logo BDSP

Base documentaire


Votre avis nous intéresse

Le réseau BDSP met en oeuvre un projet d'innovation et d'amélioration de ses services, dans le souci constant de proposer des contenus de qualité adaptés aux besoins des utilisateurs.

Identifier de nouvelles sources de financements est la condition nécessaire pour assurer la poursuite et la pérennité de cet outil unique qu'est la BDSP, tout en le faisant évoluer.

Pour définir un nouveau modèle économique, nous avons besoin de votre avis : merci de répondre à notre enquête (temps estimé : 5 minutes).

Participer maintenant
Participer plus tard J'ai déjà participé

  1. Hereditary ataxias and spastic paraplegias : Methodological aspects of a prevalence study in Portugal.

    Article - En anglais

    A project for studying the prevalence of hereditary ataxias (HA) and familial spastic paraplegias (FSP) in Portugal was set up in 1993.

    The ascertainment of patients in previous prevalence studies relied mainly on the information of hospital admissions and out-patient contacts with the neurology and other related departments at central hospitals covering the whole region surveyed.

    Many patients might he overlooked if large populations were studied using this method, since registers at central hospitals are very incomplete and for most part not yet computerized.

    On the other hand HA and FSP are rare diseases appearing in family clusters, and it would be unreasonable to undertake a sample survey based upon a suitable frame of the Portuguese population.

    Therefore we decided to carry out a two-phase prevalence survey at district level, involving the collaboration of all physicians working in the district health institutions and the population, in the screening of eligible subjects in phase I. All subjects screened as positive were examined by a neurologist in phase 2. This method provided a direct estimate of false positives and false negatives were all patients also examined in phase 2, who came to our knowledge using other sources of information.

    The prevalence of hereditary ataxias and spastic paraplegias in the pilot district was 6.4 per 100,000 inhabitants.

    The sensitivity of the screening procedure was 81.2% and the predictive value of a positive screening was 25%. (...)

    Mots-clés Pascal : Ataxie, Paraplégie spasmodique héréditaire Strumpell Lorrain, Homme, Epidémiologie, Prévalence, Portugal, Europe, Méthodologie, Source information, Système nerveux pathologie, Système nerveux central pathologie, Encéphale pathologie, Trouble neurologique, Moelle épinière pathologie, Maladie dégénérative, Maladie héréditaire

    Mots-clés Pascal anglais : Ataxia, Hereditary spastic paraplegia, Human, Epidemiology, Prevalence, Portugal, Europe, Methodology, Information source, Nervous system diseases, Central nervous system disease, Cerebral disorder, Neurological disorder, Spinal cord disease, Degenerative disease, Genetic disease

    Logo du centre Notice produite par :
    Inist-CNRS - Institut de l'Information Scientifique et Technique

    Cote : 98-0404695

    Code Inist : 002B17G. Création : 25/01/1999.