HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Figures Only Full Text Full Text (PDF) Data Supplement Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Vihola, A. Articles by Udd, B. Search for Related Content PubMed PubMed Citation Articles by Vihola, A. Articles by Udd, B. Related Collections Muscle disease Ion channel gene defects Trinucleotide repeat diseases

Histopathological differences of myotonic dystrophy type 1 (DM1) and PROMM/DM2

From the Department of Neurology (Dr. Udd and A. Vihola), Vaasa Central Hospital, Vaasa, Finland; Myology Institute (Drs. Bassez, Hogrel, Laforêt, and Eymard, A. Rouche), Salpêtrière Hospital, Paris, France; Department of Neurology (Dr. Meola), University of Milan, San Donato Hospital, San Donato Milanese, Milan, Italy; Human Cancer Genetics Program (Drs. Zhang and Krahe), Department of Molecular Virology, Immunology and Medical Genetics, Comprehensive Cancer Center, Ohio State University, Columbus, OH; Department of Pathology (Dr. Haapasalo), Tampere University Hospital, Tampere, Finland; Department of Pathology (A. Paetau), University of Helsinki, Finland; Department of Physiology and General Biochemistry (Dr. Mancinelli), University of Milan, Italy; Department of Neuropathology (Dr. Maisonobe), Salpêtrière Hospital, Paris, France; Department of Pathology and Neuropathology (Dr. Pellissier), La Timone Hospital, Marseilles, France; and Section of Cancer Genetics, Department of Molecular Genetics (Dr. Krahe), University of Texas M.D. Anderson Cancer Center, Houston, TX.

Address correspondence and reprint requests to Dr. Bjarne Udd, Department of Neurology, Vaasa Central Hospital, FIN-65130 Vaasa, Finland; e-mail: Bjarne.Udd{at}vshp.fi

Muscle biopsy findings in DM2 have been reported to be similar to those in DM1. The authors used myosin heavy chain immunohistochemistry and enzyme histochemistry for fiber type differentiation on muscle biopsies. Their results show that DM2 patients display a subpopulation of type 2 nuclear clump and other very small fibers and, hence, preferential type 2 fiber atrophy in contrast to type 1 fiber atrophy in DM1 patients.

This article has been cited by other articles:

				J. M. Margolis, B. G. Schoser, M. L. Moseley, J. W. Day, and L. P.W. Ranum DM2 intronic expansions: evidence for CCUG accumulation without flanking sequence or effects on ZNF9 mRNA processing or protein expression Hum. Mol. Genet., June 1, 2006; 15(11): 1808 - 1815. [Abstract] [Full Text] [PDF]

				I. L. Ber, M. Martinez, D. Campion, A. Laquerriere, C. Betard, G. Bassez, C. Girard, P. Saugier-Veber, G. Raux, N. Sergeant, et al. A non-DM1, non-DM2 multisystem myotonic disorder with frontotemporal dementia: phenotype and suggestive mapping of the DM3 locus to chromosome 15q21-24 Brain, September 1, 2004; 127(9): 1979 - 1992. [Abstract] [Full Text] [PDF]

				B. G. H. Schoser, W. Kress, M. C. Walter, B. Halliger-Keller, H. Lochmuller, and K. Ricker Homozygosity for CCTG mutation in myotonic dystrophy type 2 Brain, August 1, 2004; 127(8): 1868 - 1877. [Abstract] [Full Text] [PDF]