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Table 2 CSF RT-QuIC protocols and diagnostic accuracy in prion disease

From: Towards an improved early diagnosis of neurodegenerative diseases: the emerging role of in vitro conversion assays for protein amyloids

PrP Substrate

Reaction buffer

Parameters

Cohort size (N)a

Sens.

Spec.

Ref.

Hu 23–231

0.06–0.1 g/L substrate, 15 μL seed, 500 mM NaCl

30 s circular shaking, 30 s incubation, 2 min break, 37 °C

18 def sCJD

83%

 

[5]

35 def non-CJD

 

100%

25 prob. sCJD

100%

 

130 ctrl

 

100%

16 prob. sCJD

87%

 

14 ctrl

 

100%

0.05 g/L substrate, 5 μL seed, 500 mM NaCl

24 gCJD

83%

-

[81]

20 GSS

90%

-

12 FFI

83%

-

Ha 23–231

0.1 g/L substrate, 15 μl seed, 300 mM NaCl

1 min double orbital shaking 600 rpm, 1 min rest, 42 °C

cohort 1: 56 def sCJD

91%

 

[54]

52 ctrl

 

98%

cohort 2: 67 def sCJD

87%

  

52 ctrl

 

100%

 

15/13 def/prob. sCJD

79%

 

[59]

43 ctrl

 

100%

179/97/29 def/prob./pos sCJD

81%

 

[50]

46 gCJD

91%

 

348 ctrl

 

99%

24/26 def/prob. sCJD

73%

–

[13]

Ha 14–128 - Sheep 141–234 (chimera)

0.1 g/L substrate, 15 μL seed, 170 mM NaCl

1 min double orbital shaking, 600 rpm, 1 min rest, 42 °C

64 def sCJD

80%

 

[22]

39 gCJD

100%

 

400 ctrl

 

99%

Ha 90–231

0.1 g/L substrate, 15–30 μL seed, 300 mM NaCl, 0,002% SDS

1 min double orbital shaking 700 rpm, 1 min rest, 55 °C

10/1 def/prob. sCJD

91%

-

[60]

43/5 def/prob. sCJD

96%

 

39 ctrl

 

100%

36/68 def/prob. sCJD

94%

 

[36]

64 ctrl

 

100%

111 sCJD

92%

  

15 gCJD

93%

 

[30]

67 ctrl

 

98.5%

 

63 sCJD, 14 ctrlb

95%

100%

 

10/12 def/prob. sCJD

95%

 

[13]

17 ctrl

 

100%

116/73 def/prob. sCJD

93%

 

[31]

33 gCJD

100%

 

100 ctrl

 

100%

61/41 def/prob. sCJD

96%

 

[29]

80 ctrl

 

100%

439 def sCJD

93

 

[72]

31 gCJD

97%

 

69 ctrl

 

98.5%

  1. Abbreviations: Hu Human, Ha Hamster, prob Probable, pos Possible, def Definite, ctrl Controls, sens. Sensitivity, spec. Specificity, sCJD Sporadic CJD, gCJD Genetic CJD, FFI Fatal familial insomnia, GSS Gerstmann-Sträussler-Scheinker disease
  2. aResults obtained in disease groups with an n of 9 or lower have been omitted
  3. bProspective cohort