Aqueductal stenosis in X-linked hydrocephalus: a secondary phenomenon? / P. LANDRIEU in Developmental Medicine & Child Neurology, 21-5 (October 1979)

Public ISBD [article]  inDevelopmental Medicine & Child Neurology > 21-5 (October 1979) . - p.637-642 Titre : Aqueductal stenosis in X-linked hydrocephalus: a secondary phenomenon? Type de document : Texte imprimé et/ou numérique Auteurs : P. LANDRIEU, Auteur ; Gilles LYON, Auteur ; J. NINANE, Auteur ; G. FERRIERE, Auteur Année de publication : 1979 Article en page(s) : p.637-642 Langues : Anglais (eng) Index. décimale : PER Périodiques Résumé : Two cases of x-linked hydrocephalus are reported. One underwent postmortem examination: serial sections of the brain stem failed to show stenosis of the aqueduct, the mean and minimal cross-sectional areas of which were normal. However, there were some morphological changes which were compatible with a mechanical compression of the brain stem. It is postulated that in the Bickers and Adams syndrome of x-linked hereditary hydrocephalus the primary defect is a communicating hydrocephalus leading usually, but not always, to aqueductal stenosis. The importance of electrophysiological investigations for the correct diagnosis of the characteristic clasped-thumb deformity is also indicated. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=500 [article] Aqueductal stenosis in X-linked hydrocephalus: a secondary phenomenon? [Texte imprimé et/ou numérique] / P. LANDRIEU, Auteur ; Gilles LYON, Auteur ; J. NINANE, Auteur ; G. FERRIERE, Auteur . - 1979 . - p.637-642. Langues : Anglais (eng) in Developmental Medicine & Child Neurology > 21-5 (October 1979) . - p.637-642 Index. décimale : PER Périodiques Résumé : Two cases of x-linked hydrocephalus are reported. One underwent postmortem examination: serial sections of the brain stem failed to show stenosis of the aqueduct, the mean and minimal cross-sectional areas of which were normal. However, there were some morphological changes which were compatible with a mechanical compression of the brain stem. It is postulated that in the Bickers and Adams syndrome of x-linked hereditary hydrocephalus the primary defect is a communicating hydrocephalus leading usually, but not always, to aqueductal stenosis. The importance of electrophysiological investigations for the correct diagnosis of the characteristic clasped-thumb deformity is also indicated. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=500

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