Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, Anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone
Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, Anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone
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DOI：
10.1136/jnnp.68.4.479
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发表时间：
2000-04-01
影响因子：
11
通讯作者：
Delattre, JY
中科院分区：
文献类型：
--
作者：
Keime-Guibert, F;Graus, F;Delattre, JY
目的 - 评估免疫球蛋白 (IVIg)、环磷酰胺 (CTX) 和甲泼尼龙 (MP) 组合对副肿瘤性神经综合征 (PNS) 和抗神经元抗体 (Abs) 患者临床病程的影响。方法 - 17 例患有副肿瘤性脑脊髓炎/感觉神经病 (PEM/SN) 的患者，其具有抗 Hu 抗体 (n=10) 或抗 Yo 抗体 (n=7) 的小脑变性 (PCD) 患者接受 1 至 9 个周期（平均 3.5）的 IVIg（第 1 至 5 天 0.5 g/kg/天）、CTX（第 1 天 600 mg/m2）和 MP（第 1 至 3 天 1g/天）的组合治疗。使用兰金量表（RS）来评估反应。对于治疗开始时仍可卧床的患者（RS 小于或等于 3），阳性反应被认为是改善或稳定，而对于卧床不起的患者（RS 大于或等于 4），仅改善而不是稳定被认为是治疗益处。结果 - 耐受性良好，没有患者经历 3/4 级毒性（世界卫生组织）。可评估 16 名患者的反应。 RS 大于或等于 4 的 7 名患者中，没有任何改善。在 RS 大于或等于 3 的 9 例患者中，没有任何改善，但 3 例（2 例 SN 和 1 例 PCD）稳定了 4、35 和 16 个月。结论 - 这项研究表明，强力免疫抑制治疗对于具有抗神经元抗体的严重残疾 PNS 患者没有用。在少数在治疗开始时并未严重残疾的患者（主要是 SN）中，短暂稳定是可能的，值得进一步评估。
Objectives-To evaluate the effect of a combination of immunoglobulins (IVIg), cyclophosphamide (CTX), and methylprednisolone (MP) on the clinical course of patients with paraneoplastic neurological syndrome (PNS) and antineuronal antibodies (Abs).Methods-Seventeen patients with paraneoplastic encephalomyelitis/sensory neuropathy (PEM/SN) with anti-Hu Abs (n=10) or cerebellar degeneration (PCD) with anti-Yo Abs (n=7) received one to nine cycles (mean 3.5) of a combination of IVIg (0.5 g/kg/day from days 1 to 5), CTX (600 mg/m2 at day 1) and MP (1g/day from day 1 to 3). The Rankin scale (RS) was used to evaluate the response. A positive response was considered as either improvement or stabilisation in patients who were still ambulatory (RS less than or equal to 3) at the onset of treatment, whereas only improvement, and not stabilisation, was considered a therapeutic benefit in bedridden patients (RS greater than or equal to 4).Results-Tolerance was good and no patient experienced grade 3/4 toxicity (World Health Organisation). Sixteen patients were evaluable for response. Of the seven patients with RS greater than or equal to 4, none improved. Of the nine patients with RS greater than or equal to 3, none improved but three (two SN and one PCD) stabilised for 4, 35, and 16 months.Conclusions-This study suggests that vigorous immunosuppressive treatment is not useful in severely disabled PNS patients with antineuronal Abs. In a minority of patients (mainly with SN) who are not severely disabled at the onset of treatment, a transient stabilisation is possible and deserves further evaluation.