Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study

Cali, Ignazio; Cohen, Mark L.; Haїk, Stéphane; Parchi, Piero; Giaccone, Giorgio; Collins, Steven J.; Kofskey, Diane; Wang, Han; McLean, Catriona A.; Brandel, Jean-Philippe; Privat, Nicolas; Sazdovitch, Véronique; Duyckaerts, Charles; Kitamoto, Tetsuyuki; Belay, Ermias D.; Maddox, Ryan A.; Tagliavini, Fabrizio; Pocchiari, Maurizio; Leschek, Ellen; Appleby, Brian S.; Safar, Jiri G.; Schonberger, Lawrence B.; Gambetti, Pierluigi

doi:10.1186/s40478-017-0503-z

Acta Neuropathologica Communications

Table 3 Staging of Aβ CAA in iCJD and control cases of sCJD and AD

From: Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study

Case number	Age (y)	Disease duration (mo)	Codon 129 genotype	PrP^Sc type	Phase 1		Phase 2					Phase 3		Vstl.^a score	CAA^b type
Case number	Age (y)	Disease duration (mo)	Codon 129 genotype	PrP^Sc type	Neocortex	Thiofl	Hippoc.	Thiofl	Crbl	Thiofl	Midb.	Subcort.	Thiofl
iCJD
1 (1)^c	26	5	na	na	+++^d,e	+^f	++	+	+	+	na	-^e	-	3	1
2 (19)	29	6	MV	1	+++^g	+	+	+	+	nt	+	+	nt	2	2
3 (16)	32	3.5	MV	na	+^h	na	-	na	-	na	-	-	na	1	2
4 (8)	44	18	MV	i+2	++++	+	++	-	+	+	-	-	-	3	2
5 (21)	50	14	MM	na	+++^g	+	-	nt	-	nt	+	+	nt	1	2
6 (9)	51	14	MM	i	++	+	-	-	+	+	-	-	-	2	2
7 (10)	54	2	MM	1	+	+	-	-	-	-	-	-	nt	1	2
8 (17)	62	2	na	na	++++	+ⁱ	na	na	+	+ⁱ	na	na	na	na	na
9 (22)	62	4	VV	na	++^g	-	-	nt	+	-	+	+	nt	1	2
10 (23)	71	4	MV	na	-	nt	+	-	+	-	-	-	nt	1	2
11 (27)	75	3	VV	2	++++	-	+	-	+	-	-	-	nt	1	2
mean±SD	50.5±17	7±6			69%^j		35%		73%		33%	16%
sCJD
1 (55)^k	49	4	MM	1	+^g	-	-	-	-	nt	-	-	nt	1	2
2 (56)	63	4	MM	1	+^g	-	++	-	+	-	+	++	nt	1	2
3 (57)	63	3	MV	1	-	-	-	-	+	nt	-	+	nt	1	2
4 (44)	64	1	MM	1	++	+	-	-	-	nt	-	-	-	1	2
5 (58)	71	8	VV	2	+++^g	-	++	-	+	-	-	-	nt	1	2
6 (66)	74	4.5	VV	2	+	-	-	nt	-	nt	-	-	nt	1	2
mean±SD	64±9	4±2			38%		33%		50%		17%	25%
P value^l					< 0.03		NS		NS		NS	NS		= 0.05*	NS*
AD
1	56	60	na	na	++++	+	-	-	+	+	+	+	-	2	2
2	56	60	na	na	++++	+	-	-	+	+	-	-	-	3	2
3	59	na	na	na	++++	-	-	-	+	+	-	-	-	3	2
4	60	130	na	na	++++	-	-ⁿ	-	+	-	-	+	+	2	2
5	70	66	na	na	++++	+	+	-	+	+	-	-	-	2	2
6	72	111	na	na	++++	+	+	+	+	+	+	+	+	3	1
7	74	22	na	na	+++	-	-	-	+	+	-	+	-	3	2
mean±SD	64±8	75±39			96%		15%		100%		29%	29%
P value^m					< 0.006		NS		NS		NS	NS		< 0.02*	NS*

Phases 1 to 3 refer to Aβ CAA deposition in various brain regions, reflecting disease progression according to Thal et al [61]; ^aVonsattel (Vstl) 0-3 score of Aβ CAA severity to the highest score in one or more brain locations [66]; ^bPresence (type 1) or absence (type 2) of Aβ deposits in the cortical capillaries [60]; ^c,kNumbers in parenthesis in the first column refer to numerals used in ^cTable 1 and ^kAdditional file 1: Table S2; ^dMinus and plus signs indicate absence (-) or presence (+) of Aβ CAA affecting one (+), two (++), three (+++) or four (++++) brain regions in each phase; Phase 1: frontal, temporal, parietal and occipital cortical regions; Phase 2: hippocampus, entorhinal cortex, cerebellum and midbrain; Phase 3: striatum, thalamus; ^eRefers to one iCJD patient with one missing section from neocortex and subcortical regions; ^fThe (+) sign under Thiofl (Thioflavin S) indicates presence of positive staining of at least one brain region; the (-) sign indicates that all regions examined had Thioflavin S negative staining; ^gRefers to 3 iCJD and 3 sCJD cases with missing parietal cortex; ^hRefers to one iCJD with missing frontal cortex; ⁱPositive staining with Congo red [58]; ^jPercentage of brain regions with Aβ CAA; ^l,mFisher’s exact test comparing iCJD to ^lsCJD or ^mAD; ⁿRefers to one AD patient with missing entorhinal cortex; y years, mo months Hippoc. hippocampal formation (hippocampus and entorhinal cortex), Crbl cerebellum, Midb. midbrain, Subcort. subcortical regions (striatum and thalamus), na not available, nt not tested, NS not significant, SD standard deviation; *Student’s t-test

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