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Table 2 Demographics and classification of patient groups

From: The CSF neurofilament light signature in rapidly progressive neurodegenerative dementias

 

Total (n)

Typical cases (n)

Atypical cases (n)

Age at LPa, years (mean ± SD)

Female sex (%)

Prion diseases

141

82

59

65.5 ± 9.9

55.6

 Definite sCJD

97

62

35

  

  MM(V)1

37

30

7

 

  VV2

26

26

0

 

  MV2K

22

6

16

 

  MM2C

8

0

8

 

  MM2T

2

0

2

 

  VV1

1

0

1

 

  VPSPr (VV)

1

0

1

 

 Definite gCJD

16

7

9

 

  E200K-129 M

11

4

7

 

  V210I-129 M

4

3

1

 

  D178N-129 V

1

0

1

 

 Probable CJD

27

13

14

 

  MM

4

1

3

 

  MV

14

3

11

 

  VV

9

9

0

 

 GSS

1

0

1

 

AD

73

36

37

66.9 ± 9.5

61.6

DLB

35

24

11

72.3 ± 7.8

40.0

FTLD

44

35

9

63.0 ± 9.0

43.2

 FTD

25

19

6

  

  bvFTD

19

14

5

 

  PPA

6

5

1

 

 CBS

11

9

2

 

 PSP

8

7

1

 

Control subjects

30

  

63.6 ± 10.7

36.7

  1. Abbreviations: AD Alzheimer’s disease, bvFTD Behavioral variant of frontotemporal dementia, CBS Corticobasal syndrome, CJD Creutzfeldt-Jakob disease, DLB Dementia with Lewy bodies, FTLD Frontotemporal lobar degeneration, gCJD Genetic Creutzfeldt-Jakob disease, GSS Gerstmann-Sträussler-Scheinker syndrome, 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, PPA Primary progressive aphasia, PSP Progressive supranuclear palsy, 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
  2. aNo significant differences regarding age were detected between groups by one-way analysis of variance (followed by Tukey’s post hoc test) with the Bonferroni correction