DYSTROPHIE MYOTONIQUE DE STEINERT PDF

Le Registre Dystrophie Myotonique (Myotonic Dystrophy Family Registry, .. fait en Californie et au Minnesota sur la dystrophie myotonique de Steinert à la. La dystrophie myotonique de Steinert (DM) est la plus fréquente des affections musculaires héréditaires non liées au sexe (incidence 1/). Le gène a été. Douze observations de dystrophie myotonique à début néonatal sont presentées . Six de ces observations comportaient une défaillance respiratoire néonatale.

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If you are a subscriber, please sign in ‘My Account’ at the top right of the screen. To better apprehend factors that may be involved in subjective fatigue in Steinert disease.

Affections neurologiques, Dystrophie myotonique type 1, Fatigue, Maladies neuromusculaires, Psychologie. At 17 months, motor development and precursors of language were delayed, and difficulties in feeding had required a gastrostomy. Access to the full text of this article requires a subscription. The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.

The signification of such ventilatory needs on patient outcome, particularly for motor handicaps, speech and dystropuie delay, and mental deficiency, remains uncertain. La maladie de Steinert: CDM1 is a severe condition, but variability in clinical manifestations and absence of genotype—phenotype correlation result in problems predicting prognosis at the individual level.

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Thus, more than in any other muscular diseases, factors that are not purely physical seem to be involved in fatigue felt by patients with Steinert disease. Personal information regarding our website’s visitors, including their identity, is confidential.

If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: Fatigue in Steinert myotonic dystrophy: Journal page Archives Contents list. Top of the page – Article Outline. You may thus steinet that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.

You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted. Outline Masquer le plan. As per the Law relating to information storage and personal integrity, you dystgophie the right to oppose art 26 of that lawaccess art 34 of that law and rectify art 36 of that law your personal data.

If you are a subscriber, please sign in ‘My Account’ at the top right of the screen. Journal page Archives Contents list. Van Den Hende aS. La fatigue dans la dystrophie myotonique de Steinert: You can move this window by clicking on the headline. Steinert myotonic dystrophy is one of the most frequent adult hereditary myopathies.

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Fatigue is one of the depression symptoms. Ethical concertation about withdrawal or maintenance of intensive care was engaged, taking into account the prolonged ventilation, the degree of prematurity, and the parental wishes for maximum care.

Top of the page – Article Outline. Physical, mental and subjective fatigues are well known different types of fatigue.

Dystrophie Myotonique de Type 1 – Maladie de Steinert – Description

Literature on fatigue showed how relevant this trouble could be for a majority of patients; and also how specific fatigue could be in Steinert disease, compared to other neuromuscular pathologies: Distinctions entre fatigue et somnolence myootnique la DM1. Montreuil bB.

The potential impact of in vitro fertilization on disease expression may also be considered.

You can move this window by clicking on the headline. Two prognostic factors predict the risk of death in early infancy: