Furneaux H M, Rosenblum M K, Dalmau J, Wong E, Woodruff P, Graus F, Posner J B
Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021.
N Engl J Med. 1990 Jun 28;322(26):1844-51. doi: 10.1056/NEJM199006283222604.
Paraneoplastic cerebellar degeneration is a rare syndrome that occurs in patients with gynecologic cancer and is characterized by widespread loss of Purkinje cells. To determine whether Purkinje-cell antigens are selectively expressed in the tumors of patients with the syndrome, we examined tumor tissue from 10 patients whose serum contained anti-Purkinje-cell (anti-Yo) antibodies. The origins of the cancers were the breast (five patients), ovary (three), endometrium (one), and fallopian tube (one). We used as controls tumor tissue from 11 patients with ovarian cancer and 10 patients with breast cancer who were neurologically normal. Using immunohistochemical and Western blot analysis, we found that Purkinje-cell antigens were expressed in all the tumors from the 10 patients with paraneoplastic cerebellar degeneration but in none of the tumors from the 21 neurologically normal patients. When IgG from patients with paraneoplastic cerebellar degeneration was affinity-purified to cerebellar Purkinje-cell antigen, immunohistochemical analysis showed that it reacted specifically with the tumor tissue from those patients. We conclude that in patients with paraneoplastic cerebellar degeneration, the anti-Yo antibody results from an immune response to neural antigens expressed by the gynecologic tumors in the patients.
副肿瘤性小脑变性是一种罕见的综合征，发生于妇科癌症患者，其特征是浦肯野细胞广泛丢失。为了确定浦肯野细胞抗原是否在该综合征患者的肿瘤中选择性表达，我们检测了10例血清中含有抗浦肯野细胞（抗Yo）抗体患者的肿瘤组织。癌症起源于乳腺（5例）、卵巢（3例）、子宫内膜（1例）和输卵管（1例）。我们将11例神经系统正常的卵巢癌患者和10例乳腺癌患者的肿瘤组织作为对照。通过免疫组织化学和蛋白质印迹分析，我们发现10例副肿瘤性小脑变性患者的所有肿瘤中均表达浦肯野细胞抗原，而21例神经系统正常患者的肿瘤中均未表达。当将副肿瘤性小脑变性患者的IgG亲和纯化至小脑浦肯野细胞抗原时，免疫组织化学分析显示其与这些患者的肿瘤组织特异性反应。我们得出结论，在副肿瘤性小脑变性患者中，抗Yo抗体是由患者对妇科肿瘤表达的神经抗原产生的免疫反应所致。
