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Defective Fas-mediated T-cell apoptosis predicts acute onset CIDP

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Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are immune-mediated neuropathies. GBS is characterized by acute onset and subsequent remission of symptoms, whereas CIDP displays slow progression over at least 2 months. However, a small proportion of CIDP patients display acute onset CIDP (a-CIDP) resembling that of GBS. The Fas receptor is involved in shutting off the immune response and its defective function predisposes to auto-immune diseases. In CIDP patients, Fas function is lower than in GBS patients and healthy controls. This study is aimed at assessing whether evaluation of T-cell Fas function helps in early discrimination between GBS and a-CIDP. Fas function was evaluated in patients with acute onset polyneuropathy: 55 retrospective patients analyzed after development of GBS or a-CIDP before year 2005; 50 prospective patients analyzed at onset after year 2005 and followed up for development of GBS or a-CIDP. In both groups, a-CIDP patients displayed defective Fas function, whereas GBS patients displayed normal function. These findings suggest that the evaluation of Fas function in acute onset polyneuropathy helps in early prediction of long-term outcome.
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Keywords: Fas; autoimmunity; peripheral neuropathy

Document Type: Research Article

Affiliations: 1: Department of Neurology, L. Sacco Hospital, University of Milan, Milan, Italy 2: Interdisciplinary Research Centre of Autoimmune Diseases (IRCAD), ‘A. Avogadro’ University of Eastern Piedmont, Novara 3: Department of Neurology, ‘A. Avogadro’ University of Eastern Piedmont, Maggiore Hospital, Novara

Publication date: June 1, 2009

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