Author(s):
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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.
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Subject:
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Neuromuscular and neurometabolic disorders Neuromusculaire en neurometabole aandoeningen |
Organization:
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Neurology Radboudumc Extern |
Abstract:
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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.
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