Literature DB >> 4073846

Treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid.

T A Pedley, R G Emerson, C L Warner, L P Rowland, G Salen.   

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.

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Year:  1985        PMID： 4073846     DOI： 10.1002/ana.410180418

Source DB:  PubMed          Journal:  Ann Neurol        ISSN： 0364-5134            Impact factor:   10.422


  8 in total

Review 1.  Cerebrotendinous xanthomatosis: clinical, electrophysiological and nerve biopsy findings, and response to treatment with chenodeoxycholic acid.

Authors:  M Donaghy; R H King; R O McKeran; M S Schwartz; P K Thomas
Journal:  J Neurol       Date:  1990-06       Impact factor: 4.849

2.  Case report 427: Cerebrotendinous xanthomatosis.

Authors:  M Burnstein; K A Buckwalter; W Martel; K D McClatchey; D Quint
Journal:  Skeletal Radiol       Date:  1987       Impact factor: 2.199

3.  The spectrum of magnetic resonance findings in cerebrotendinous xanthomatosis: redefinition and evidence of new markers of disease progression.

Authors:  Andrea Mignarri; Maria Teresa Dotti; Antonio Federico; Nicola De Stefano; Marco Battaglini; Irene Grazzini; Paolo Galluzzi; Lucia Monti
Journal:  J Neurol       Date:  2017-03-21       Impact factor: 4.849

4.  Cerebrotendinous xanthomatosis: cranial CT and MRI studies in eight patients.

Authors:  Y Hokezu; M Kuriyama; R Kubota; M Nakagawa; J Fujiyama; M Osame
Journal:  Neuroradiology       Date:  1992       Impact factor: 2.804

5.  Cerebrotendinous xanthomatosis: clinical and MRI study (a case report).

Authors:  M Fiorelli; V Di Piero; S Bastianello; L Bozzao; A Federico
Journal:  J Neurol Neurosurg Psychiatry       Date:  1990-01       Impact factor: 10.154

6.  Electrophysiological studies in cerebrotendinous xanthomatosis.

Authors:  Y Tokimura; M Kuriyama; K Arimura; J Fujiyama; M Osame
Journal:  J Neurol Neurosurg Psychiatry       Date:  1992-01       Impact factor: 10.154

7.  Quantification of brain damage in cerebrotendinous xanthomatosis with magnetization transfer MR imaging.

Authors:  Matilde Inglese; Nicola De Stefano; Elisabetta Pagani; Maria T Dotti; Giancarlo Comi; Antonio Federico; Massimo Filippi
Journal:  AJNR Am J Neuroradiol       Date:  2003-03       Impact factor: 3.825

8.  Cerebrotendinous xanthomatosis: a review of biochemical findings of the patient population in The Netherlands.

Authors:  B J Koopman; B G Wolthers; J C van der Molen; W van der Slik; R J Waterreus; A van Spreeken
Journal:  J Inherit Metab Dis       Date:  1988       Impact factor: 4.982
  8 in total

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