Gilman S, Junck L, Markel D S, Koeppe R A, Kluin K J
Department of Neurology, University of Michigan, Ann Arbor 48109-0316.
Ann Neurol. 1990 Dec;28(6):750-7. doi: 10.1002/ana.410280605.
Local cerebral metabolic rate for glucose was studied with 18F-2-fluoro-2-deoxy-D-glucose and positron emission tomography (PET) in 22 patients with Friedreich's ataxia and 23 age-matched normal control subjects. The diagnosis of Friedreich's ataxia was established by the history and physical findings and by excluding other diseases through laboratory investigations. PET studies revealed a statistically significant widespread increase of local cerebral metabolic rate for glucose in the brains of patients with Friedreich's ataxia who were still ambulatory, in comparison with normal control subjects. Nonambulatory patients with Friedreich's ataxia, in comparison with normal control subjects, had significantly increased local cerebral metabolic rates for glucose in the caudate and lenticular nuclei, but not in the other structures studied. The rate was significantly greater in ambulatory patients with Friedreich's ataxia than in nonambulatory patients in all structures studied except the caudate and lenticular nuclei. The data suggest that early in the course of Friedreich's ataxia, the local cerebral metabolic rate for glucose is increased extensively in the central nervous system, and as the disease progresses, it decreases in a regionally specific manner.
采用18F-2-氟-2-脱氧-D-葡萄糖和正电子发射断层扫描（PET）技术，对22例弗里德赖希共济失调患者及23名年龄匹配的正常对照者进行了局部脑葡萄糖代谢率研究。弗里德赖希共济失调的诊断依据病史、体格检查结果，并通过实验室检查排除其他疾病来确定。PET研究显示，与正常对照者相比，仍能行走的弗里德赖希共济失调患者大脑局部脑葡萄糖代谢率在统计学上有显著的广泛升高。与正常对照者相比，不能行走的弗里德赖希共济失调患者尾状核和豆状核的局部脑葡萄糖代谢率显著升高，但在所研究的其他结构中未出现这种情况。除尾状核和豆状核外，在所有研究结构中，能行走的弗里德赖希共济失调患者的代谢率显著高于不能行走的患者。数据表明，在弗里德赖希共济失调病程早期，中枢神经系统局部脑葡萄糖代谢率广泛升高，随着疾病进展，代谢率以区域特异性方式降低。
