Abstract
Study of 55 cases of progressive muscular dystrophies (34 Duchenne, 12 Duchenne with residual dystrophin and 9 Becker patients) comparing age, age at the initial symptoms, duration of symptoms, levels of serum enzymes, degree of disability measured by the Vignos and Archibald scale, and the type and amount of dystrophin found in the muscle biopsies by immunofluorescence. Statistical analysis showed a tendency of the symptoms and progression of disease to be related with the low quantity of dystrophin in the biopsies. There was no difference in the parameter analysed between the Duchenne patients with and without residual dystrophin, as well as the Duchenne with residual dystrophin and Becker patients. There was an inverse relation with the amount of dystrophin and the endomysial connective tissue and fatty infiltration, and a direct relation with hypertrophic fibers and atrophic angulated fibers in the NADH-tetrazolium reductase. In the comments a discussion is made about the difficulties in differentiate Duchenne and Becker dystrophies, the cases with residual dystrophin and the importance of the correct diagnosis.
References
Mar 1, 1978·Muscle & Nerve·W G BradleyP R Fawcett
Jul 1, 1977·Archives of Neurology·S P RingelS C Schold
Dec 24, 1987·Nature·E P HoffmanL M Kunkel
Apr 1, 1989·Neurology·R MedoriR H Waterston
Aug 1, 1989·Neurology·E P HoffmanJ B Harris
Aug 1, 1989·Annals of Neurology·D H Gutmann, K H Fischbeck
Sep 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·K ArahataH Sugita
Oct 1, 1989·Neurology·S M GospeK H Fischbeck
Oct 1, 1989·Archives of Disease in Childhood·A M NormanL V Nicholson
Apr 1, 1989·Neurology·L L BaumbachC T Caskey
Mar 1, 1987·Human Genetics·K A HartM Bobrow
Aug 12, 1988·Cell·E BonillaL P Rowland
Jul 1, 1988·Neuron·E P HoffmanL M Kunkel
Jun 1, 1988·Arquivos de neuro-psiquiatria·L C Werneck, J G Lima
Apr 22, 1988·Cell·M KoenigL M Kunkel
May 26, 1988·The New England Journal of Medicine·E P HoffmanL Specht
Jun 1, 1988·Brain : a Journal of Neurology·L P Rowland
Dec 24, 1987·Cell·E P HoffmanL M Kunkel
Jul 31, 1987·Cell·M KoenigL M Kunkel
Oct 16, 1986·Nature·A P MonacoL M Kunkel
May 1, 1969·Archives of Neurology·R F Shaw, F E Dreifuss
Jul 1, 1969·Neurology·O N MarkandD D Daly
Feb 1, 1983·Muscle & Nerve·M H BrookeM A Province
Citations
Dec 1, 1992·Arquivos de neuro-psiquiatria·A S OliveiraE Bonilla
Dec 1, 1992·Arquivos de neuro-psiquiatria·L C WerneckR H Scola
Sep 25, 2001·American Journal of Medical Genetics·L C WerneckM C Werneck
Apr 11, 2007·Arquivos de neuro-psiquiatria·Aline Andrade FreundLineu Cesar Werneck