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September 1994

Cerebral Abnormalities in Myotonic Dystrophy

Arch Neurol. 1994;51(9):850. doi:10.1001/archneur.1994.00540210020007

The article by Chang et al, entitled "Cerebral Abnormalities in Myotonic Dystrophy," ratifies the observations made by others that patients with myotonic dystrophy (MD) suffer cognitive deficits. The authors present data supporting the notion that patients with maternally inherited MD manifest earlier and more severe cognitive defects than do patients with paternally inherited MD. Their single-photon emission computed tomography data show a variety of cerebral blood flow reductions that are likely secondary to the cerebral functional deficit, while the magnetic resonance imaging studies are inconclusive, perhaps because the studies were not longitudinal.

The authors attempt to explain the cognitive changes in MD through linkage with the sleep apnea syndrome. They fail to consider that in most patients with MD, sleep apneas are relatively mild and, although contributory, they are likely not an important cause of cognitive change in these patients. Sleep apnea syndrome in MD may not even be a