Spinal muscular atrophy in childhood: review of 50 cases / S. G. BENADY in Developmental Medicine & Child Neurology, 20-6 (December 1978)

Public ISBD [article]  inDevelopmental Medicine & Child Neurology > 20-6 (December 1978) . - p.746-757 Titre : Spinal muscular atrophy in childhood: review of 50 cases Type de document : Texte imprimé et/ou numérique Auteurs : S. G. BENADY, Auteur Année de publication : 1978 Article en page(s) : p.746-757 Langues : Anglais (eng) Index. décimale : PER Périodiques Résumé : The clinical findings in 50 children with intermediate or mild spinal muscular atrophy are reviewed. An early age of onset and the presence of tongue fasciculation carry a poor prognosis for disability. Scoliosis appears early in nearly all children with the more severe forms of spinal muscular atrophy and early energetic treatment is indicated. Contractures are also found in both the more severe and the milder forms and are the main cause of progressive disability, since continuous deterioration of muscle power was not evident in the present series. Respiratory failure is rare but potentially fatal, and may occur even in mild forms of the disease. Clinically evident weakness of the axial musculature may give warning of this complication. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=485 [article] Spinal muscular atrophy in childhood: review of 50 cases [Texte imprimé et/ou numérique] / S. G. BENADY, Auteur . - 1978 . - p.746-757. Langues : Anglais (eng) in Developmental Medicine & Child Neurology > 20-6 (December 1978) . - p.746-757 Index. décimale : PER Périodiques Résumé : The clinical findings in 50 children with intermediate or mild spinal muscular atrophy are reviewed. An early age of onset and the presence of tongue fasciculation carry a poor prognosis for disability. Scoliosis appears early in nearly all children with the more severe forms of spinal muscular atrophy and early energetic treatment is indicated. Contractures are also found in both the more severe and the milder forms and are the main cause of progressive disability, since continuous deterioration of muscle power was not evident in the present series. Respiratory failure is rare but potentially fatal, and may occur even in mild forms of the disease. Clinically evident weakness of the axial musculature may give warning of this complication. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=485

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