Myotonia Drugs to Help prevent Muscle Contractures and Seizures

Here is some information about a drug that may help with muscle contractures and seizures. My son recently had several episodes where he would lose balance and sink to the ground, not really tripping but losing strength and we suspected that he may be having myotonia contractures. What this is simply is that muscles in his legs are seizing up and not moving so that the body can not remain upright. We are looking at some of these drugs to see if they may be helpful. The one that has been mentioned most is mexiletine. There are a couple others but Dr. Moxley at the University of Rochester has studies this drug more than most. His experience with Myotonic Dystrophy is extensive.

The study did not measure outcomes. That is they studies it and saw it reduced ontraction time in patients muscles. They did not look to see if it made a difference in their everyday lives such as walking or opening cans. So no real evidence it helps outcomes, but it seems to work from the researchers standpoint.

The study here was presented at one of the IDMC meetings. Will be giving this drug a try if the doctors agree it may help and not affect any of his cardiac issues, like level 1 heart block.

 

Mexiletine is an effective antimyotonia treatment in myotonic dystrophy type 1(LOE Classification)

This article has been cited by other articles in PMC.

Abstract

Objective:

To determine if mexiletine is safe and effective in reducing myotonia in myotonic dystrophy type 1 (DM1).

Background:

Myotonia is an early, prominent symptom in DM1 and contributes to decreased dexterity, gait instability, difficulty with speech/swallowing, and muscle pain. A few preliminary trials have suggested that the antiarrhythmic drug mexiletine is useful, symptomatic treatment for nondystrophic myotonic disorders and DM1.

Methods:

We performed 2 randomized, double-blind, placebo-controlled crossover trials, each involving 20 ambulatory DM1 participants with grip or percussion myotonia on examination. The initial trial compared 150 mg of mexiletine 3 times daily to placebo, and the second trial compared 200 mg of mexiletine 3 times daily to placebo. Treatment periods were 7 weeks in duration separated by a 4- to 8-week washout period. The primary measure of myotonia was time for isometric grip force to relax from 90% to 5% of peak force after a 3-second maximum grip contraction. EKG measurements and adverse events were monitored in both trials.

Results:

There was a significant reduction in grip relaxation time with both 150 and 200 mg dosages of mexiletine. Treatment with mexiletine at either dosage was not associated with any serious adverse events, or with prolongation of the PR or QTc intervals or of QRS duration. Mild adverse events were observed with both placebo and mexiletine treatment.

Conclusions:

Mexiletine at dosages of 150 and 200 mg 3 times daily is effective, safe, and well-tolerated over 7 weeks as an antimyotonia treatment in DM1.

Continue reading

Print Friendly

Staying upright with a Neuromuscular disease like Myotonic Dystrophy

Staying Upright with a Neuromuscular Disease

From: Multiple Sclerosis Society Quest newsletter, written by Christan Medvescek

Some people have nightmares about falling off cliffs. Brad Williams has nightmares about falling — period.

“Whenever I’m walking, falling down is always the major thought on my mind,” says Williams, 39, of Alexandria, Va. “It has to be on my mind a lot for me to be dreaming about it.”

Williams has Miyoshi distal myopathy, a slowly progressive form of muscular dystrophy that primarily affects the extremities. He hosts an MDA Internet chat under the nickname “dysf,” and notes that other chat participants also have reported falling nightmares. “It’s like a flying-falling dream, except it’s just about falling down.”

Continue reading

Print Friendly

Falls and stumbles in Myotonic Dystrophy DM Patients

Falls and stumbles are TEN times more likely in patients with myotonic Dystrophy that in a normal healthy population. One reason for the fall or stumble would be for a sustained muscle contraction that can not be counterbalanced by other muscles. Fall prevention programs should be implemented for patients with DM.

 

J Neurol Neurosurg Psychiatry. 2006 March; 77(3): 393–396.

Published online 2005 September 30. doi:  10.1136/jnnp.2005.066258
PMCID: PMC2077718

Falls and stumbles in myotonic dystrophy

This article has been cited by other articles in PMC.

Abstract

Objective

To investigate falls and risk factors in patients with myotonic dystrophy type 1 (DM1) compared with healthy volunteers.

Methods

13 sequential patients with DM1 from different kindreds were compared with 12 healthy volunteers. All subjects were evaluated using the Rivermead Mobility Index, Performance Oriented Mobility Assessment, and modified Activities Specific Balance Confidence scale. Measures of lower limb muscle strength, gait speed, and 7‐day ambulatory activity monitoring were recorded. Subjects returned a weekly card detailing stumbles and falls.

Results

11 of 13 patients (mean age 46.5 years, seven female) had 127 stumbles and 34 falls over the 13 weeks, compared with 10 of 12 healthy subjects (34.4 years, seven female) who had 26 stumbles and three falls. Patients were less active than healthy subjects but had more falls and stumbles per 5000 right steps taken (mean (SD) events, 0.21 (0.29) v 0.02 (0.02), p = 0.007). Patients who fell (n = 6) had on average a lower Rivermead Mobility score, slower self selected gait speed, and higher depression scores than those who did not.

Conclusions

DM1 patients stumble or fall about 10 times more often than healthy volunteers. Routine inquiry about falls and stumbles is justified. A study of multidisciplinary intervention to reduce the risk of falls seems warranted.

Continue reading

Print Friendly