You are seeing this message because your Web browser does not support basic Web standards. Find out more about why this message is appearing and what you can do to make your experience on this site better.


ABOUT ARCHIVES
Advanced Search

Welcome   | My Account | E-mail Alerts | Access Rights | Sign In


  Vol. 49 No. 5, May 1992 TABLE OF CONTENTS
  Archives
  •  Online Features
  ARTICLE
 This Article
 • Reply to article
 •Send to a friend
 • Save in My Folder
 •Save to citation manager
 •Permissions
 Citing Articles
 •Citing articles on HighWire
 •Contact me when this article is cited
 Related Content
 •Similar articles in this journal

Evoked potentials in cerebrotendinous xanthomatosis and effect induced by chenodeoxycholic acid

M. Mondelli, A. Rossi, C. Scarpini, M. T. Dotti and A. Federico
Institute of Neurological Sciences, Medical School, University of Siena, Italy.

Evoked potentials are reported in 10 patients with cerebrotendinous xanthomatosis, eight of whom had peripheral neuropathy. Four subjects showed delayed N13 to N20 interpeak latencies for arm somatosensory evoked potentials, and five showed moderately prolonged I to III and I to V interpeak latencies of brain-stem auditory evoked potentials. Six of seven patients showed marked delay and desynchronization of visual evoked potentials. All five patients undergoing transcutaneous magnetic stimulation of the motor cortex presented greatly delayed central motor conduction time, especially of the lower limbs. After treatment with chenodiol (750 mg/d for at least 2 years), there was a significant improvement in nerve conduction velocities, N13 to N20 interpeak latencies, and visual evoked potential latencies. Brain-stem auditory evoked potentials remained unchanged.

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Cerebrotendinous Xanthomatosis: Clinical Manifestations, Diagnostic Criteria, Pathogenesis, and Therapy
Federico and Dotti
J Child Neurol 2003;18:633-638.
ABSTRACT  

Cerebrotendinous xanthomatosis
Federico et al.
Neurology 2001;57:1743-1743.
FULL TEXT  

Mutations producing premature termination of translation and an amino acid substitution in the sterol 27-hydroxylase gene cause cerebrotendinous xanthomatosis associated with parkinsonism
Wakamatsu et al.
J. Neurol. Neurosurg. Psychiatry 1999;67:195-198.
ABSTRACT | FULL TEXT  





HOME | CURRENT ISSUE | PAST ISSUES | TOPIC COLLECTIONS | CME | SUBMIT | SUBSCRIBE | HELP
CONDITIONS OF USE | PRIVACY POLICY | CONTACT US | SITE MAP
 
© 1992 American Medical Association. All Rights Reserved.