Document Detail

Cerebrotendinous xanthomatosis: treatments with simvastatin, lovastatin, and chenodeoxycholic acid in 3 siblings.
MedLine Citation:
PMID:  2006015     Owner:  NLM     Status:  MEDLINE    
We report 3 sisters treated for cerebrotendinous xanthomatosis. We treated one, with a severe neurologic form of the illness, with chenodeoxycholic acid, then lovastatin and simvastatin. These drugs had different efficacy and tolerance, but induced no clinical improvement. Her sisters, without neurologic symptoms, received chenodeoxycholic acid, which normalized the cholestanol level. Optimal treatment of this illness must begin before there is significant clinical symptomatology.
J Peynet; A Laurent; P De Liege; P Lecoz; P Gambert; A Legrand; J Mikol; A Warnet
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neurology     Volume:  41     ISSN:  0028-3878     ISO Abbreviation:  Neurology     Publication Date:  1991 Mar 
Date Detail:
Created Date:  1991-04-25     Completed Date:  1991-04-25     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  434-6     Citation Subset:  AIM; IM    
Laboratoire de Biochimie, Hospital Lariboisière, Paris.
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MeSH Terms
Anticholesteremic Agents / therapeutic use
Brain Diseases / drug therapy*,  genetics,  radiography
Chenodeoxycholic Acid / therapeutic use*
Lovastatin / analogs & derivatives*,  therapeutic use*
Muscular Diseases / drug therapy,  genetics,  radiography
Tomography, X-Ray Computed
Xanthomatosis / drug therapy*,  genetics,  radiography
Reg. No./Substance:
0/Anticholesteremic Agents; 474-25-9/Chenodeoxycholic Acid; 75330-75-5/Lovastatin; 79902-63-9/Simvastatin
Comment In:
Neurology. 1994 Nov;44(11):2218   [PMID:  7970001 ]

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