Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.
Headache Science and Neurorehabilitation Center, IRCCS Mondino Foundation, Pavia, Italy.
Cerebellum. 2024 Jun;23(3):1249-1253. doi: 10.1007/s12311-023-01601-w. Epub 2023 Oct 18.
Antibodies against the neuronal protein Ma2 have been reported in a peculiar form of paraneoplastic encephalitis with prominent involvement of the limbic, brainstem, and diencephalic structures and usually associated with germ cell testicular, lung, or breast cancer. The diagnosis is frequently challenged by atypical clinical manifestations including parkinsonism, sleep disturbances, hypothalamic-pituitary dysfunctions, and motor neuron-like syndrome. In recent years, the advent of monoclonal antibodies targeting immune checkpoints has deeply changed the treatment of different tumors, especially melanoma and lung cancer. However, given their nature, an increasing number of neurological immune-related adverse events, including ocular motor abnormalities, have been described. Here, we report a woman with advanced non-small cell lung cancer treated with anti-PD-L1 durvalumab, presenting with an isolated pendular torsional nystagmus, in association with anti-Ma2 antibodies. This peculiar case widens our knowledge on the clinical presentation of anti-Ma2 encephalitis associated with checkpoint inhibitors.
已报道抗神经元蛋白 Ma2 的抗体存在于一种特殊形式的副肿瘤性脑炎中,其明显累及边缘系统、脑干和间脑结构,通常与生殖细胞睾丸癌、肺癌或乳腺癌相关。诊断通常受到非典型临床表现的挑战,包括帕金森病、睡眠障碍、下丘脑-垂体功能障碍和运动神经元样综合征。近年来,针对免疫检查点的单克隆抗体的出现极大地改变了不同肿瘤的治疗方法,尤其是黑色素瘤和肺癌。然而,鉴于其性质,越来越多的与神经免疫相关的不良反应,包括眼球运动异常,已经被描述。在这里,我们报告了一例晚期非小细胞肺癌患者,使用抗 PD-L1 度伐利尤单抗治疗,表现为孤立性摆动性扭转性眼球震颤,同时伴有抗 Ma2 抗体。这种特殊情况拓宽了我们对与检查点抑制剂相关的抗 Ma2 脑炎临床表现的认识。