HLA class I and II in Lambert-Eaton myasthenic syndrome without associated tumor.
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.
Wirtz
P W
PW
Department of Neurology, Leiden University Medical Center, The Netherlands. pwwirtz@lumc.nl
Roep
B O
BO
Schreuder
G M
GM
van Doorn
P A
PA
van Engelen
B G
BG
Kuks
J B
JB
Twijnstra
A
A
de Visser
M
M
Visser
L H
LH
Wokke
J H
JH
Wintzen
A R
AR
Verschuuren
J J
JJ
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
D015395:Histocompatibility Antigens Class I; D000949:Histocompatibility Antigens Class II