Meloni Carlo, Iani Cesare, Dominijanni Sara, Arciprete Flavio, Cipriani Silvia, Caramiello Maria Stella, Tozzo Carmela, Lombardo Pietrina Angela, Tatangelo Paola, Cecilia Annalisa, Straccialano Emilia, Casciani Carlo Umberto
Nephrology and Dialysis Unit, S. Eugenio Hospital, Rome Italy.
Ther Apher Dial. 2004 Dec;8(6):500-2. doi: 10.1111/j.1774-9987.2004.00198.x.
A 71-year-old-woman was admitted to the S. Eugenio Hospital for a history of progressively impaired standing and gait. Anamnesis revealed systemic hypertension, gastric polyposis and juvenile pulmonary tuberculosis. Neurological examination showed a severe truncal and gait ataxia, without any sensory-motor impairment. Motor and somato-sensory evoked potentials were normal. Brain Magnetic Resonance Imaging (MRI) showed minimal signs of chronic ischemia only at a supratentorial level. Cerebral Single Photon Emission Computed Tomography, spinal MRI, total body computed tomography, Esophagogastroduodenoscopy, and finally total body Positron Emission Tomography resulted negative for neoplasms. Oncological serum markers were negative. Serum antibody against Purkinje's cells (Anti-Yo) was detected and titer was 1:80, while normally it should be undetectable. Other autoantibodies (Anti-Hu, Anti-Ri) were undetectable. Two sessions of plasma exchange (PE) were thus performed, leading to a rapid, marked and durable improvement of standing and gait and to a reduction of the autoantibody, which became undetectable. No serious adverse effect was noted. Although no definite therapy for autoimmune cerebellar ataxia has been established, PE should be considered as one of the main therapeutic choices.
一名71岁女性因进行性站立和步态障碍病史入住圣欧金尼奥医院。既往史显示有系统性高血压、胃息肉和青少年肺结核。神经系统检查发现严重的躯干和步态共济失调,无任何感觉运动障碍。运动和躯体感觉诱发电位正常。脑磁共振成像(MRI)仅在幕上水平显示出轻微的慢性缺血迹象。脑单光子发射计算机断层扫描、脊髓MRI、全身计算机断层扫描、食管胃十二指肠镜检查,最后全身正电子发射断层扫描均未发现肿瘤。肿瘤血清标志物为阴性。检测到抗浦肯野细胞血清抗体(抗Yo),滴度为1:80,而正常情况下应检测不到。其他自身抗体(抗Hu、抗Ri)未检测到。因此进行了两次血浆置换(PE),使站立和步态迅速、显著且持久地改善,自身抗体减少且变为检测不到。未观察到严重不良反应。尽管尚未确立自身免疫性小脑共济失调的确切治疗方法,但血浆置换应被视为主要治疗选择之一。