Schirmer L, Worthington V, Solloch U, Loleit V, Grummel V, Lakdawala N, Grant D, Wassmuth R, Schmidt A H, Gebhardt F, Andlauer T F M, Sauter J, Berthele A, Lunn M P, Hemmer Bernhard
Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.
MRC Centre for Neuromuscular Diseases and Neuroimmunology and CSF Laboratory, National Hospital for Neurology, London, UK.
J Neurol. 2016 Oct;263(10):2105-13. doi: 10.1007/s00415-016-8237-6. Epub 2016 Aug 2.
Few regional and seasonal Guillain-Barré syndrome (GBS) clusters have been reported so far. It is unknown whether patients suffering from sporadic GBS differ from GBS clusters with respect to clinical and paraclinical parameters, HLA association and antibody response to glycosphingolipids and Campylobacter jejuni (Cj). We examined 40 consecutive patients with GBS from the greater Munich area in Germany with 14 of those admitted within a period of 3 months in fall 2010 defining a cluster of GBS. Sequencing-based HLA typing of the HLA genes DRB1, DQB1, and DPB1 was performed, and ELISA for anti-glycosphingolipid antibodies was carried out. Clinical and paraclinical findings (Cj seroreactivity, cerebrospinal fluid parameters, and electrophysiology) were obtained and analyzed. GBS cluster patients were characterized by a more severe clinical phenotype with more patients requiring mechanical ventilation and higher frequencies of autoantibodies against sulfatide, GalC and certain ganglioside epitopes (54 %) as compared to sporadic GBS cases (13 %, p = 0.017). Cj seropositivity tended to be higher within GBS cluster patients (69 %) as compared to sporadic cases (46 %, p = 0.155). We noted higher frequencies of HLA class II allele DQB105:01 in the cluster cohort (23 %) as compared to sporadic GBS patients (3 %, p = 0.019). Cluster of severe GBS was defined by higher frequencies of autoantibodies against glycosphingolipids. HLA class II allele DQB105:01 might contribute to clinical worsening in the cluster patients.
迄今为止，鲜有关于区域性和季节性吉兰-巴雷综合征（GBS）聚集性病例的报道。目前尚不清楚散发性GBS患者与GBS聚集性病例在临床和辅助临床参数、HLA关联以及针对糖鞘脂和空肠弯曲菌（Cj）的抗体反应方面是否存在差异。我们对德国大慕尼黑地区连续40例GBS患者进行了研究，其中14例在2010年秋季的3个月内入院，构成了一个GBS聚集性病例组。对HLA基因DRB1、DQB1和DPB1进行了基于测序的HLA分型，并开展了抗糖鞘脂抗体的酶联免疫吸附测定（ELISA）。获取并分析了临床和辅助临床检查结果（Cj血清反应性、脑脊液参数和电生理学）。与散发性GBS病例相比，GBS聚集性病例组患者的临床表型更为严重，更多患者需要机械通气，针对硫脂、半乳糖脑苷脂（GalC）和某些神经节苷脂表位的自身抗体频率更高（54%）（散发性GBS病例为13%，p = 0.017）。与散发性病例相比，GBS聚集性病例组患者的Cj血清阳性率有升高趋势（69%）（散发性病例为46%，p = 0.155）。我们注意到，与散发性GBS患者相比，聚集性病例组中HLA II类等位基因DQB105:01的频率更高（23%）（散发性GBS患者为3%，p = 0.019）。严重GBS聚集性病例的特点是针对糖鞘脂的自身抗体频率较高。HLA II类等位基因DQB105:01可能导致聚集性病例组患者的临床症状恶化。
