Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
Neuroimmunology and Brain Autoimmunity Groups, Kids Neuroscience Centre, Children's Hospital at Westmead; Brain and Mind Centre and Sydney Medical School, Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia.
Ann Neurol. 2021 Oct;90(4):683-690. doi: 10.1002/ana.26189. Epub 2021 Aug 30.
Pain is a under-recognized association of leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) antibodies. Of 147 patients with these autoantibodies, pain was experienced by 17 of 33 (52%) with CASPR2- versus 20 of 108 (19%) with LGI1 antibodies (p = 0.0005), and identified as neuropathic in 89% versus 58% of these, respectively. Typically, in both cohorts, normal nerve conduction studies and reduced intraepidermal nerve fiber densities were observed in the sampled patient subsets. In LGI1 antibody patients, pain responded to immunotherapy (p = 0.008), often rapidly, with greater residual patient-rated impairment observed in CASPR2 antibody patients (p = 0.019). Serum CASPR2 antibodies, but not LGI1 antibodies, bound in vitro to unmyelinated human sensory neurons and rodent dorsal root ganglia, suggesting pathophysiological differences that may underlie our clinical observations. ANN NEUROL 2021;90:683-690.
疼痛是富含亮氨酸胶质瘤失活 1 型 (LGI1) 和接触蛋白相关蛋白样 2 (CASPR2) 抗体的一个未被充分认识的关联。在这 147 名自身抗体患者中，33 名 CASPR2 抗体患者中有 17 名（52%）出现疼痛，而 108 名 LGI1 抗体患者中有 20 名（19%）出现疼痛（p=0.0005），并且这些患者中分别有 89%和 58%被确定为神经病理性疼痛。通常情况下，在这两个队列中，在抽样的患者亚组中观察到正常的神经传导研究和表皮内神经纤维密度降低。在 LGI1 抗体患者中，疼痛对免疫治疗有反应（p=0.008），通常反应迅速，而在 CASPR2 抗体患者中观察到更大的残留患者评分受损（p=0.019）。血清 CASPR2 抗体，但不是 LGI1 抗体，在体外与未髓鞘化的人类感觉神经元和啮齿动物背根神经节结合，这表明可能是潜在的病理生理学差异导致了我们的临床观察。神经病学年鉴 2021；90:683-690。
