Table 2.

Results of the diagnosing tests and frequency of clinical symptoms in E200K mutation carrier and V210I mutation carrier as well as the sCJD control groups (different sCJD controls matched by age, gender and codon 129 PRNP genotype separately for both mutations with the same number of patients)

Diagnostic tests	E200K	sCJD	V210I	sCJD
14-3-3
MM (%)	100	100	100	100
MV (%)	82	82	100	67
Median tau [pg/ml]
MM	8628	11472	7628	10716
MV	4172	2319	7686	2348
EEG
MM (%)	27	42	46	50
MV (%)	27	10	33	50
MRI
MM (%)	88	90	100	80
MV (%)	90	100	100	100

Clinical signs (%)	Disease onset		During disease		Disease onset		During disease
	E200K	sCJD	E200K	sCJD	V210I	sCJD	V210I	sCJD
Prodromal phase	74	83	74	83	75	81	75	81
Dementia	61	48	87	87	69	69	88	100
Cerebellar signs	52	78	100	100	63	75	100	100
Myoclonic jerks	9	4	65	87	25	19	88	88
Pyramidal signs	35	17	83	57	13	31	38	75
Extrapyramidal signs	13	9	70	52	31	6	81	75
Psychiatric features	30	48	70	70	13	25	31	63
Sensory disturbances	30	13	30	13	19	6	38	6