title: HLA class I and II in Lambert-Eaton myasthenic syndrome without associated tumor.
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.
authors.affiliation: Department of Neurology, Leiden University Medical Center, The Netherlands. pwwirtz@lumc.nl
authors.forename: P W
authors.initials: PW
authors.lastname: Wirtz
authors.forename: B O
authors.initials: BO
authors.lastname: Roep
authors.forename: G M
authors.initials: GM
authors.lastname: Schreuder
authors.forename: P A
authors.initials: PA
authors.lastname: van Doorn
authors.forename: B G
authors.initials: BG
authors.lastname: van Engelen
authors.forename: J B
authors.initials: JB
authors.lastname: Kuks
authors.forename: A
authors.initials: A
authors.lastname: Twijnstra
authors.forename: M
authors.initials: M
authors.lastname: de Visser
authors.forename: L H
authors.initials: LH
authors.lastname: Visser
authors.forename: J H
authors.initials: JH
authors.lastname: Wokke
authors.forename: A R
authors.initials: AR
authors.lastname: Wintzen
authors.forename: J J
authors.initials: JJ
authors.lastname: Verschuuren
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000483:Alleles; D002648:Child; D005260:Female; D015395:Histocompatibility Antigens Class I / Q000145:classification*; D000949:Histocompatibility Antigens Class II / Q000145:classification*; D006650:Histocompatibility Testing; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000276:immunology*; D008297:Male; D008875:Middle Aged
chemical_list: D015395:Histocompatibility Antigens Class I; D000949:Histocompatibility Antigens Class II