Gungor Serdal, Kilic Betul, Arslan Mujgan, Ozgen Unsal
Faculty of Medicine, Department of Pediatric Neurology, Inonu University, Malatya, Turkey.
Faculty of Medicine, Department of Pediatric Hematology, Inonu University, Malatya, Turkey.
Childs Nerv Syst. 2017 Mar;33(3):509-512. doi: 10.1007/s00381-016-3284-y. Epub 2016 Oct 29.
Paraneoplastic cerebellar degeneration (PCD) can occur severely and appear as subacute cerebellar syndrome. PCD may be associated with small cell lung cancer, adenocarcinoma, breast cancer, ovarian carcinoma, and Hodgkin's lymphoma. An 11-year-old male was admitted with acute cerebellar ataxia, dysarthria, and diplopia. Mediastinal conglomerated lymph nodes were depicted in a chest computed tomography (CT) examination, and diagnosis of stage IV Hodgkin's lymphoma was obtained after a lymph node biopsy. The antibodies against Purkinje cells (anti-Tr antibody) were positive immunohistochemically. Thus, paraneoplastic cerebellar degeneration depending on Hodgkin's disease was diagnosed. Despite the completion of chemotherapy, neurological recovery was not observed in the patient and plasmapheresis with immunoadsorption, and intravenous immunoglobulin (IVIG) was performed. Truncal ataxia, gait disturbance, and tremors decreased. Consequently, we thought that plasmapheresis with the immunoadsorption method and IVIG therapy might be a treatment option for cerebellar ataxia caused by a mechanism of immune ancestry.
副肿瘤性小脑变性(PCD)可严重发生并表现为亚急性小脑综合征。PCD可能与小细胞肺癌、腺癌、乳腺癌、卵巢癌和霍奇金淋巴瘤有关。一名11岁男性因急性小脑共济失调、构音障碍和复视而入院。胸部计算机断层扫描(CT)检查显示纵隔有融合的淋巴结,淋巴结活检后确诊为IV期霍奇金淋巴瘤。免疫组化显示抗浦肯野细胞抗体(抗Tr抗体)呈阳性。因此,诊断为依赖霍奇金病的副肿瘤性小脑变性。尽管完成了化疗,但患者未观察到神经功能恢复,遂进行了免疫吸附血浆置换和静脉注射免疫球蛋白(IVIG)治疗。躯干共济失调、步态障碍和震颤有所减轻。因此,我们认为免疫吸附法血浆置换和IVIG治疗可能是一种针对免疫源性机制引起的小脑共济失调的治疗选择。