Pure argyrophilic grain disease revisited: independent effects on limbic, neocortical, and striato-pallido-nigral degeneration and the development of dementia in a series with a low to moderate Braak stage

Yokota, Osamu; Miki, Tomoko; Nakashima-Yasuda, Hanae; Ishizu, Hideki; Haraguchi, Takashi; Ikeda, Chikako; Hasegawa, Masato; Miyashita, Akinori; Ikeuchi, Takeshi; Nishikawa, Naoto; Takenoshita, Shintaro; Sudo, Koichiro; Terada, Seishi; Takaki, Manabu

doi:10.1186/s40478-024-01828-6

Table 1 Demographic data in pAGD and control cases

From: Pure argyrophilic grain disease revisited: independent effects on limbic, neocortical, and striato-pallido-nigral degeneration and the development of dementia in a series with a low to moderate Braak stage

	pAGD					Control (n = 34)
	All pAGD (n = 30)	Diffuse form (n = 3)	Saito AG stage III (n = 3)	Saito AG stage II (n = 12)	Saito AG stage I (n = 12)	Control (n = 34)
Female (%)	43.3	33.3	66.7	58.3	25.0	36.4
Age at death (mean ± SD)	76.5 ± 9.0	76.3 ± 14.6	85.7 ± 4.2	77.7 ± 9.1	73.1 ± 7.2	65.3 ± 8.8
Brain weight (g, mean ± SD)	1190.5 ± 154.8	1114.0 ± 104.7	1107.7 ± 208.5	1191.6 ± 127.8	1222.9 ± 177.9	1210.3 ± 174.7
Braak NFT stage (n, %)
Stage 6	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)
Stage 5	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)
Stage 4	7 (0.0)	1 (33.3)	1 (33.3)	4 (33.3)	1 (8.3)	2 (5.9)
Stage 3	3 (0.0)	0 (0.0)	1 (33.3)	0 (0.0)	2 (16.7)	4 (11.8)
Stage 2	17 (23.3)	1 (33.3)	1 (33.3)	7 (58.3)	8 (66.7)	10 (29.4)
Stage 1	3 (10.0)	1 (33.3)	0 (0.0)	1 (8.3)	1 (8.3)	18 (52.9)
Stage 0	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)
Median	2.5	2.0	3.0	2.0	2.0	1.0
25th percentile	1.0	1.0	2.0	2.0	2.0	1.0
75th percentile	2.0	4.0	4.0	4.0	3.0	2.0
Thal phase (n, %)
Phase 5	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)
Phase 4	1 (3.3)	0 (0.0)	1 (33.3)	0 (0.0)	0 (0.0)	0 (0.0)
Phase 3	4 (13.3)	0 (0.0)	1 (33.3)	3 (25.0)	0 (0.0)	0 (0.0)
Phase 2	5 (16.7)	0 (0.0)	0 (0.0)	2 (16.7)	3 (25.0)	2 (5.9)
Phase 1	4 (13.3)	1 (33.3)	0 (0.0)	2 (16.7)	1 (8.3)	9 (26.5)
Phase 0	16 (53.3)	2 (66.7)	1 (33.3)	5 (41.7)	8 (66.7)	23 (67.6)
Median	0.0	0.0	3.0	1.0	0.0	0.0
25th percentile	0.0	0.0	0.0	0.0	0.0	0.0
75th percentile	2.0	1.0	4.0	2.8	1.8	1.0
Lewy body disease (n, %)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)
LATE stage (n, %)
Stage 3	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)	0 (0.0)
Stage 2	6 (20.0)	2 (66.7)	1 (33.3)	3 (25.0)	0 (0.0)	0 (0.0)
Stage 1	2 (6.7)	0 (0.0	0 (0.0)	1 (8.3)	1 (8.3)	0 (0.0)
Stage 0	22 (73.3)	1 (33.3)	2 (66.7)	8 (66.7)	11 (91.7)	34 (100.0)
Median	0.0	2.0	0.0	0.0	0.0	0.0
25th percentile	0.0	1.0	0.0	0.0	0.0	0.0
75th percentile	0.75	2.0	1.0	1.25	0.0	0.0
Dementia, n (%)^a	12/23 (52.2)	3/3 (100.0)	2/2 (100.0)	4/9 (44.4)	3/9 (33.3)	2/28 (7.1)

pAGD pure argyrophilic grain disease, diffuse form diffuse form of pAGD. All diffuse form cases fit the criteria of Saito AG stage III. n the number of cases, SD standard deviation, NFT neurofibrillary tangle, LATE limbic-predominant age-related TDP-43 encephalopathy. n.e. not examined.
^aThe number of cases with dementia but without vascular lesions and the number of all cases without vascular lesions in each group are indicated. Cases having one or more large infarction or two or more lacunae in the cortical and/or subcortical regions were excluded

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