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A newly developed technique to detect motor nerve abnormalities has been used to examine the rate of axonal conduction in the phasic component of the peroneal nerve in 10 patients with DM1. The technique was found to be well suited to detect a significant decrease in the rate of axonal conduction at the elbow of DM1 patients as compared with healthy controls, thus demonstrating that peripheral axons are indeed affected by DM1 [247]. This technique has the potential to detect early changes in motor nerve function that may be the cause of complaints of musculoskeletal abnormalities. It also may provide an opportunity to explore new treatments for DM1.

The balance of evidence available at present does not support short term use of corticosteroids for patients with DM1. First, the data from the study by Mudd et al [248] showed that dexamethasone treatment did not lead to a significant improvement in DM1-related symptoms or laboratory abnormalities. Further, in the short term (up to 12 weeks) studies of the effects of short term corticosteroid use on DM1, the data from the study by Bergamo et al.[10] showed decreased plasma creatine kinase and creatinine kinase myocardial band in treated versus untreated subjects. There was also a trend towards fewer subjects with abnormal screening for hematological abnormalities and the only suggestion of improvement of the DM1-related urinary tract symptoms. Longer term studies are therefore warranted in order to confirm the potential for growth in muscle volume and/or strength with the use of corticosteroids.

These preliminary data may provide the basis for a program of objective testing of the relationships between symptom presentation and CTG expansion size. Sympathomimetic drugs should be able to be documented in a large cohort of subjects for objective trends in CTG expansion size with effective drugs. Anti-depressants should be investigated to determine if they have any activity in DM1 in terms of symptom reduction or amelioration of insulin resistance. Future studies will need to document the effects of treatment on metabolic markers of insulin resistance. A series of trials in patients with the most severely ill/symptomatic patients in DM1 would lead to treatments that can be developed as potential new therapies. d2c66b5586