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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Generalized hypotonia led to the diagnosis of the disease. If you want to subscribe to this journal, d our rates You can purchase this item in Pay Per View: Congenital myotonic dystrophy type I in a very premature neonate: Affections neurologiques, Dystrophie myotonique type 1, Fatigue, Maladies neuromusculaires, Psychologie.

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The signification of such ventilatory needs on patient outcome, particularly for motor handicaps, speech and language delay, and mental deficiency, remains uncertain. As per the Law relating to information storage and personal integrity, you have the right to oppose art 26 of that lawaccess art 34 of that law and rectify art 36 of that law your personal data. Ethical issues about the level of care, notably for tracheostomy and gastrostomy, should be adapted to each case, in partnership with parents.

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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. 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.

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You can move this window by clicking on the headline. Personal information regarding our website’s visitors, including their identity, is confidential. We have examined literature about other affections for which fatigue were richly documented. Access to the PDF text. Fatigue is one of the depression symptoms.

Dystrophie Myotonique de Type 1 – Maladie de Steinert – Description

You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, myotoique or deleted.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

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.

Contact Help Who are we? Access to the text HTML. Access to the full text of this article requires a subscription. La fatigue dans la dystrophie myotonique de Vystrophie La maladie de Steinert: Access to the PDF text.

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If you want to subscribe dyshrophie this journal, see our rates You can purchase this item in Pay Per View: 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.

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.

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: Contact Help Who are we?

Physical, mental and subjective fatigues dystropbie well known different types of fatigue. Filleron bG. If you are a subscriber, please sign in ‘My Account’ at the top right of the screen.

Mesnage aA. Steinert myotonic dystrophy is one of dystrolhie most frequent adult hereditary myopathies. Distinctions entre fatigue et somnolence dans la DM1. Two prognostic factors predict the risk of death in early infancy: However, clinically, it seems inefficient to try to understand those concepts separately, since they are highly intricate.