TY - JOUR
T1 - Inflammatory markers of CHMP2B-mediated frontotemporal dementia
AU - Roos, Peter
AU - von Essen, Marina Rode
AU - Nielsen, Troels Tolstrup
AU - Johannsen, Peter
AU - Stokholm, Jette
AU - Bie, Anne Sigaard
AU - Waldemar, Gunhild
AU - Simonsen, Anja Hviid
AU - Heslegrave, Amanda
AU - Zetterberg, Henrik
AU - FReJA Consortium
AU - Sellebjerg, Finn
AU - Nielsen, Jørgen Erik
PY - 2018/11/15
Y1 - 2018/11/15
N2 - Histopathological studies and animal models have suggested an inflammatory component in the pathomechanism of the CHMP2B associated frontotemporal dementia (FTD-3). In this cross-sectional study, serum and cerebrospinal fluid were analyzed for inflammatory markers in CHMP2B mutation carriers. Serum levels of CCL4 were increased throughout life. Serum levels of IL-15, CXCL10, CCL22 and TNF-α were significantly associated with cognitive decline, suggesting a peripheral inflammatory response to neurodegeneration. CSF levels of sTREM2 appeared to increase more rapidly with age in CHMP2B mutation carriers. The identification of a peripheral inflammatory response to disease progression supports the involvement of an inflammatory component in FTD-3.
AB - Histopathological studies and animal models have suggested an inflammatory component in the pathomechanism of the CHMP2B associated frontotemporal dementia (FTD-3). In this cross-sectional study, serum and cerebrospinal fluid were analyzed for inflammatory markers in CHMP2B mutation carriers. Serum levels of CCL4 were increased throughout life. Serum levels of IL-15, CXCL10, CCL22 and TNF-α were significantly associated with cognitive decline, suggesting a peripheral inflammatory response to neurodegeneration. CSF levels of sTREM2 appeared to increase more rapidly with age in CHMP2B mutation carriers. The identification of a peripheral inflammatory response to disease progression supports the involvement of an inflammatory component in FTD-3.
U2 - 10.1016/j.jneuroim.2018.08.009
DO - 10.1016/j.jneuroim.2018.08.009
M3 - Journal article
C2 - 30193769
SN - 0165-5728
VL - 324
SP - 136
EP - 142
JO - Journal of Neuroimmunology
JF - Journal of Neuroimmunology
ER -