期刊
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
卷 90, 期 1, 页码 4-10出版社
BMJ PUBLISHING GROUP
DOI: 10.1136/jnnp-2018-318868
关键词
-
资金
- EU Joint Programme-Neurodegenerative Disease Research (JPND) network SOPHIA [01ED1202A]
- EU Joint Programme-Neurodegenerative Disease Research (JPND) network BiomarkAPD [01ED1203F]
- EU Joint Programme-Neurodegenerative Disease Research (JPND) network PreFrontAls [01ED1512]
- German Federal Ministry of Education and Research [FTLDcO1GI1007A, MND-Net 01GM1103A]
- EU [NADINE 246513, FAIR-PA RK II 633190]
- German Research Foundation/DFG [VO2028, SFB1279]
- foundation of the state Baden-Wurttemberg [D3830]
- Boehringer Ingelheim Ulm University BioCenter [D5009]
- Thierry Latran Foundation
- Departmental Funds (GPS-ALS Study)
- Charcot Foundation
- Swedish Brain Research Foundation
- Bertil Hallsten Foundation
- Swedish Research Council
- Knut and Alice Wallenberg Foundation
- Ulla-Carin Lindquist Foundation
Objective To investigate the role of neuroinflammation in asymptomatic and symptomatic amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) mutation carriers. Methods The neuroinflammatory markers chitotriosidase 1 (CHIT1), YKL-40 and glial fibrillary acidic protein (GFAP) were measured in cerebrospinal fluid (CSF) and blood samples from asymptomatic and symptomatic ALS/FTD mutation carriers, sporadic cases and controls by ELISA. Results CSF levels of CHIT1, YKL-40 and GFAP were unaffected in asymptomatic mutation carriers (n=16). CHIT1 and YKL-40 were increased in gALS (p<0.001, n=65) whereas GFAP was not affected. Patients with ALS carrying a CHIT1 polymorphism had lower CHIT1 concentrations in CSF (-80%) whereas this polymorphism had no influence on disease severity. In gFTD (n=23), increased YKL-40 and GFAP were observed (p<0.05), whereas CHIT1 was nearly not affected. The same profile as in gALS and gFTD was observed in sALS (n=64/70) and sFTD (n=20/26). CSF and blood concentrations correlated moderately (CHIT1, r=0.51) to weak (YKL-40, r=0.30, GFAP, r=0.39). Blood concentrations of these three markers were not significantly altered in any of the groups except CHIT1 in gALS of the Ulm cohort (p<0.05). Conclusion Our data indicate that neuroinflammation is linked to the symptomatic phase of ALS/FTD and shows a similar pattern in sporadic and genetic cases. ALS and FTD are characterised by a different neuroinflammatory profile, which might be one driver of the diverse presentations of the ALS/FTD syndrome.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据