TABLE 1.

Patient demographic data and clinical features

Patient no.	Age of onset (yr)	Disease duration (mo)	Gender	PRNP genotype at codon 129	sCJD subtype	Clinical signs at onset	Visual symptomsa	Visuospatial dysfunctionb
1	60	1.5	M	MM	1	Cognitive/visual	Yes	No
2	60	20	M	MV	1-2	Visuospatial	Yes	Yes
3	69	15	M	MV	2	Behavior/memory	No	No
4	79	27	M	MV	2	Cognitive	No	No
5	55	6	F	VV	2	Apraxia	Yes	No
6	56	10	F	MM	1-2	Language	No	No
7	57	4	F	MM	1	Motor	No	No
8	55	24	F	MV	1-2	Behavior	No	No
9	63	2	F	MM	1	Language	No	Yes
10	69	6	F	MV	1	Behavior/memory	No	No
11	69	10	F	MM	1-2	Cognitive/apraxia	No	Yes

^aVisual symptoms noted at first through last UCSF visit.

^bVisuospatial dysfunction based on neuropsychological testing or neurological examination.