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排序方式: 共有1710条查询结果,搜索用时 15 毫秒
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Emanuele D'amico MD Meredith Pasmantier BA Yei‐won Lee MA Louis Weimer MD Hiroshi Mitsumoto MD DSc 《Muscle & nerve》2013,47(1):28-32
Introduction: PLS is defined as pure upper motor neuron disease/dysfunction (PUMND) beyond 48 months after symptom onset. We know little about its early stages, but such knowledge would help to identify the mechanisms underlying PLS and ALS and determine why PLS patients seem to be protected against lower MND (LMND). Methods: We reviewed 622 MND cases during a 4‐year period and identified 34 patients with PUMND (5.4%). Results: Among 23 cases with follow‐up data/electromyograms (EMGs; 2 had only 1 EMG), 13 (57%) remained classified as PUMND, and 8 (35%) developed LMND (mean, 51.4 months after onset). Of these 8, LMND developed in 3 after 48 months from symptom onset. Patients with PUMND and LMND were more functionally impaired (P = 0.02). Separately, we identified 5 patients with PUMND who developed LMND long after 48 months (range, 50–127 months). Conclusions: PLS belongs to the ALS spectrum, and perhaps all cases eventually develop LMND. Muscle Nerve, 2013 相似文献
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Furio Zucco MD Roberta Ciampichini MSc Angelo Lavano MD Amedeo Costantini MD Marisa De Rose MD Paolo Poli MD Gianpaolo Fortini MD Laura Demartini MD Enrico De Simone MD Valentino Menardo MD Piero Cisotto MD Mario Meglio MD Luciana Scalone PhD Lorenzo G. Mantovani DSc 《Neuromodulation》2015,18(4):266-276
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Neil G. Simon MBBS Martin R. Turner PhD Steve Vucic PhD Ammar Al‐Chalabi PhD Jeremy Shefner MD PhD Catherine Lomen‐Hoerth MD PhD Matthew C. Kiernan DSc 《Annals of neurology》2014,76(5):643-657
Amyotrophic lateral sclerosis (ALS) exhibits characteristic variability of onset and rate of disease progression, with inherent clinical heterogeneity making disease quantitation difficult. Recent advances in understanding pathogenic mechanisms linked to the development of ALS impose an increasing need to develop strategies to predict and more objectively measure disease progression. This review explores phenotypic and genetic determinants of disease progression in ALS, and examines established and evolving biomarkers that may contribute to robust measurement in longitudinal clinical studies. With targeted neuroprotective strategies on the horizon, developing efficiencies in clinical trial design may facilitate timely entry of novel treatments into the clinic. Ann Neurol 2014;76:643–657 相似文献
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Zhao JH Brunner EJ Kumari M Singh-Manoux A Hawe E Talmud PJ Marmot MG Humphries SE 《Social psychiatry and psychiatric epidemiology》2005,40(7):557-563
Objective The aim of this study was to investigate the association of the common apolipoprotein E gene (APOE) variants with cognitive function and cognitive decline in adult mid-life and explore the possibility that APOE genotype mediates the link between socioeconomic status (SES) and cognitive function.Methods Data on cognitive function, as measured by five cognitive tests, together with APOE genotype were obtained in an occupational cohort (the Whitehall II study) of 6,004 participants aged 44–69 years (1997–1999). Cognitive change was examined in 2,717 participants who had cognitive function measured at baseline (1991–1993).Results SES based on civil service employment grade was strongly related to cognitive function. There was no association between APOE genotype and employment grade. In women, participants with APOE-ɛ4 had a lower memory score (p<0.05), but the result was sensitive to data from a small number of individuals. A marginal cross-sectional difference in the semantic fluency score was found (p=0.07), and there was a relative decline at follow-up (p<0.001, net change=−1.19; 95% CI, −1.90 to −0.49) in those with APOE-ɛ4 genotypes.Conclusions APOE-ɛ4 has little influence on cognitive decline in mid-life, whereas SES is a strong determinant, although APOE genotype may emerge as an important factor in cognitive function in later life. 相似文献
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