RT Journal Article
SR Electronic
T1 Different neuroinflammatory profile in amyotrophic lateral sclerosis and frontotemporal dementia is linked to the clinical phase
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 4
OP 10
DO 10.1136/jnnp-2018-318868
VO 90
IS 1
A1 Patrick Oeckl
A1 Patrick Weydt
A1 Petra Steinacker
A1 Sarah Anderl-Straub
A1 Frida Nordin
A1 Alexander E Volk
A1 Janine Diehl-Schmid
A1 Peter M Andersen
A1 Johannes Kornhuber
A1 Adrian Danek
A1 Klaus Fassbender
A1 Klaus Fliessbach
A1 German Consortium for Frontotemporal Lobar Degeneration
A1 Holger Jahn
A1 Martin Lauer
A1 Kathrin Müller
A1 Antje Knehr
A1 Johannes Prudlo
A1 Anja Schneider
A1 Dietmar R Thal
A1 Deniz Yilmazer-Hanke
A1 Jochen H Weishaupt
A1 Albert C Ludolph
A1 Markus Otto
YR 2019
UL http://jnnp.bmj.com/content/90/1/4.abstract
AB 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.