Yang Zhao, He Lu, Ren Manli, Lu Yizhou, Meng Huanyu, Yin Dou, Chen Sheng, Zhou Qinming
Department of Neurology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200240, China.
Institute of Neurology, Shanghai Jiaotong University, Shanghai 200240, China.
Brain Sci. 2022 Aug 8;12(8):1053. doi: 10.3390/brainsci12081053.
Paraneoplastic amyotrophic lateral sclerosis (ALS) is a rare and special type of ALS. The pathogenesis, clinical presentation, treatment and prognosis remain poorly understood. We herein presented three cases of paraneoplastic ALS. In case 1, we first reported an ALS patient with the positive serum antibodies against both Sry-like high mobility group box 1 (SOX1) and glutamic acid decarboxylase 65 (GAD65). However, immunotherapy did not improve his neurological symptoms. We also reported two ALS patients with renal clear cell carcinoma and chronic myelogenous leukemia. No positive paraneoplastic antibodies were detected in either the serum or the cerebrospinal fluid of the two patients, and their clinical symptoms progressed slowly after tumor treatment. The three cases enriched the existing case pool of this rare disorder. In addition, we have comprehensively reviewed the literature of paraneoplastic ALS. The clinical features, treatment effect and prognosis were summarized to broaden our understanding of paraneoplastic ALS.
副肿瘤性肌萎缩侧索硬化症（ALS）是一种罕见且特殊类型的ALS。其发病机制、临床表现、治疗及预后仍知之甚少。我们在此报告3例副肿瘤性ALS病例。在病例1中，我们首次报道了1例血清抗性别决定区Y盒蛋白1（SOX1）和谷氨酸脱羧酶65（GAD65）抗体均呈阳性的ALS患者。然而，免疫治疗并未改善其神经症状。我们还报告了2例分别患有肾透明细胞癌和慢性粒细胞白血病的ALS患者。这2例患者的血清和脑脊液中均未检测到阳性副肿瘤抗体，且在肿瘤治疗后其临床症状进展缓慢。这3例病例丰富了这种罕见疾病现有的病例库。此外，我们全面回顾了副肿瘤性ALS的文献。总结了其临床特征、治疗效果及预后，以拓宽我们对副肿瘤性ALS的认识。
