Hu Feng-Qi, Shang Fu-Rong, Liu Jing-Jing, Yuan Hai
Department of Nephrology.
Department of Neurology, Xiangyang Central Hospital, Affiliated Hospital of Hubei University of Arts and Science, Xiangyang, Hubei, China.
Medicine (Baltimore). 2020 Aug 14;99(33):e21760. doi: 10.1097/MD.0000000000021760.
Paraneoplastic cerebellar degeneration (PCD) is a rare neurodegenerative syndrome associated with antibodies targeting the Purkinje cells of the cerebellum. Most cases of anti-Yo-associated PCD occur in females, with <20 cases reported in males. Herein, we report a male patient with anti-Yo-associated PCD who was treated with plasma exchange and achieved a favorable outcome.
A 64-year-old man presented with progressive ataxia, gait instability, and dysuria. Electroencephalography, electromyography, brain and spinal neuroimaging, and routine laboratory examinations were all normal. The anti-neuronal antibody Anti-Yo was detected in the serum but not in the cerebrospinal fluid (CSF).
The patient was diagnosed with definite anti-Yo-associated PCD based on the clinical manifestations, anti-Yo was detected in the serum and response to treatment.
At beginning, the patient was treated with dexamethasone (10 mg/day for 10 days). Then, plasma exchange was performed.
After treated with dexamethasone, no clinical improvement was noted in this patient. In the following month, his condition deteriorated. However, after two courses of plasma exchange, neurological examination showed marked improvement in gait. After four courses of plasma exchange, the patient could walk independently, the Romberg test was negative, and anti-Yo antibodies were undetectable. At the 6-month follow-up, the patients' symptoms were relieved, and tests for anti-Yo antibodies remained negative.
Treatment with plasma exchange for anti-Yo-associated male PCD patients without a concomitant tumor are recommend and need more studies.
副肿瘤性小脑变性（PCD）是一种罕见的神经退行性综合征，与靶向小脑浦肯野细胞的抗体有关。大多数抗Yo相关的PCD病例发生在女性中，男性报告病例少于20例。在此，我们报告一名患有抗Yo相关PCD的男性患者，该患者接受了血浆置换治疗并取得了良好的效果。
一名64岁男性出现进行性共济失调、步态不稳和排尿困难。脑电图、肌电图、脑和脊髓神经影像学检查以及常规实验室检查均正常。血清中检测到抗神经元抗体抗Yo，但脑脊液（CSF）中未检测到。
根据临床表现、血清中检测到抗Yo以及对治疗的反应，该患者被诊断为确诊的抗Yo相关PCD。
开始时，患者接受地塞米松治疗（10毫克/天，共10天）。然后进行了血浆置换。
用地塞米松治疗后，该患者未出现临床改善。在接下来的一个月里，他的病情恶化。然而，经过两个疗程的血浆置换后，神经检查显示步态有明显改善。经过四个疗程的血浆置换后，患者能够独立行走，闭目难立试验为阴性，且未检测到抗Yo抗体。在6个月的随访中，患者症状缓解，抗Yo抗体检测仍为阴性。
对于无伴发肿瘤的抗Yo相关男性PCD患者，建议采用血浆置换治疗，且需要更多研究。
