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

Table 2 Staging and amyloid presence in Aβ CP from iCJD associated with Aβ pathology along with cases of sCJD and AD used as controls

Case number	Age (y)	Disease durat. (mo)	Codon 129 genotype	PrP^Sc type	Phase 1		Phase 2		Phase 3-4		Phase 5
Case number	Age (y)	Disease durat. (mo)	Codon 129 genotype	PrP^Sc type	Neocortex	Thiofl	Hippoc.	Thiofl	Subcortical	Thiofl	Cerebellum	Thiofl
iCJD
1 (1)^a	26	5	na	na	+++^b,c	+^d	++	-	+^e	-	+	-
2 (19)	29	6	MV	1	+++^f	+	+	+	-	nt	-	nt
3 (8)	44	18	MV	i+2	++++	+	++	-	+++	-	-	-
4 (9)	51	14	MM	i	-	-	-	-	-	nt	+	+
5 (17)	62	2	na	na	++++	+^g	na	na	na	na	-	-
mean±SD	42±15	9±7			78%^h		62.5%		40%		40%
sCJD
1 (57)ⁱ	63	3	MV	1	+++^f	+	++	+	++	nt	-	nt
2 (59)	71	9	MV	2	+^f	+	-	-	-	nt	-	nt
3 (65)	71	10	VV	2	+	+	-	nt	-	nt	-	nt
4 (67)	79	6	VV	2	+++	+	-	nt	-	nt	-	nt
mean±SD	71±6.5	7±3			57%		25%		17%		0%
P value^j					NS		NS		NS		NS
AD
1	56	60	na	na	++++	+	++	+	+++	+	+	+
2	56	60	na	na	++++	+	++	+	+++	+	+	+
3	59	na	na	na	++++	+	++	+	+++	+	+	+
4	60	130	na	na	++++	+	++	+	+++	+	+	-
5	70	66	na	na	++++	+	++	+	+++	+	+	+
6	72	111	na	na	++++	+	++	+	+++	-	+	+
7	74	22	na	na	++++	+	++	+	+++	+	+	+
mean±SD	64±8	75±39			100%		100%		100%		100%
P value^k					< 0.02		< 0.04		< 0.0004		< 0.05

Phases 1 to 5 refer to Aβ plaques deposition in various brain regions reflecting disease progression according to Thal et al [63]; ^a,iNumbers in parenthesis in the first column refer to numerals used in ^aTable 1 and ⁱAdditional file 1: Table S2; ^bMinus and plus signs indicate absence (-) or presence (+) of Aβ pathology affecting one (+), two (++), three (+++) or four (++++) of the brain regions that characterize each phase; Phase 1: frontal, temporal, parietal and occipital cortical regions; Phase 2: hippocampus and entorhinal cortex; Phase 3-4: striatum, thalamus and midbrain; Phase 5: cerebellum; ^c,eRefers to one iCJD with one or two missing sections from the ^cneocortex or ^esubcortical regions; ^dThe (+) sign under Thiofl (Thioflavin S) indicates presence of amyloid plaques in at least one brain region; the (-) sign indicates that all regions tested had Thioflavin S negative staining; ^fRefers to one iCJD and two sCJD cases with missing parietal cortex; ^gPositive staining with Congo red [58]; ^hPercentage of brain regions with CP; ^j,kFisher’s exact test comparing iCJD to ^jsCJD or ^kAD; Hippoc. hippocampal formation (hippocampus and entorhinal cortex), y years, durat. duration, mo months, na not available, nt not tested, NS not significant, SD standard deviation

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