Widdess-Walsh Peter, Tavee Jinny O, Schuele Stephan, Stevens Glen H
Department of Neurology, Cleveland Clinic Foundation, Cleveland, OH 44195, USA.
J Neurooncol. 2003 Jun;63(2):187-90. doi: 10.1023/a:1023931501503.
Paraneoplastic cerebellar degeneration (PCD) is a debilitating neuro-degenerative disease associated with antibodies directed against the purkinje cells of the cerebellum. Treatment using chemotherapy or other treatment of the primary tumor to various immunologically directed therapies has been attempted but outcomes have been poor. We discuss a patient with ovarian carcinoma and PCD seen in our institution who showed a marked beneficial response to intravenous immunoglobulin (IVIG) and methylprednisolone. A Medline search from 1966-2002 produced fifteen cases of PCD confirmed by antibody testing that were treated with IVIG, either alone, or with a combination of other therapies. The clinical characteristics and treatment responses of these patients are analyzed in this review. Most patients that were treated with IVIG and had what was defined as a good response were treated within one month of symptoms. Patients treated between one month and three months often had stable disease and patients treated after three months of symptoms usually had a poor outcome. Early treatment with sufficiently high doses of IVIG seems to provide a better chance of treatment success. The additional benefit of early high dose intravenous methylprednisolone is unclear. Due to the devastating nature of the disease, a trial of IVIG and steroids is warranted as early as possible in a dose of 2g/kg to any patient with a clinical picture of PCD and positive antibodies.
副肿瘤性小脑变性（PCD）是一种使人衰弱的神经退行性疾病，与针对小脑浦肯野细胞的抗体有关。人们尝试了使用化疗或对原发肿瘤进行其他治疗以及各种免疫导向疗法，但效果不佳。我们讨论了在我们机构中见到的一名患有卵巢癌和PCD的患者，该患者对静脉注射免疫球蛋白（IVIG）和甲基强的松龙表现出明显的有益反应。对1966年至2002年的医学文献数据库检索发现了15例经抗体检测确诊为PCD且接受过IVIG治疗的病例，这些治疗或单独使用IVIG，或与其他疗法联合使用。本综述分析了这些患者的临床特征和治疗反应。大多数接受IVIG治疗且有良好反应的患者在出现症状后的一个月内接受了治疗。在症状出现后的一至三个月内接受治疗的患者病情往往稳定，而在症状出现三个月后接受治疗的患者通常预后较差。尽早使用足够高剂量的IVIG进行治疗似乎能提供更好的治疗成功机会。早期高剂量静脉注射甲基强的松龙的额外益处尚不清楚。鉴于该疾病的严重性，对于任何具有PCD临床表现且抗体呈阳性的患者，应尽早进行IVIG和类固醇的试验，剂量为2g/kg。
