Friedreich共济失调患者皮质区的亚兴奋性:一项TMS研究

作     者：Brighina F. Scalia S. Gennuso M. B. Fierro 史利利 

作者机构：Via G. La Loggia 1 90129 Palermo Italy 

出 版 物：《世界核心医学期刊文摘（神经病学分册）》 (Digest of the World Core Medical Journals:Clinical Neurology)

年 卷 期：2005年第1卷第11期

页      面：48-49页

学科分类：1002[医学-临床医学] 100204[医学-神经病学] 10[医学] 

主　　题：共济失调 TMS 皮质区 光幻视 运动阈值 大脑皮质 神经生理学 拇短展肌 活化作用 磁刺激 

摘      要：The aim of the study was to explore excitability of a motor and a non-motor (visual) area in patients affected by Friedreich ataxia and to correlate neurophysiological data with clinical parameters. Seven patients (3M/4F) and ten healthy controls (5M/5F) participated in the study. The hot-spot for activation of right abductor pollicis brevis was checked by means of a figure-of-eight coil and the motor threshold (MT) on this point was recorded. The phosphene threshold (PT) was measured by means of a focal coil over the occipital cortex as the lower intensity of magnetic stimulation able to induce the perception of phosphenes. The patients showed a significantly higher mean PT (p .03) and MT values (p .001) than controls. In all but one patient unable to perceive phosphenes (42%vs. 50%of controls), TMS at 100%intensity did not elicit motor response at rest. The difference in percentage of patients (57.1%) and controls (100%) with motor responses was nearly significant. The size of GAA1 expansion showed significant correlations with PT and MT values. The results of our study showed that FA patients had reduced cortical activation, involving both the motor and the visual cortex. The cortical involvement in these patients seems to be mainly genetically determined. The study provides the first evidence of cortical dysfunction in patients with genetically defined Friedreich ataxia.