Occurrence of Parkinson's syndrome in type 1 Gaucher disease
Identifieur interne : 002087 ( Main/Exploration ); précédent : 002086; suivant : 002088Occurrence of Parkinson's syndrome in type 1 Gaucher disease
Auteurs : O. Neudorfer [Israël] ; N. Giladi [Israël] ; D. Elstein [Israël] ; A. Abrahamov [Israël] ; T. Turezkite [Israël] ; E. Aghai [Israël] ; A. Reches [Israël] ; B. Bembi [Israël] ; A. Zimran [Israël]Source :
- QJM: An International Journal of Medicine [ 1460-2725 ] ; 1996-09.
Abstract
Gaucher disease, the most prevalent glycolipid storage disorder, is classically subdivided into types according to the presence or absence of neurological involvement. Type I has hitherto been considered non-neuronopathic. We present six cases and a review of the literature of Parkinsonian symptoms in type I Gaucher disease patients. The hallmark of this atypical Parkinsonian syndrome is a relatively severe clinical course with early appearance of neurological signs in the 4th to 6th decade of life, aggressive progression of the signs and refractoriness to conventional anti-Parkinson therapy. We discuss the implications of these findings in the light of enzyme replacement therapy for Gaucher disease.
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DOI: 10.1093/qjmed/89.9.691
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Type I has hitherto been considered non-neuronopathic. We present six cases and a review of the literature of Parkinsonian symptoms in type I Gaucher disease patients. The hallmark of this atypical Parkinsonian syndrome is a relatively severe clinical course with early appearance of neurological signs in the 4th to 6th decade of life, aggressive progression of the signs and refractoriness to conventional anti-Parkinson therapy. We discuss the implications of these findings in the light of enzyme replacement therapy for Gaucher disease.</div></front></TEI><affiliations><list><country><li>Israël</li></country></list><tree><country name="Israël"><noRegion><name sortKey="Neudorfer, O" sort="Neudorfer, O" uniqKey="Neudorfer O" first="O." last="Neudorfer">O. Neudorfer</name></noRegion><name sortKey="Abrahamov, A" sort="Abrahamov, A" uniqKey="Abrahamov A" first="A." last="Abrahamov">A. Abrahamov</name><name sortKey="Aghai, E" sort="Aghai, E" uniqKey="Aghai E" first="E." last="Aghai">E. Aghai</name><name sortKey="Bembi, B" sort="Bembi, B" uniqKey="Bembi B" first="B." last="Bembi">B. Bembi</name><name sortKey="Elstein, D" sort="Elstein, D" uniqKey="Elstein D" first="D." last="Elstein">D. Elstein</name><name sortKey="Giladi, N" sort="Giladi, N" uniqKey="Giladi N" first="N." last="Giladi">N. Giladi</name><name sortKey="Reches, A" sort="Reches, A" uniqKey="Reches A" first="A." last="Reches">A. Reches</name><name sortKey="Turezkite, T" sort="Turezkite, T" uniqKey="Turezkite T" first="T." last="Turezkite">T. Turezkite</name><name sortKey="Zimran, A" sort="Zimran, A" uniqKey="Zimran A" first="A." last="Zimran">A. Zimran</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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