Marelli Cecilia, Hourregue Claire, Gutierrez Laure-Anne, Paquet Claire, Menjot de Champfleur Nicolas, De Verbizier Delphine, Jacob Melissa, Dubois Jonathan, Maleska Aleksandra Maceski, Hirtz Christophe, Navucet Sophie, Bennys Karim, Dumurgier Julien, Cognat Emmanuel, Berr Claudine, Magnin Eloi, Lehmann Sylvain, Gabelle Audrey
Memory Research and Resources Alzheimer Center, Department of Neurology, University Hospital Center, Gui de Chauliac Hospital, Montpellier, France.
Inserm U1198 MMDN, Montpellier, France; EPHE, Paris, France, Montpellier, France.
J Alzheimers Dis. 2020;74(3):903-911. doi: 10.3233/JAD-190378.
Memory troubles and hippocampal atrophy are considered more frequent and focal atrophy less severe in late-onset (>65 years) than in presenile behavioral variant of frontotemporal dementia (bvFTD).
To compare cerebrospinal fluid (CSF) and plasma biomarkers in late-onset and presenile bvFTD.
Multicentric retrospective study (2007-2017) on patients with clinical diagnosis of bvFTD.
This study included 44 patients (67%) with presenile and 22 (33%) with late-onset bvFTD (comparable mean disease duration; n = 11 with causal mutations). Hippocampal atrophy was more frequent (80% versus 25.8%) and severe in late-onset bvFTD (median Scheltens score: 3 [0-4] versus 1 [0-3]), without difference after adjustment for age. Lobar atrophy and focal hypometabolism/hypoperfusion were not different between groups. The median CSF Aβ1-42 and phosphorylated tau (P-tau) concentrations were in the normal range and comparable between groups. Axonal neurodegeneration biomarkers were within the normal range (CSF T-tau; plasma T-tau in late-onset bvFTD) or higher (plasma neurofilament light chain (NFL); plasma T-tau in presenile bvFTD) than the normal values, but globally not different between bvFTD groups. Plasma glial fibrillary acid protein (GFAP) was strongly increased in both bvFTD groups compared with the values in controls of the same age.
The CSF and plasma biomarker profiles did not suggest a more aggressive neurodegeneration in the presenile group (comparable T-tau, NFL, and GFAP levels) or the co-existence of Alzheimer's disease in the late-onset group (comparable and within normal range CSF Aβ1-42 and P-tau). The severity of the neurodegenerative process seems comparable in presenile and late-onset bvFTD.
与早发性行为变异型额颞叶痴呆（bvFTD）相比，晚发性（>65岁）记忆障碍和海马萎缩更为常见，局灶性萎缩则较轻。
比较晚发性和早发性bvFTD患者的脑脊液（CSF）和血浆生物标志物。
对临床诊断为bvFTD的患者进行多中心回顾性研究（2007 - 2017年）。
本研究纳入44例早发性bvFTD患者（67%）和22例晚发性bvFTD患者（33%）（平均病程相当；11例有致病突变）。晚发性bvFTD中海马萎缩更常见（80%对25.8%）且更严重（Scheltens评分中位数：3 [0 - 4]对1 [0 - 3]），年龄校正后无差异。两组间叶性萎缩和局灶性低代谢/低灌注无差异。CSF Aβ1 - 42和磷酸化tau（P - tau）浓度中位数在正常范围内，两组间相当。轴突神经变性生物标志物在正常范围内（CSF T - tau；晚发性bvFTD中的血浆T - tau）或高于正常值（血浆神经丝轻链（NFL）；早发性bvFTD中的血浆T - tau），但总体上bvFTD组间无差异。与同年龄对照组相比，两组bvFTD患者血浆胶质纤维酸性蛋白（GFAP）均显著升高。
CSF和血浆生物标志物谱未提示早发性组存在更具侵袭性的神经变性（T - tau、NFL和GFAP水平相当），也未提示晚发性组存在阿尔茨海默病共存（CSF Aβ1 - 42和P - tau相当且在正常范围内）。早发性和晚发性bvFTD中神经变性过程的严重程度似乎相当。
