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1 disease involving dying-back degeneration of upper motor neurons.
2 ease that is thought to predominantly affect upper motor neurons.
3 ly held view of the motor cortex as just an "upper motor neuron."
5 d ATP production in demyelinated segments of upper motor neuron axons impacts ion homeostasis, induce
6 llosal body, and linked strongly to clinical upper motor neuron burden, but also to limb disability s
7 r sensory abnormalities and/or a significant upper-motor-neuron component, and there is often an over
8 ese include loss of lower (ventral horn) and upper motor neurons (corticospinal motor neurons in laye
9 magnetic stimulation has been used to detect upper motor neuron damage and to explore cortical excita
10 ipheral neurodegeneration, including ataxia, upper-motor-neuron damage, peripheral neuropathy, hearin
11 from families affected by diabetes, ataxia, upper-motor-neuron damage, peripheral neuropathy, or hea
12 weakness in ALS2(-/-) mice, consistent with upper motor neuron defects that lead to spasticity in hu
13 ically and clinically heterogeneous group of upper-motor-neuron degenerative diseases characterized b
14 n developmental principles of both lower and upper motor neuron development that have led to specific
15 mans shows that deficiency in ALS2 causes an upper motor neuron disease that in humans closely resemb
20 (HR, 1.48; 95% CI, 0.58-3.75; P = .41), and upper motor neuron dominant ALS (HR, 0.12; 95% CI, 0.02-
22 ssociated with bulbar onset and dysfunction, upper motor neuron dysfunction, cognitive impairment, de
25 ding one family with exclusively adult-onset upper motor neuron features, consistent with a diagnosis
26 ysfunction and thus an important role of the upper motor neuron in this animal model of ALS and perha
27 cospinal/corticobulbar motor neurons (CSMN; "upper motor neurons") in cerebral cortex, and spinal/bul
29 n age, 57.3 years) with clinical evidence of upper motor neuron involvement and 10 healthy control su
30 Elevation in NFL levels in patients with upper motor neuron involvement and FTD might reflect the
31 imaging spectroscopy can be used to identify upper motor neuron involvement and predict disease durat
32 ential as a quantitative test of subclinical upper motor neuron involvement in motor neuron disease.
38 It is often difficult to identify signs of upper motor neuron lesion in the limbs of patients with
40 progressive degeneration of lower (LMN) and upper motor neurons, likely due to the same unknown toxi
41 endpoint, we wanted to establish whether the upper motor neuron might still play a critical role in d
42 This relative lack of knowledge regarding upper motor neuron pathology in these ALS model mice has
43 o also correlated with both the ALSFRS-R and upper motor neuron scores (r=0.50 and 0.54, respectively
44 gnized limited change in individual clinical upper motor neuron scores, despite advancing disability.
45 abnormalities, including a constellation of upper motor neuron signs (n = 19), ataxia (n = 22), and
57 l rating scale (ALSFRS-R score), whereas the upper motor neuron (UMN) score correlated with widesprea
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