Dou Jingjing, Yue Xiaotong, Ren Haitao, Wang Chunjuan, Guo Shougang
Department of Neurology, Shandong Provincial Hospital, Shandong University, Jinan, Shandong, China.
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Front Immunol. 2024 Dec 16;15:1476397. doi: 10.3389/fimmu.2024.1476397. eCollection 2024.
Paraneoplastic cerebellar degeneration (PCD) with anti-Yo antibodies represents a rare immune-mediated paraneoplastic neurological syndrome. Its diagnosis and management remain clinically challenging. Here, we present a case of PCD with confirmed anti-Yo antibodies, validated through anti-cerebellar degeneration protein 2 (CDR2) and anti-CDR2-like antibodies detection, which demonstrated a favorable response to ofatumumab therapy. The patient initially manifested with dizziness, nystagmus, dysarthria, and ataxia. Initial testing revealed weakly positive anti-Yo antibodies, accompanied by positive serum tissue-based assay result for cerebellum. Following one course of intravenous immunoglobulin and methylprednisolone pulse therapy, improvement of the patient's dizziness was observed. Oral prednisone was prescribed for maintenance therapy. However, after discharge, the patient experienced progressive deterioration of symptoms, including worsening dizziness, dysarthria, and limb ataxia. Upon readmission to our hospital, further immunological testing confirmed the presence of anti-CDR2 and anti-CDR2-like antibodies. When a second course of methylprednisolone pulse therapy proved ineffective, treatment was switched to ofatumumab. After two doses, the patient achieved partial symptomatic relief.
伴有抗Yo抗体的副肿瘤性小脑变性(PCD)是一种罕见的免疫介导的副肿瘤性神经综合征。其诊断和治疗在临床上仍然具有挑战性。在此,我们报告一例经抗小脑变性蛋白2(CDR2)和抗CDR2样抗体检测证实伴有抗Yo抗体的PCD病例,该病例对奥法木单抗治疗表现出良好反应。患者最初表现为头晕、眼球震颤、构音障碍和共济失调。初始检测显示抗Yo抗体弱阳性,同时小脑血清组织检测结果呈阳性。经过一个疗程的静脉注射免疫球蛋白和甲泼尼龙冲击治疗后,患者头晕症状有所改善。随后给予口服泼尼松进行维持治疗。然而,出院后患者症状逐渐恶化,包括头晕、构音障碍和肢体共济失调加重。再次入院后,进一步的免疫学检测证实存在抗CDR2和抗CDR2样抗体。当第二疗程的甲泼尼龙冲击治疗无效后,治疗改为奥法木单抗。两剂治疗后,患者症状部分缓解。