Table 1

Age at disease onset, disease duration, and PRNP codon 129 genotype in patients with PRNP octapeptide repeat insertions

Article	n Repeats	n Patients*	PRNP codon 129†	Age at onset (years)	Disease duration (months)‡
*Patients with mutation analysis, or patients with autopsy and a blood relative with mutation analysis, or patients with mutation analysis as well as autopsy, are reported. †M, methionine; V, valine; the numbers in superscript denote the number of carriers. ‡For patients alive at time of publication, the disease duration is indicated with a “+” sign. §Not included in the analyses because of missing age at onset and duration for each patient. n.a., not available.
Laplanche²⁹	1	1	MM	73	4
Goldfarb¹⁹	2	2	MM	58/75	3/156+
Van Harten²⁰	2	1	VV	61	84
This article	2	1	MV	59	120+
Laplanche²⁹	4	1	VV	82	4
Rossi³⁶	4	1	MM	65	6
Campbell²¹	4	1	MM	56	2
Yanagihara⁹	4	1	MM	56	5
Goldfarb²⁶	5	2	n.a.	31/45	60/180
Cochran²³	5	3	MM	26 to 44	84 to 168+
Skworc³⁷	5	3	MV	51 to 61	4 to 96
Oda³³	6	3	MM	33 to 34	60 to 84+
Capellari²²	6	2	MV	38	48/120
Nicholl³²	6	2	n.a.	34/46	3/60
Collinge,²⁴ Poulter³⁵	6	13	MM⁸/MV³	22 to 47	24 to 156
Goldfarb²⁶	7	3	n.a.	23 to 35	120 to 156
Dermaut⁴⁰	7	2	MM	24/31	132
Goldfarb²⁷	8	3	MM	35 to 54	3 to 24
Laplanche³⁰	8	5	MM	21 to 32	36 to 144+
Van Gool³⁸	8	3	MV¹/VV²	21 to 54	12+ to 72
Moore,³¹ Xiang⁴²^§	8	4	n.a.	23 to 41	n.a.
Owen³⁴	9	1	n.a.	53	30
Krasemann²⁸	9	1	MM	32	72+