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Clinical Chemistry and Laboratory Medicine (CCLM)

Published in Association with the European Federation of Clinical Chemistry and Laboratory Medicine (EFLM)

Editor-in-Chief: Plebani, Mario

Ed. by Gillery, Philippe / Lackner, Karl J. / Lippi, Giuseppe / Melichar, Bohuslav / Schlattmann, Peter / Tate, Jillian R. / Tsongalis, Gregory J.

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Activated Immune System in Patients with Huntington's Disease

Friedrich Leblhuber / Johannes Walli / Kurt Jellinger / Gernot P Tilz / Bernhard Widner / Franco Laccone / Dietmar Fuchs

Citation Information: Clinical Chemistry and Laboratory Medicine. Volume 36, Issue 10, Pages 747–750, ISSN (Print) 1434-6621, DOI: 10.1515/CCLM.1998.132, June 2005

Publication History

Published Online:


Abnormalities of immune system compartments were determined in 12 patients with Huntington's disease (eight males, four females; age 42.4 ± 11.7 years) and 11 controls (7 males, 4 females; age 47.0 ± 12.0). All patients were free from infectious diseases. Serum concentrations of a panel of serum soluble markers of immune activation were investigated, namely neopterin, 55-kDa-type soluble tumor necrosis factor receptor (sTNF-R), interleukin-2-receptor (sIL-2R), kynurenine, tryptophan, immunoglobulins (Ig) A, M and G as well as routine laboratory tests. Compared to controls, we found significantly higher serum levels of IgA (p < 0.01), sTNF-R, sIL-2R, neopterin, and complement component C3 (all p < 0.05), and serum tryptophan was decreased (p < 0.001). Higher concentrations of circulating immune complexes, cardiolipin antibodies, IgM, neopterin and lower tryptophan were associated with loss of cognitive function as assessed by the minimental-test. Five patients died within 1 year after measurements were performed. In these patients IgM, circulating immune complexes and neopterin concentrations were higher compared to survivors and serum tryptophan was lower. The data indicate an activation of various immune system compartments in Huntington's disease and that systemic immunological alterations might be important in the course of the disease.

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