S.P.42 Effects of a physical exercise programme in adults with myotonic dystrophy type 1 – A one-year follow-up study

2012 ◽  
Vol 22 (9-10) ◽  
pp. 895-896 ◽  
Author(s):  
M. Kierkegaard ◽  
L. Widén Holmqvist
Keyword(s):  
Physical Exercise ◽  
Myotonic Dystrophy ◽  
Exercise Programme ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
Follow Up Study ◽  
One Year

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

scholarly journals Age‐related cognitive decline in myotonic dystrophy type 1: An 11‐year longitudinal follow‐up study

2019 ◽  
Vol 14 (1) ◽  
pp. 121-134 ◽  
Author(s):  
Garazi Labayru ◽  
Jone Aliri ◽  
Miren Zulaica ◽  
Adolfo López de Munain ◽  
Andone Sistiaga
Keyword(s):  
Cognitive Decline ◽  
Myotonic Dystrophy ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
Follow Up Study ◽  
Age Related ◽  
Age Related Cognitive Decline

Cognition in myotonic dystrophy type 1: a 5-year follow-up study

2016 ◽  
Vol 23 (9) ◽  
pp. 1471-1476 ◽  
Author(s):  
S. Winblad ◽  
L. Samuelsson ◽  
C. Lindberg ◽  
G. Meola
Keyword(s):  
Myotonic Dystrophy ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
Follow Up Study

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.

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