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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

Subtype

Number

CHIT1 all (pg/ml), median (IQR)

CHIT1 WT + Het (pg/ml), median (IQR)

YKL-40 (ng/ml), median (IQR)

GFAP (ng/ml), median (IQR)

All sCJD

85

4092 (1509–8644)

4154 (1872–8645)

321 (222–471)

1.115 (0.633–1.655)

sCJD MM(V)1

34

3069 (1229–6921)

3290 (1235–7191)

259 (175–358)

0.810 (0.611–1.295)

sCJD VV2

26

5060 (2474–15,725)

5718 (2877–30,500)

533 (314–783)

1.638 (0.857–2.559)

sCJD MV2K

20

5064 (2170–8532)

5064 (2170–8532)

321 (214–406)

0.763 (0.352–1.230)

sCJD MM2C

4

2158 (646–6125)

2572

195 (178–314)

1.146 (0.577–1.716)

sCJD VV1

1

38,000

38,000

455

0.829

VPSPr

1

2562

2562

341

2.779

gCJD E200K

5

2572 (828–4415)

2767

269 (206–782)

1.127 (0.955–2.088)

gCJD V210I

5

1820 (934–4415)

1820 (1226–3838)

349 (184–472)

0.412 (0.329–1.351)

FFI (D178N)

3

16,800, 1571, 4156

16,800,1571, 4156

146, 253, 165

0.827, 0.415, 0.227

Pre-symptomatic GSS (P102L)

1

8353*

8353*

186*

1.114*

Symptomatic GSS (P102L)

2

18000*, 2356

18000*, 2356

297*, 450

1.712*, 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