Table 1:

Main clinical and paraclinical data of patients

No.	Form	Age (yr)	PRNP	EEGa	14.3.3b	Durationc	MRI Delayedd
1	FFIe	54	MM D178N-129 mol/L	NS	–	6	5
2	gCJDe	69	MM E200K	+	+	4	6
3	gCJDe	58	MM E200K	+	NA	6	3
4	gCJDe	49	MV D178N-129V	–	–	10+	4
5	gCJDe	70	MV V203I	NS	+	11	10
6	gCJDe	67	MM E200K	NA	NA	5	3
7	iCJD	18	MV	NA	–	16	6
8	iCJD	34	MV	+	+	23	8
9	iCJDe	25	MM	–	–	12	8
10	GSSe	47	MV P102 L	–	–	54	27
11	vCJD	43	MM	+	–	15	12
12	vCJD	52	MM	NS	–	8	7
13	sCJD	66	NA	NA	NA	NA	3
14	sCJDe	54	MV	+	+	10	6
15	sCJDe	62	MM	+	+	19	7
16	sCJDe	66	VV	+	+	4	2
17	sCJDe	51	MM	+	+	4	2
18	sCJD	56	MV	–	+	13	8
19	sCJD	74	MM	NA	NA	3	2
20	sCJD	52	MM	+	+	3	2
21	sCJDe	81	MM	+	+	4	4
22	sCJD	53	MV	NS	+	21	1
23	sCJD	55	MV	+	–	60+	23
24	sCJD	80	MV	+	–	9	8
25	sCJD	77	MV	+	–	24+	10
26	sCJD	40	NA	NA	NA	10+	9
27	sCJD	72	MM	+	+	3	2
28	sCJDe	64	MM	+	+	4	1
29	sCJD	55	NA	–	+	26	20
30	sCJD	84	NA	NA	NA	5	3
31	sCJD	80	NA	NS	–	2	1

Note:— – indicates not present.

^a+ Indicates periodic sharp wave complexes.

^bDetection of 14.3.3 protein in the CSF.

^cDuration of the disease in months.

^dTime in months between first symptoms and MR imaging examination.

^eThe diagnosis was confirmed by postmortem examination and/or mutation was present in the PRNP. PRNP: genotype at codon 129 (MM, VV, MV) and mutation when present.