Canosa Antonio, Moglia Cristina, Manera Umberto, Vasta Rosario, Torrieri Maria Claudia, Arena Vincenzo, D'Ovidio Fabrizio, Palumbo Francesca, Zucchetti Jean Pierre, Iazzolino Barbara, Peotta Laura, Calvo Andrea, Pagani Marco, Chiò Adriano
"Rita Levi Montalcini" Department of Neuroscience, University of Turin, ALS Centre, Turin, Italy
SC Neurologia 1U, Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino, Turin, Italy.
J Neurol Neurosurg Psychiatry. 2020 Nov 23. doi: 10.1136/jnnp-2020-323876.
To identify the metabolic changes related to the various levels of cognitive deficits in amyotrophic lateral sclerosis (ALS) using F-2-fluoro-2-deoxy-D-glucose positron emission tomography (F-FDG-PET) imaging.
274 ALS patients underwent neuropsychological assessment and brain F-FDG-PET at diagnosis. According to the criteria published in 2017, cognitive status was classified as ALS with normal cognition (ALS-Cn, n=132), ALS with behavioural impairment (ALS-Bi, n=66), ALS with cognitive impairment (ALS-Ci, n=30), ALS with cognitive and behavioural impairment (ALS-Cbi, n=26), ALS with frontotemporal dementia (ALS-FTD, n=20). We compared each group displaying some degree of cognitive and/or behavioural impairment to ALS-Cn patients, including age at PET, sex and ALS Functional Rating Scale-Revised as covariates.
We identified frontal lobe relative hypometabolism in cognitively impaired patients that resulted more extensive and significant across the continuum from ALS-Ci, through ALS-Cbi, to ALS-FTD. ALS-FTD patients also showed cerebellar relative hypermetabolism. ALS-Bi patients did not show any difference compared with ALS-Cn.
These data support the concept that patients with cognitive impairment have a more widespread neurodegenerative process compared with patients with a pure motor disease: the more severe the cognitive impairment, the more diffuse the metabolic changes. Otherwise, metabolic changes related to pure behavioural impairment need further characterisation.
使用F-2-氟-2-脱氧-D-葡萄糖正电子发射断层扫描（F-FDG-PET）成像来识别与肌萎缩侧索硬化症（ALS）不同程度认知缺陷相关的代谢变化。
274例ALS患者在诊断时接受了神经心理学评估和脑部F-FDG-PET检查。根据2017年发表的标准，认知状态分为认知正常的ALS（ALS-Cn，n = 132）、有行为障碍的ALS（ALS-Bi，n = 66）、有认知障碍的ALS（ALS-Ci，n = 30）、有认知和行为障碍的ALS（ALS-Cbi，n = 26）、有额颞叶痴呆的ALS（ALS-FTD，n = 20）。我们将每组显示出一定程度认知和/或行为障碍的患者与ALS-Cn患者进行比较，将PET检查时的年龄、性别和修订的ALS功能评定量表作为协变量。
我们在认知受损患者中发现额叶相对代谢减低，从ALS-Ci到ALS-Cbi再到ALS-FTD，这种代谢减低在整个连续过程中更为广泛和显著。ALS-FTD患者还表现出小脑相对代谢增高。ALS-Bi患者与ALS-Cn患者相比未显示出任何差异。
这些数据支持这样的概念，即与单纯运动疾病患者相比，认知受损患者有更广泛的神经退行性过程：认知障碍越严重，代谢变化越弥漫。否则，与单纯行为障碍相关的代谢变化需要进一步明确。
