Table 1.
Demographic and clinical characteristics of patients with GGT pathologic diagnosis vs non-GGT 4-repeat tauopathy pathology (PSP or CBD)
| Clinical and demographic characteristics | GGT n = 12 |
NG4T‡ n = 50 |
P-value† |
|---|---|---|---|
|
| |||
| Female, n, % | 10 (83) | 21 (42) | 0.022* |
| White, n, % | 11 (92) | 49 (98) | >0.99 |
| Education, median (range), years | 16 (9 – 16) | 16 (12 – 20) | 0.55 |
| Right-handed, n, % | 10 (91) | 42 (84) | 0.64 |
| Family history, n, % | 6 (50)§ | 16 (32) | 0.32 |
| Onset age, median (range), years | 67 (50 – 81) | 63 (47 – 76) | 0.19 |
| Disease duration, median (range), years | 6 (3.4 – 15) | 9 (4.4 – 15) | 0.13 |
| Age at death, median (range), years | 76.5 (55 – 87) | 72 (55 – 88) | 0.37 |
| Age at scan, median (range), years | 71 (54– 82) | 68 (48 – 79) | 0.25 |
| Initial clinical diagnosis, n, % | 0.004** | ||
| Primary progressive AOS | 3 (25) | 17 (34) | |
| Behavioral variant FTD | 2 (17) | 1 (2) | |
| Primary lateral sclerosis | 2 (17) | 0 (0) | |
| Richardson syndrome | 1 (8) | 14 (28) | |
| Progressive supranuclear palsy (non-RS) | 0 (0) | 7 (14) | |
| Corticobasal syndrome | 1 (8) | 2 (4) | |
| Alzheimer’s dementia | 1 (8) | 0 (0) | |
| Semantic variant PPA | 1 (8) | 0 (0) | |
| Nonfluent/agrammatic variant PPA | 0 (0) | 9 (18) | |
| Luria’s dynamic aphasia | 1 (8) | 0 (0) | |
| Final clinical diagnosis, n, % | 0.12 | ||
| Behavioral variant FTD | 2 (17) | 1 (2) | |
| Corticobasal syndrome | 4 (33) | 19 (38) | |
| Richardson syndrome | 2 (17) | 14 (28) | |
| Progressive supranuclear palsy (non-RS) | 0 (0) | 10 (20) | |
| Primary lateral sclerosis | 1 (8) | 0 (0) | |
| Alzheimer’s dementia | 1 (8) | 0 (0) | |
| Primary progressive AOS | 0 (0) | 1 (2) | |
| Semantic variant PPA | 1 (8) | 0 (0) | |
| Nonfluent/agrammatic variant PPA | 1 (8) | 3 (6) | |
| Logopenic variant PPA | 0 (0) | 1 (2) | |
| Progressive auditory agnosia | 0 (0) | 1 (2) | |
| Clinical features, n, % | |||
| Pyramidal tract | 5 (42) | 14 (28) | 0.49 |
| Limb weakness, lower motor neuron | 2 (17) | 0 (0) | 0.035* |
| Speech abnormalities | 5 (42) | 42 (84) | 0.005** |
| Eye movement abnormal | 6 (50) | 31 (62) | 0.52 |
| Parkinsonism | 6 (50) | 48 (96) | 0.003** |
| Language | 4 (33) | 19 (38) | >0.99 |
| Behavior/personality | 6 (50) | 25 (50) | >0.99 |
| Memory/attention/executive function | 6 (50) | 25 (50) | >0.99 |
| Other | 3(25)¶ | 1 (2)¥ | n/a |
†
P-values are from two-tailed Wilcoxon test for continuous variable and from two-tailed Fisher’s exact test for categorical variables
‡
Non-GGT 4R tauopathy group included autopsy-confirmed PSP (n = 30) and CBD (n = 20)
§
Mutation in MAPT gene was identified in 2 patients upon death
¶
One patient had hallucinations and delusions; another patient developed voice tremor; third patient had nonverbal orobuccal apraxia and Luria’s dynamic aphasia
¥
One patient had progressive auditory agnosia (verbal and environmental)