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

Table 1 All examined cases of iCJD from countries of treatment with clinical, molecular and histopathological features

Case number	Iatrogenic exposure	Country	Sex	Age at death (years)	Disease duration (months)	Mean incubation period^a (years)	Codon 129 genotype	PrP^Sc type	Histopath. phenotype	Two or more histological sections
1 ^b,c	DM_L	United States	M	26	5	19	na	na	MM(MV)1	yes
2^d,e	DM_T		F	39	4	6	MM	1	MM(MV)1	yes
3	GH		M	33	6	23	VV	na	VV2	yes
4	GH		M	39	17	24	MV	na	MV2K	yes
5	GH		M	39	7	27	MM	2	Atypical	yes
6^e	GH		F	41	2	26	MM	1	MM(MV)1	yes
7	GH		M	43	26	23	MV	i+2	MV2K	yes
8	GH		M	44	18	32.5	MV	i+2	MV2K	yes
9 ^e	GH		M	51	14	38	MM	i	MMi	yes
10 ^e	GH		M	54	2	41.5	MM	1	MM(MV)1	yes
11	GH		M	23	19	10	na	na	MV2K or MMi	no
12	GH		M	34	18	23	na	na	MV2K or MMi	no
13	GH		M	37	3	21	na	na	MM(MV)1	no
14	GH		M	42	5	>28^f	MM	na	Undetermined^g	no
15^h	GH		M	52	4	43	na	na	MM(MV)1	no
mean±SDⁱ				41±8.5	11±8	28±9.5^j
16	DM_L	Australia	M	32	3.5	16.5	MV	na	MM(MV)1	yes
17 ^k	DM_L		M	62	2	5	na	na	MM(MV)1	yes
mean±SD				47±21	3±1	11±8
18	DM_L	France	M	25	8	8	MV	na	MM(MV)1	yes
19	DM_Unk		M	29	6	25	MV	1	MM(MV)1	yes
20	DM_L		M	42	5	6	VV	na	VV2	yes
21	DM_L		F	50	14	11	MM	na	MMi	yes
22	DM_L		F	62	4	4	VV	na	VV2	yes
23	DM_L		F	71	4	4	MV	na	MV2K	yes
mean±SD				46.5±18	7±4	10±8
24	DM_L	Italy	F	23	27	21	VV	2	VV2	yes
25	DM_L		F	26	6	12	MM	na	MM(MV)1	no
26	DM_L		M	42	3.5	18	MM	1+2	MM1+2	yes
27	DM_L		M	75	3	18	VV	2	VV2	yes
mean±SD				41.5±24	10±11.5	17±4

^aThe mean incubation period was measured from the mid-point of GH therapy or date of receipt of the dura graft to the clinical onset of the disease; ^b[3]; ^cNumbers in bold indicate iCJD cases with Aβ-positive pathology; ^d[32]; ^e [10]; ^fUnknown starting date for GH treatment (incubation period thought to be greater than 28 years); ^gAssociated with severe spongiosis and gliosis; ^hBiopsy; ⁱMean±SD of the US iCJD cases does not include DM_L (case 1) and DM_T (case 2); ^jIt does not include case 14; ^k[58]; Histopath. histopathological, DM_Unk dura mater of an unknown (Unk) brand, na not available SD standard deviation

ISSN: 2051-5960