Cognitive loss in myotonic dystrophy remains to be explained at the biochemical level. Chang et alArticle measured specific biochemical changes in the brain using proton magnetic resonance spectroscopy and correlated these changes with the severity of the genetic defect as measured by the number of trinucleotide repeats. Patients with myotonic dystrophy had elevated brain levels of myoinositol, total creatine, and choline-containing compounds compared with findings in control subjects. These biochemical changes directly correlated with the increased number of CTG repeats from leukocytes. Thus, cognitive loss seen in some patients may be correlated with these important new findings.
This Month in The Archives of Neurology. Arch Neurol. 1998;55(3):285-286. doi:10.1001/archneur.55.3.285