HLA class I and II in Lambert-Eaton myasthenic syndrome without associated tumor.

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Title:	HLA class I and II in Lambert-Eaton myasthenic syndrome without associated tumor.
Author(s):	Wirtz, P.W.; Roep, B.O.; Schreuder, G.M.; Doorn, P. van; Engelen, B.G.M. van ; Kuks, J.B.M.; Twijnstra, A.; Visser, L.H.; Wokke, J.H.J.; Wintzen, A.R.; Verschuuren, J.J.; Visser, J.E.
Publication year:	2001
Source:	Human Immunology, vol. 62, iss. 8, (2001), pp. 809--13
ISSN:	0198-8859
DOI:	https://doi.org/10.1016/S0198-8859(01)00270-1
Publication type:	Article / Letter to editor
Please use this identifier to cite or link to this item : https://hdl.handle.net/2066/186819
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Subject:	Neuromuscular and neurometabolic disorders Neuromusculaire en neurometabole aandoeningen
Organization:	Neurology Radboudumc Extern
Journal title:	Human Immunology
Volume:	vol. 62
Issue:	iss. 8
Page start:	p. 809-
Page end:	p. 13
Abstract:	Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder, in which antibodies against voltage-gated calcium channels located at nerve terminals cause muscle weakness and autonomic dysfunction. In approximately half of the patients the autoimmune process is initiated by a tumor. In the other half of patients no tumor is found and the etiology is unknown. The aims of this study were to investigate the strength of HLA-associations with nontumor LEMS (NT-LEMS) and to study the relation of HLA-haplotypes with age at onset of LEMS and other clinical features. Therefore, typing of HLA class I and II was performed in 19 patients with NT-LEMS, who were clinically evaluated. NT-LEMS was significantly associated with alleles of both HLA-class I (i.e. HLA-B8) as well as -class II (i.e. HLA-DR3 and -DQ2). HLA-B8+ patients had significantly younger age at onset of LEMS and tended to be female. This study shows that HLA-class I haplotype is associated with a distinct phenotype in NT-LEMS.