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Cerebral Abnormalities in Myotonic Dystrophy-Reply

Cerebral Abnormalities in Myotonic Dystrophy-Reply Abstract In reply 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, References 1. Chang L, Anderson T, Migneco O, et al. Cerebral abnormalities in myotonic dystrophy . Arch Neurol . 1993;50:917-923.Crossref 2. Culebras A, Feldman RG, Merk FB. Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy . J Neurol Sci . 1973;19: 319-329.Crossref 3. Guberman A, Stuss D. The syndrome of bilateral paramedian thalamic infarction . Neurology . 1983;33:540-546.Crossref 4. Park YD, Radtke RA. Hypersomnolence in myotonic dystrophy . Neurology . 1992;42( (suppl 3) ):352. 5. Bliwise DL. Sleep apnea, dementia and Alzheimer disease: a mini-review . Bull Clin Neurosci . 1989;54:123-126. 6. Daly JA, Giombetti RJ, Miller BL. Impaired cerebral perfusion in chronic respiratory disorder: effect of treatment . Bull Clin Neurosci . 1987;54:137-143. 7. Issa F, Sullivan C. Reversal of central sleep apnea using nasal CPAP . Chest . 1986;90:165-171.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Cerebral Abnormalities in Myotonic Dystrophy-Reply

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References (9)

Publisher
American Medical Association
Copyright
Copyright © 1994 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1994.00540210020008
Publisher site
See Article on Publisher Site

Abstract

Abstract In reply 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, References 1. Chang L, Anderson T, Migneco O, et al. Cerebral abnormalities in myotonic dystrophy . Arch Neurol . 1993;50:917-923.Crossref 2. Culebras A, Feldman RG, Merk FB. Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy . J Neurol Sci . 1973;19: 319-329.Crossref 3. Guberman A, Stuss D. The syndrome of bilateral paramedian thalamic infarction . Neurology . 1983;33:540-546.Crossref 4. Park YD, Radtke RA. Hypersomnolence in myotonic dystrophy . Neurology . 1992;42( (suppl 3) ):352. 5. Bliwise DL. Sleep apnea, dementia and Alzheimer disease: a mini-review . Bull Clin Neurosci . 1989;54:123-126. 6. Daly JA, Giombetti RJ, Miller BL. Impaired cerebral perfusion in chronic respiratory disorder: effect of treatment . Bull Clin Neurosci . 1987;54:137-143. 7. Issa F, Sullivan C. Reversal of central sleep apnea using nasal CPAP . Chest . 1986;90:165-171.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Sep 1, 1994

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