Myotonic Dystrophy Steinert disease







2011 - 2010 - 2009 - 2008


December, 2010
A Canadian study perfomed in 20 patients with DM1 and gastrointestinal symptoms revealed the presence of a small intestinal bacterial overgrowth. Ciprofloxacin was the most common antibiotic used for treatment, and 70% of patients reported a good response to the initial course of treatment. Although the causes of gastrointestinal symptoms in patients with DM1 are multifactorial, small intestinal bacterial overgrowth is an important diagnostic consideration that is easily diagnosed using glucose breath hydrogen testing and often shows a good response to treatment with common antibiotics.

November 26, 2010
A French study showed that the mean time for ECG changes was about 5 years in patients with DM1. The most frequent arrythmia was atrail fibrillation and was associated with a higfh risk of death. The study conclude that repetition of ECG every year is probably not useful.

October 14, 2010
A study conducted at the University of Baltimore in 70 patients with DM1 revealed that Paroxysmal atrial flutter or fibrillation, older age, and larger CTG expansions predict greater time-dependent PR and QRS interval prolongation and warrant particular attention in the arrhythmic evaluation of this high risk patient subset.

September 15, 2010 - IGF-1 in Myotonic dystrophy type 1
Trial of insulin like growth factor 1 (IGL-1) coupled to insulin -like growth factor binding protein 3 (IGF-BP3) in myotonic dystrophy type 1. A study performed at the University of Rochester and University of Texas revealed that treatment with IGF-1/IGF-BP3 was well tolerated by patients with DM1 but had no effect on muscle strength. For more details see: Heatwole et al, Arch. Neurol., September 15, 2010.

May 4, 2010 - Treatment of myotonia by mexiletine.
A study performed at the university of Rochester shows that mexiletine at dosages of 150 and 200 mg 3 times daily is effective, safe, and well-tolerated as an antimyotonia treatment in DM1. For more détails, see : Logigian et al., Neurology, 2010, May 4;74(18):1441-8.

January 15, 2010 - Anesthesia and myotonic dystrophy type 2
(Weingarten TN et al., Can J. Anaesth. 2010, Jan15) This study shows that patients with DM2 tolerated commonly used anesthetics without obvious complications and suggests that these medications may be used safely in patients with DM2.

January 13, 2010 - MBNL1 binds GC motifs embedded in pyrimidines to regulate alternative splicing
(Goers et al., Nucleic Acids Res. 2010 Jan 13) A recent study shows that the binding of MBNL1 to RNA YGCY motifs is sufficient to promote alternative splicing. This result may open new therapeutic avenues for DM.