuromuscular diseases from January 2006 to August 2016. We investigated genetic and clinical backgrounds including health care, activities of daily living, dietary intake, cardiac involvement, and respiratory involvement during follow‐up. The cause of death or the occurrence of composite cardiac events (ie, ventricular arrhythmias, advanced atrioventricular blocks, and device implantations) were evaluated as significant outcomes. During a median follow‐up period of 87 months (Q1–Q3, 37–138 months), 71 patients expired. In the univariate analysis, pacemaker implantations (hazard ratio [HR], 4.35; 95% CI, 1.22–15.50) were associated with sudden death. In contrast, PQ interval ≥240 ms, QRS duration ≥120 ms, nutrition, or respiratory failure were not associated with the incidence of sudden death. The multivariable analysis revealed that a PQ interval ≥240 ms (HR, 2.79; 95% CI, 1.9–7.19, P <0.05) or QRS duration ≥120 ms (HR, 9.41; 95% CI, 2.62–33.77, P < 0.01) were independent factors associated with a higher occurrence of cardiac events than those observed with a PQ interval <240 ms or QRS duration <120 ms; these cardiac conduction parameters were not related to sudden death. Conclusions Cardiac conduction disorders are independent markers associated with cardiac events. Further investigation on the prediction of occurrence of sudden death is warranted.

Retrospective study on PET–SPECT imaging in a large cohort of myotonic dystrophy type 1 patients

2010 ◽  
Vol 31 (6) ◽  
pp. 757-763 ◽  
Author(s):  
Vincenzo Romeo ◽  
E. Pegoraro ◽  
F. Squarzanti ◽  
G. Sorarù ◽  
C. Ferrati ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Myotonic Dystrophy ◽  
Large Cohort ◽  
Spect Imaging ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type

A 9-year follow-up study of quantitative muscle strength changes in myotonic dystrophy type 1

2018 ◽  
Vol 265 (7) ◽  
pp. 1698-1705 ◽  
Author(s):  
Cynthia Gagnon ◽  
Émilie Petitclerc ◽  
Marie Kierkegaard ◽  
Jean Mathieu ◽  
Élise Duchesne ◽  
...  
Keyword(s):  
Muscle Strength ◽  
Myotonic Dystrophy ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
Follow Up Study

Does Bachmann's bundle pacing prevent atrial fibrillation in myotonic dystrophy type 1 patients? A 12 months follow-up study

EP Europace ◽  
2010 ◽  
Vol 12 (9) ◽  
pp. 1219-1223 ◽  
Author(s):  
G. Nigro ◽  
V. Russo ◽  
L. Politano ◽  
N. Della Cioppa ◽  
A. Rago ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Myotonic Dystrophy ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
Follow Up Study ◽  
Bachmann’S Bundle
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