نتایج جستجو برای: myotonic dystrophy

تعداد نتایج: 22886  

Journal: :Journal of neurology, neurosurgery, and psychiatry 1975
J P Ballantyne S Hansen

Single motor unit potentials recorded from surface electrodes over the extensor digitorum brevis muscle and evoked by stimulation of the anterior tibial nerve at the ankle were obtained by a computer subtraction method. Their latencies, durations, amplitudes, and areas were measured in control subjects and patients with Duchenne, limb-girdle, facioscapulohumeral, and myotonic muscular dystrophy...

2001
Fahri Akbas Piraye Serdaroglu Feza Deymeer Fikret Aysal Nihan Erginel-Unaltuna

EDITOR—Myotonic dystrophy (DM) is the most common form of inherited neuromuscular disease in adults and is characterised by progressive muscle wasting and myotonia. The mutation responsible for DM has been identified as the amplification of a polymorphic (CTG)n repeat in the 3' untranslated region of a gene encoding a serine/threonine kinase (DMPK). The DM trinucleotide repeat is highly polymor...

2018
Kévin Chapron Valère Plantevin Florentin Thullier Kevin Bouchard Elise Duchesne Sébastien Gaboury

Many people in the world are affected by muscle wasting, especially the population hits by myotonic dystrophy type 1 (DM1). Those people are usually given a program of multiple physical exercises to do. While DM1 and many other people have difficulties attending commercial centers to realize their program, a solution is to develop such a program completable at home. To this end, we developed a ...

Journal: :European heart journal 2014
Perry Elliott

Lund and colleagues report on the association between cardiac disease and myotonic dystrophy using data derived from Danish patient registries. The major finding that myotonic dystrophy is strongly associated with cardiac disease throughout life emphasizes the importance of continuous cardiac follow-up and the urgent need for prevention and treatment of cardiac complications in this disease. Th...

Journal: :Journal of medical genetics 1996
A M Cobo J J Poza A Blanco A López de Munain A Saénz M Azpitarte J Marchessi J F Martí Massó

DNA samples from 231 unselected patients with cataracts were studied to determine the frequency of the DM mutation in cataract patients. A previous epidemiological study established a high prevalence of DM in the population of Guipúzcoa (Basque Country, Spain), 26.5 cases/100,000. We have found two carriers (0.9%) of the DM mutation in patients who are not related to any previously known DM fam...

Journal: :Thorax 1994
A J Finnimore R V Jackson A Morton E Lynch

BACKGROUND Tiredness and daytime respiratory failure occur frequently in myotonic dystrophy. Sleep hypoxaemia was studied in 12 patients with myotonic dystrophy and correlations were sought with their daytime lung and respiratory muscle function. METHODS All patients underwent overnight sleep studies, clinical assessment, measurement of flow-volume loops and carbon monoxide transfer factor, a...

Journal: :Circulation. Cardiovascular genetics 2017
Caroline Chong-Nguyen Karim Wahbi Vincent Algalarrondo Henri Marc Bécane Hélène Radvanyi-Hoffman Pauline Arnaud Denis Furling Arnaud Lazarus Guillaume Bassez Anthony Béhin Abdallah Fayssoil Pascal Laforêt Tanya Stojkovic Bruno Eymard Denis Duboc

BACKGROUND In myotonic dystrophy type 1, the association between mutation size (CTG expansion) and the severity of cardiac involvement is controversial. METHODS AND RESULTS We selected 855 patients with myotonic dystrophy type 1 (women, 51%; median age, 37 years), with genetic testing performed at the moment of their initial cardiac evaluation, out of 1014 patients included in the Myotonic Dy...

Journal: :British Journal of Anaesthesia 1995

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