Abdulaziz Ammar Taha Abdullah, Yu Xiao Qing, Zhang Le, Jiang Xin Yue, Zhou Dong, Li Jin Mei
West China Hospital, Sichuan University, Chengdu, P.R. China.
Medicine (Baltimore). 2018 Jun;97(24):e10717. doi: 10.1097/MD.0000000000010717.
Paraneoplastic cerebellar degeneration (PCD) is an immune-mediated neurological deficit affecting the cerebellum. Anti-Yo antibody positive PCD is a rare occurrence most likely associated with gynecologic or breast malignancies. The identification of the underlying tumor is a diagnostic challenge in many of these patients.
We present a 68-year-old woman with acute symptoms of PCD as a first sign of underlying occult malignancy. Further investigation revealed a positive anti-Yo antibody. Although brain magnetic resonance imaging (MRI) was unremarkable, positron emission tomography (PET)/computed tomography (CT) revealed intense hypermetabolism of cerebellum and diffused hypometabolism in the rest of brain. On 1-year follow-up, despite the primary malignancy is still unknown, her symptoms improved significantly after immunotherapy.
Paraneoplastic cerebellar degeneration.
The patient was given IV methylprednisolone 500 mg once a day for 5 consecutive days, followed by oral prednisone 60 mg once a day for 3 months.
The patient's symptoms were gradually improved during the hospitalization period. On one year follow up, she was able to walk independently and perform some simple tasks.
Cerebellar hypermetabolism in PCD suspected patients may help confirming the diagnosis in an earlier stage and may predict a better outcome after immunotherapy.
副肿瘤性小脑变性(PCD)是一种影响小脑的免疫介导的神经功能缺损。抗Yo抗体阳性的PCD较为罕见,最可能与妇科或乳腺恶性肿瘤相关。在许多这类患者中,识别潜在肿瘤是一项诊断挑战。
我们报告一名68岁女性,以PCD的急性症状作为潜在隐匿性恶性肿瘤的首发体征。进一步检查发现抗Yo抗体呈阳性。尽管脑部磁共振成像(MRI)无明显异常,但正电子发射断层扫描(PET)/计算机断层扫描(CT)显示小脑代谢极度活跃,而脑部其他区域代谢弥漫性减低。在1年的随访中,尽管原发性恶性肿瘤仍不明,但免疫治疗后她的症状显著改善。
副肿瘤性小脑变性。
患者接受静脉注射甲泼尼龙500毫克,每日1次,连续5天,随后口服泼尼松60毫克,每日1次,共3个月。
患者症状在住院期间逐渐改善。随访1年时,她能够独立行走并完成一些简单任务。
疑似PCD患者的小脑代谢活跃可能有助于早期确诊,并可能预示免疫治疗后有更好的预后。