TABLE 1:

Patient data, clinical findings, and MR imaging results

Case No./Patient Sex/Age (y)	Dementia	Myoclonus	PSD	Prion Protein Gene	CDJ Type	Diagnostic Level	Diffusion-weighted Imaging	FLAIR Imaging	ADC Analysis
1/F/55	Yes	Yes	Yes	M232R	F	Definite	E(1), L(2)	E(1), L(2)	E(1), L(2)
2/M/78	Yes	No	No	V180I	F	Definite	L(2)	L(2)	L(2)
3/M/70	No	Yes	Yes	NA	S?	Probable	E(4), L(1)	E(1)	NA
4/M/66	Yes	Yes	Yes	Wild type	S	Probable	E(1), L(3)	E(1), L(3)	E(1), L(3)
5/M/60	Yes	No	Yes	Wild type	S	Probable	E(1), L(1)	E(1), L(1)	E(1), L(1)
6/M/58	Yes	Yes	Yes	E200K	F	Definite	L(3)	NA	NA
7/F/75^*	Yes	No	No	Wild type^†	S	Definite	L(1)	L(1)	L(1)
8/M/68	Yes	Yes	Yes	Wild type	S	Probable	E(2)	E(1)	NA
9/F/74	Yes	Yes	Yes	Wild type	S	Probable	L(1)	L(1)	L(1)
10/F/76	Yes	Yes	Yes	NA	S?	Probable	E(1)	E(1)	E(1)
11/M/78	Yes	Yes	Yes	NA	S?	Probable	E(1)	E(1)	E(1)
12/F/76	Yes	Yes	Yes	NA	S?	Probable	E(2)	E(2)	E(1)
13/F/82	Yes	No	No	V180I	F	Definite	E(1)	E(1)	E(1)

Note.—E200K indicates the change of codon 200 from glutamate to lysine; M232R, change of codon 232 from methionine to arginine; V180I, change of codon 180 from valine to isoleucine; NA, not available; F, familial; S, sporadic; S?, possibly sporadic; E, the early-stage study; L, late-stage study. The number in the parenthesis indicates the number of MR studies performed. Total numbers of examinations were as follows: diffusion-weighted imaging, 14 early stage and 14 late stage; FLAIR imaging, 10 early stage and 10 late stage; ADC imaging, seven early stage and 10 late stage.
* The diagnosis of CJD was confirmed at histopathologic analysis.
† Wild-type prion protein gene with valine homozygosity at codon 129.