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抗 Yo 相关副肿瘤性小脑变性的恶化。

Deterioration of anti-Yo-associated paraneoplastic cerebellar degeneration.

机构信息

Krankenanstalt Rudolfstiftung, Vienna, Austria.

出版信息

J Neurol Sci. 2011 Sep 15;308(1-2):139-41. doi: 10.1016/j.jns.2011.06.051. Epub 2011 Jul 16.

Abstract

OBJECTIVES

Usually, the course of paraneoplastic cerebellar degeneration(PCD) is stable or progresses only slowly. Sudden marked progression after several years, as in the following case, has not been reported.

CASE REPORT

After a 57 year old female had developed diplopia, cerebellar signs, upper-limb weakness, and bilateral stocking-type hypoesthesia, and Yo-antibodies were positive, PCD and sensory polyneuropathy were diagnosed. Upon further diagnostic work-up ovarian cancer FIGO-IIC was detected and treated with ovarectomy, hysterectomy, omentectomy, and chemotherapy. Within the following years she experienced several relapses and developed multifocal metastasis requiring surgery, various chemotherapies, thermocoagulations, and radiotherapy. During the first years, PCD showed only minor progression. After 5 years, however, asymmetric ataxia and dysarthria acutely deteriorated such that she became severely handicapped and dependent on the help of others. Several cycles of immunoglobulines were ineffective and she died at age 64 years in a severely disabled state without recovery of the PCD.

CONCLUSIONS

PCD, which usually progresses only slowly, can acutely deteriorate without recovery.

摘要

目的

副肿瘤性小脑变性(PCD)的病程通常稳定或仅缓慢进展。以下病例报告中,数年后突然出现明显进展的情况尚未见报道。

病例报告

一位 57 岁女性出现复视、小脑征、上肢无力和双侧袜套样感觉减退,且 Yo 抗体阳性,诊断为 PCD 和感觉性多神经病。进一步检查发现卵巢癌FIGO-IIC,并接受卵巢切除术、子宫切除术、网膜切除术和化疗。在随后的几年中,她经历了多次复发,并出现多处转移,需要手术、多种化疗、热凝固和放疗。在最初的几年中,PCD 仅出现轻微进展。然而,5 年后,非对称的共济失调和构音障碍急性恶化,导致她严重残疾,依赖他人的帮助。几轮免疫球蛋白治疗无效,她在 64 岁时死于严重残疾状态,PCD 未恢复。

结论

通常进展缓慢的 PCD 可突然恶化而无法恢复。

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