Cerebrotendinous xanthomatosis: Evidence of lipomatous hypertrophy of the atrial septum

Maria Teresa Dotti, Sergio Mondillo, Katrin Plewnia, Eustachio Agricola, Antonio Federico

Research output: Contribution to journalArticle

Abstract

Premature atherosclerosis and cardiac complications have been reported among the systemic manifestations of cerebrotendinous xanthomatosis (CTX), a rare bile acid disorder with predominantly neurological features. In some cases, myocardial infarction has been the cause of sudden death. We examined nine CTX patients to determine whether they also had clinical or subclinical signs of cardiovascular disorders. In four of them, transthoracic echocardiography showed thickening of the interatrial septum compatible with lipomatous hypertrophy. The unexpected association of the two abnormalities is unlikely to be coincidental and suggests that careful cardiac examination should be considered, even in the absence of clinical manifestations.

Original languageEnglish
Pages (from-to)723-726
Number of pages4
JournalJournal of Neurology
Volume245
Issue number11
DOIs
Publication statusPublished - 1998

Fingerprint

Cerebrotendinous Xanthomatosis
Atrial Septum
Hypertrophy
Sudden Death
Bile Acids and Salts
Echocardiography
Cause of Death
Atherosclerosis
Myocardial Infarction

Keywords

  • Cerebrotendinous xanthomatosis
  • Interatrial septum
  • Lipomatous hypertrophy

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Cerebrotendinous xanthomatosis : Evidence of lipomatous hypertrophy of the atrial septum. / Dotti, Maria Teresa; Mondillo, Sergio; Plewnia, Katrin; Agricola, Eustachio; Federico, Antonio.

In: Journal of Neurology, Vol. 245, No. 11, 1998, p. 723-726.

Research output: Contribution to journalArticle

Dotti, Maria Teresa ; Mondillo, Sergio ; Plewnia, Katrin ; Agricola, Eustachio ; Federico, Antonio. / Cerebrotendinous xanthomatosis : Evidence of lipomatous hypertrophy of the atrial septum. In: Journal of Neurology. 1998 ; Vol. 245, No. 11. pp. 723-726.
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