Table 2.

CSF RT-QuIC protocols and diagnostic accuracy in prion disease

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%

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

^aResults obtained in disease groups with an n of 9 or lower have been omitted

^bProspective cohort