Abnormal Iris Vasculature in Myotonic Dystrophy

Abstract

• The microvasculature of the iris was studied in 35 patients with neuromuscular disease and 14 control subjects, using anterior segment fluorescein angiography. Myotonic muscular dystrophy, in which a variety of ocular changes have previously been reported, was found to be associated with both focal and generalized vascular abnormalities. Changes were seen in the fluorescein angiograms of all nine of the myotonic dystrophy patients in which the iris vessels could be seen. No evidence of a microcirculatory disorder was seen in patients with Duchenne's dystrophy, for which a vascular pathogenesis has been proposed. The angiograms of patients with limb-girdle dystrophy, facioscapulohumeral dystrophy, and Friedreich's ataxia were also normal.