Kanzaki M, Kaida K, Ueda M, Morita D, Hirakawa M, Motoyoshi K, Kamakura K, Kusunoki S
Division of Neurology, Internal Medicine 3, National Defense Medical College, Saitama, Japan.
J Neurol Neurosurg Psychiatry. 2008 Oct;79(10):1148-52. doi: 10.1136/jnnp.2007.142950. Epub 2008 Mar 13.
Serum antibodies to GQ1b are associated with Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) with ophthalmoplegia. Antibodies to ganglioside complexes (GSCs) have not yet been examined in a large population of patients with MFS or GBS. This study aimed to determine the clinical significance of antibodies to GSCs in MFS and GBS.
The study investigated serum anti-GSC antibodies and the clinical features in 64 MFS patients, 53 GBS patients with ophthalmoplegia (GBS-OP(+)) and 53 GBS patients without ophthalmoplegia (GBS-OP(-)).
Thirty patients with MFS (47%), 25 with GBS-OP(+) (47%) and none with GBS-OP(-) had antibodies to GSCs containing GQ1b or GT1a. Patients with MFS and GBS-OP(+) were subdivided according to the antibody reactivities; patients with antibodies specific to GQ1b and/or GT1a (without anti-GSCs antibodies) were placed in Group 1, those with antibodies against GSCs with a total of two sialic acids in the terminal residues, such as GQ1b/GM1, were placed in Group 2, and those with antibodies against GSCs with a total of three sialic acids in the terminal residue, such as GQ1b/GD1a, were placed in Group 3. In MFS, sensory disturbances were infrequent in Group 2 compared with the other groups (p<0.0001). Antibodies specific to GQ1b were observed more often in MFS than in GBS-OP(+) (p = 0.0002).
IgG antibodies to GSCs containing GQ1b or GT1a were closely associated with the development of ophthalmoplegia in GBS, as well as MFS. Both GQ1b and clustered epitopes of GSCs containing GQ1b or GT1a may be prime target antigens for MFS and GBS-OP(+).
抗GQ1b血清抗体与米勒费雪综合征（MFS）及伴有眼肌麻痹的吉兰-巴雷综合征（GBS）相关。尚未在大量MFS或GBS患者中检测针对神经节苷脂复合物（GSCs）的抗体。本研究旨在确定抗GSCs抗体在MFS和GBS中的临床意义。
本研究调查了64例MFS患者、53例伴有眼肌麻痹的GBS患者（GBS-OP(+)）和53例不伴有眼肌麻痹的GBS患者（GBS-OP(-)）的血清抗GSC抗体及临床特征。
30例MFS患者（47%）、25例GBS-OP(+)患者（47%）以及无GBS-OP(-)患者检测到针对含GQ1b或GT1a的GSCs的抗体。根据抗体反应性对MFS和GBS-OP(+)患者进行细分；将针对GQ1b和/或GT1a的特异性抗体（无抗GSCs抗体）的患者归为第1组，将针对末端残基共有两个唾液酸的GSCs的抗体患者，如GQ1b/GM1，归为第2组，将针对末端残基共有三个唾液酸的GSCs的抗体患者，如GQ1b/GD1a，归为第3组。在MFS中，与其他组相比，第2组感觉障碍较少见（p<0.0001）。MFS中GQ1b特异性抗体的观察频率高于GBS-OP(+)（p = 0.0002）。
针对含GQ1b或GT1a的GSCs的IgG抗体与GBS以及MFS中眼肌麻痹的发生密切相关。GQ1b以及含GQ1b或GT1a的GSCs的聚集表位可能是MFS和GBS-OP(+)的主要靶抗原。
