We appreciate the thoughtful comments from Culebras regarding our article on cerebral abnormalities in myotonic dystrophy (MD).1 He suggests that the progressive cognitive deficits associated with the paternally inherited form of myotonic dystrophy (pMD) may relate to progressive damage in the dorsomedial thalamic nuclei.2 This is an interesting and original idea that certainly warrants careful study. With the emergence of powerful co-registration programs that allow superimposition of positron-emission tomography or single-photon emission computed tomography over magnetic resonance imaging, this hypothesis can now be systematically tested.The patients with pMD whom we studied did not appear to have a thalamic dementia syndrome3 as the one cognitive sphere that appeared relatively intact was memory.1 All of the patients with pMD were either cognitively normal or had subtle cognitive deficits, and one patient showed the progressive dementia found in the original study by Culebras et al. However,
Chang L, Anderson T, Boone K, Berman N, Mena I. Cerebral Abnormalities in Myotonic Dystrophy-Reply. Arch Neurol. 1994;51(9):850–851. doi:10.1001/archneur.1994.00540210020008
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