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Table 3 CSF biomarkers of neuroinflammation in prion disease subtypes

From: CSF biomarkers of neuroinflammation in distinct forms and subtypes of neurodegenerative dementia

SubtypeNumberCHIT1 all (pg/ml), median (IQR)CHIT1 WT + Het (pg/ml), median (IQR)YKL-40 (ng/ml), median (IQR)GFAP (ng/ml), median (IQR)
All sCJD854092 (1509–8644)4154 (1872–8645)321 (222–471)1.115 (0.633–1.655)
sCJD MM(V)1343069 (1229–6921)3290 (1235–7191)259 (175–358)0.810 (0.611–1.295)
sCJD VV2265060 (2474–15,725)5718 (2877–30,500)533 (314–783)1.638 (0.857–2.559)
sCJD MV2K205064 (2170–8532)5064 (2170–8532)321 (214–406)0.763 (0.352–1.230)
sCJD MM2C42158 (646–6125)2572195 (178–314)1.146 (0.577–1.716)
sCJD VV1138,00038,0004550.829
VPSPr1256225623412.779
gCJD E200K52572 (828–4415)2767269 (206–782)1.127 (0.955–2.088)
gCJD V210I51820 (934–4415)1820 (1226–3838)349 (184–472)0.412 (0.329–1.351)
FFI (D178N)316,800, 1571, 415616,800,1571, 4156146, 253, 1650.827, 0.415, 0.227
Pre-symptomatic GSS (P102L)18353*8353*186*1.114*
Symptomatic GSS (P102L)218000*, 235618000*, 2356297*, 4501.712*, 0.418
  1. CHIT1 chitotriosidase 1, CJD Creutzfeldt-Jakob disease, FFI fatal familial insomnia, gCJD genetic Creutzfeldt-Jakob disease, GFAP glial fibrillary acidic protein, GSS Gerstmann-Sträussler-Scheinker syndrome, Het heterozygotes for CHIT1 24-bp duplication, IQR interquartile range, LP lumbar puncture, MM(V)1 methionine homozygosity (valine) and scrapie prion protein type 1, MM2C methionine homozygosity and scrapie prion protein type 2, cortical type MM2T methionine homozygosity and scrapie prion protein type 2, thalamic type, MV2K methionine/valine heterozygosity and scrapie prion protein type 2, kuru type, sCJD sporadic Creutzfeldt-Jakob disease, VPSPr variably protease-sensitive prionopathy, VV1 valine homozygosity and scrapie prion protein type 1, VV2 valine homozygosity and scrapie prion protein type 2, WT wild type for CHIT1 24-bp duplication, YKL-40 chitinase-3-like protein 1
  2. *The same GSS case