Motor Neuron dysfunction in frontotemporal dementia
Motor Neuron dysfunction in frontotemporal dementia
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DOI：
10.1093/brain/awr195
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发表时间：
2011-09-01
期刊：
影响因子：
14.5
通讯作者：
Hodges, John R.
中科院分区：
文献类型：
--
作者：
Burrell, James R.;Kiernan, Matthew C.;Hodges, John R.
额颞叶痴呆和运动神经元病具有共同的临床、遗传和病理特征。运动神经元疾病在部分额颞叶痴呆患者中发生，但额颞叶痴呆患者运动系统功能障碍的发生率、严重程度和功能意义尚未确定。神经生理学生物标记物已被开发用于证明运动系统功能障碍，包括：皮质内短间期抑制，皮质脊髓运动神经元功能障碍的标志，以及神经生理指数，运动神经元功能低下的标志。本研究对108名参与者进行了详细的临床和神经生理学评估，其中包括40名连续的额颞叶痴呆患者，42名年龄和性别匹配的运动神经元病患者和26名对照组。40例额颞叶痴呆患者中，12.5%伴有运动神经元病。另有27.3%的额颞叶痴呆患者有轻微运动系统功能障碍的临床证据，如偶尔出现束状肌、轻度消瘦或虚弱。额颞叶痴呆患者运动系统功能的生物标志物异常。额颞叶痴呆组平均短程皮质内抑制(4.3±-1.7%)较对照组(9.1+/-1.1%)降低(P<；0.05)。在进行性非流利性失语症亚组中，皮质内短时间间隔抑制作用尤其减少，但在行为变异型额颞叶痴呆和语义性痴呆患者中，这是正常的。与对照组(1.9，P<；0.001)相比，额颞叶痴呆组的神经生理指数(1.1，P<；0.05)降低，表明运动神经元功能障碍程度较低，但与运动神经元病(0.7，P<；0.05)相比仍相对保存。额颞叶痴呆的运动系统功能障碍可能是由于初级运动皮质受累，继发于脑干和脊髓前角的低运动神经元变性所致。
Frontotemporal dementia and motor neuron disease share clinical, genetic and pathological characteristics. Motor neuron disease develops in a proportion of patients with frontotemporal dementia, but the incidence, severity and functional significance of motor system dysfunction in patients with frontotemporal dementia has not been determined. Neurophysiological biomarkers have been developed to document motor system dysfunction including: short-interval intracortical inhibition, a marker of corticospinal motor neuron dysfunction and the neurophysiological index, a marker of lower motor neuron dysfunction. The present study performed detailed clinical and neurophysiological assessments on 108 participants including 40 consecutive patients with frontotemporal dementia, 42 age- and gender-matched patients with motor neuron disease and 26 control subjects. Of the 40 patients with frontotemporal dementia, 12.5% had concomitant motor neuron disease. A further 27.3% of the patients with frontotemporal dementia had clinical evidence of minor motor system dysfunction such as occasional fasciculations, mild wasting or weakness. Biomarkers of motor system function were abnormal in frontotemporal dementia. Average short-interval intracortical inhibition was reduced in frontotemporal dementia (4.3 +/- 1.7%) compared with controls (9.1 +/- 1.1%, P < 0.05). Short-interval intracortical inhibition was particularly reduced in the progressive non-fluent aphasia subgroup, but was normal in patients with behavioural variant frontotemporal dementia and semantic dementia. The neurophysiological index was reduced in frontotemporal dementia (1.1) compared with controls (1.9, P < 0.001), indicating a degree of lower motor neuron dysfunction, although remained relatively preserved when compared with motor neuron disease (0.7, P < 0.05). Motor system dysfunction in frontotemporal dementia may result from pathological involvement of the primary motor cortex, with secondary degeneration of lower motor neurons in the brainstem and anterior horn of the spinal cord.