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Treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid
Author(s) -
Pedley Timothy A.,
Emerson Ronald G.,
Warner Carolyn L.,
Rowland Lewis P.,
Salen Gerald
Publication year - 1985
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410180418
Subject(s) - cerebrotendinous xanthomatosis , chenodeoxycholic acid , medicine , cholesterol
Abstract We describe a patient with cerebrotendinous xanthomatosis who was treated for one year with chenodeoxycholic acid. Modest clinical improvement was accompanied by marked improvement in visual and brainstem auditory evoked potentials. Improved central nervous system function coincided with return of plasma and cerebrospinal fluid cholestanol levels to normal.