CORTICAL FUNCTION IN PROGRESSIVE LOWER MOTOR-NEURON DISORDERS AND AMYOTROPHIC-LATERAL-SCLEROSIS - A COMPARATIVE PET STUDY
CORTICAL FUNCTION IN PROGRESSIVE LOWER MOTOR-NEURON DISORDERS AND AMYOTROPHIC-LATERAL-SCLEROSIS - A COMPARATIVE PET STUDY
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DOI：
10.1212/wnl.44.6.1101
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发表时间：
1994-06-01
期刊：
影响因子：
9.9
通讯作者：
LEIGH, PN
中科院分区：
文献类型：
--
作者：
KEW, JJM;BROOKS, DJ;LEIGH, PN
目的：比较进行性下运动神经元变性（LMND）和肌萎缩侧索硬化（ALS）患者静息和肢体运动时的脑功能。方法：采用PET技术测定5例进行性LMND患者、6例运动功能障碍程度相似的典型ALS患者和6例年龄匹配的对照组的局部脑血流量（rCBF）。结果：ALS患者静息时初级感觉运动皮层（SMC）的rCBF显著（p < 0.001）低于对照组和LMND患者。LMND患者和对照组之间静息时rCBF无显著差异。在操纵杆运动过程中，ALS患者双侧SMC的手/臂区域、对侧SMC的面部区域、双侧第二躯体感觉（SII）皮层以及对侧运动前区和辅助运动皮层的rCBF增加显著（p < 0.001）大于对照或LMND患者。LMND患者表现出显着（p < 0.001）更大的rCBF增加比对照组和ALS患者仅在前岛叶cortex bilates.Conclusions：发现减少rCBF在休息，连同异常的双边激活和改变躯体活动在运动过程中，在感觉运动皮层的ALS，但不是LMND患者表明，这些异常反映了锥体神经元的损失。LMND和ALS患者在肢体运动过程中外侧裂周区（岛叶和SII皮质）的异常激活表明，这些可能是非特异性地响应肢体无力而招募的辅助感觉运动区。
Objective: To compare cortical function at rest and during limb movement in patients with progressive lower motor neuron degeneration (LMND) and amyotrophic lateral sclerosis (ALS).Methods: PET was used to measure regional cerebral blood flow (rCBF) in five patients with progressive LMND, six patients with classic ALS with a similar degree of motor impairment, and six age-matched control subjects; measurements were taken in the resting state and while subjects moved a joystick with their right hand.Results: rCBF at rest in the primary sensorimotor cortex (SMC) was significantly (p < 0.001) lower in ALS patients than in control subjects or LMND patients. rCBF at rest did not differ significantly between LMND patients and controls. During joystick movement, ALS patients showed significantly (p < 0.001) greater rCBF increases than controls or LMND patients in the hand/arm area of the SMC bilaterally, the face area of the contralateral SMC, the second somatic sensory (SII) cortex bilaterally, and the contralateral premotor and supplementary motor cortices. LMND patients showed significantly (p < 0.001) greater rCBF increases than controls and ALS patients only in the anterior insular cortex bilaterally.Conclusions: The finding of reduced rCBF at rest, together with abnormal bilateral activation and altered somatotopy during movement, in the sensorimotor cortex of ALS but not LMND patients suggests that these abnormalities reflect loss of pyramidal neurons. Abnormal activation of perisylvian areas (insular and SII cortices) during limb movement in both LMND and ALS patients suggests that these may be accessory sensorimotor areas that are recruited nonspecifically in response to Limb weakness.