Eleven patients with acquired cerebellar degeneration (10 of whom had paraneoplastic cerebellar degeneration [PCD]) were evaluated using neuropsychological tests and 18F-fluorodeoxyglucose/positron emission tomography to (1) quantify motor, cognitive, and metabolic abnormalities; (2) determine if characteristic alterations in the regional cerebral metabolic rate for glucose (rCMRGlc) are associated with PCD; and (3) correlate behavioral and metabolic measures of disease severity. Eighteen volunteer subjects served as normal controls. Although some PCD neuropsychological test scores were abnormal, these results could not, in general, be dissociated from the effects of dysarthria and ataxia. rCMRGlc was reduced in patients with PCD (versus normal control subjects) in all regions except the brainstem. Analysis of patient and control rCMRGlc data using a mathematical model of regional metabolic interactions revealed two metabolic pattern descriptors, SSF1 and SSF2, which distinguished patients with PCD from normal control subjects; SSF2, which described a metabolic coupling between cerebellum, cuneus, and posterior temporal, lateral frontal, and paracentral cortex, correlated with quantitative indices of cerebellar dysfunction. Our inability to document substantial intellectual impairment in 7 of 10 patients with PCD contrasts with the 50% incidence of dementia in PCD reported by previous investigators. Widespread reductions in PCD rCMRGlc may result from the loss of cerebellar efferents to thalamus and forebrain structures, a "reverse cerebellar diaschisis."

译文

使用神经心理学测试和18f-氟脱氧葡萄糖/正电子发射断层扫描对11例获得性小脑变性患者 (其中10例患有副肿瘤性小脑变性 [PCD]) 进行评估,以 (1) 量化运动,认知和代谢异常; (2) 确定葡萄糖区域脑代谢率 (rCMRGlc) 的特征性改变是否与PCD相关; (3) 将疾病严重程度的行为和代谢指标相关联。18名志愿者作为正常对照。尽管某些PCD神经心理学测试分数异常,但这些结果通常无法与构音障碍和共济失调的影响分离。除脑干外,所有区域的PCD患者 (与正常对照组相比) 的rCMRGlc均降低。使用区域代谢相互作用的数学模型对患者和对照rCMRGlc数据进行分析,揭示了两个代谢模式描述符SSF1和SSF2,将PCD患者与正常对照组区分开来; SSF2,描述了小脑,楔形和后颞,外侧额叶和中央旁皮质之间的代谢耦合,与小脑功能障碍的定量指标相关。我们无法证明10例PCD患者中有7例存在实质性的智力障碍,这与先前研究者报告的PCD中痴呆的50% 发生率形成了对比。PCD rCMRGlc的广泛减少可能是由于小脑传出到丘脑和前脑结构的丧失,即 “反向小脑困难”。

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