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Epilepsy and Turner's syndrome: report of a case and review of the literature

Trevisol-Bittencourt, PC; Sander, JW; (1990) Epilepsy and Turner's syndrome: report of a case and review of the literature. Arquivos de neuro-psiquiatria , 48 (3) 360 - 365. 10.1590/S0004-282X1990000300016. Green open access

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Abstract

Fifty years ago Henry Turner identified the association of sexual infantilism, webbed neck and cubitus valgus to be a separate entity and subsequently gonadal dysgenesis was appended to the definition. Twenty years after the original report it was demonstrated that in typical patients with Turner's syndrome the chromosomal composition was 45,X0 and in addition cases of mosaicism were described. Although much general information is now available about Turner's syndrome the neurological complications are largely neglected. In this paper we review the case of a patient with Turner's syndrome who at age of 16 years developed severe epilepsy. In addition, a survey of the literature concerning neurological abnormalities associated with this syndrome is presented. It seems clear that neurological symptoms in patients with Turner's syndrome deserves consideration for many reasons; may be the most significant is the evidence that patients with this syndrome have a high incidence of vascular abnormalities, which may also affect the CNS. Empirically patients with Turner's syndrome are at risk of developing cerebral vascular diseases. Also the relatively high frequency of cognitive abnormalities in these patients seems to justify the hypothesis that in a certain percentage of cases a CNS dysfunction or malformation, not necessarily of a vascular nature, may be a component of the syndrome still nor described.

Type: Article
Title: Epilepsy and Turner's syndrome: report of a case and review of the literature
Open access status: An open access version is available from UCL Discovery
DOI: 10.1590/S0004-282X1990000300016
Publisher version: http://www.scielo.br/scielo.php?pid=S0004-282X1990...
Language: Portuguese
Additional information: Article in Portuguese. Original title: Epilepsia e sindrome de Turner: apresentacao de um caso e revisao da literatura. All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License.
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Experimental Epilepsy
URI: https://discovery.ucl.ac.uk/id/eprint/81168
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