Benzing T, Rump L C, Kaiser R, Peter H H
Abteilungen Medizin IV, Medizinischen Universitätsklinik und Neurologische Universitätsklinik, Freiburg.
Dtsch Med Wochenschr. 1998 Apr 17;123(16):493-6. doi: 10.1055/s-2007-1024000.
A 30-year-old previously healthy man suddenly developed double vision, unsteady gait and some difficulty in speech articulation. Within 4 weeks he had become markedly ataxic, unable to walk, stand or sit down unaided. Neurological examination indicated a severe cerebellar syndrome. There were no other abnormal findings on physical examination.
There was no pleocytosis and no oligoclonal bands in cerebrospinal fluid (CSF). A test for anti-Purkinje cell antibodies was negative in both CSF and serum. Computed tomography and nuclear magnetic imaging (NMI) of the brain were normal.
As a para- or postinfectious or paraneoplastic process was suspected. I.v. immunoglobulin and oral corticosteroids were administered, but without improvement. 13 month later, a mediastinal mass was noted on a chest radiogram. This led to the diagnosis of a stage IA Hodgkin's disease. Retrospectively the cerebellar degeneration was most likely a paraneoplastic change related to the Hodgkin's disease. However, an independent second disease cannot be excluded. While the treatment of Hodgkin's disease was successful, the neurological symptoms remained unchanged. Severe cerebellar atrophy was demonstrated on NMI.
In case of cerebellar atrophy of undetermined aetiology a paraneoplastic cause should be considered and an underlying malignant disease looked for.
一名30岁既往健康的男性突然出现复视、步态不稳及言语清晰度有些困难。在4周内,他变得明显共济失调,无法独立行走、站立或坐下。神经系统检查显示为严重的小脑综合征。体格检查无其他异常发现。
脑脊液(CSF)中无细胞增多及寡克隆带。CSF和血清中抗浦肯野细胞抗体检测均为阴性。脑部计算机断层扫描和核磁共振成像(NMI)均正常。
怀疑为感染后或肿瘤旁相关过程。给予静脉注射免疫球蛋白和口服皮质类固醇,但病情无改善。13个月后,胸部X线片发现纵隔肿块。这导致诊断为IA期霍奇金病。回顾性分析,小脑变性很可能是与霍奇金病相关的肿瘤旁改变。然而,不能排除独立的第二种疾病。虽然霍奇金病的治疗成功,但神经症状无变化。NMI显示严重的小脑萎缩。
对于病因不明的小脑萎缩病例,应考虑肿瘤旁病因并寻找潜在的恶性疾病。