Table 1.
Demographics | Total | Tauopathy | FTLD-Ua | DLDH | AD | Other |
---|---|---|---|---|---|---|
Age at onset (range), yr | 60.4 (30-82), n = 111b |
61.0 (30-77), n = 51 |
60.3 (46-79), n = 33 |
54.8 (43-80), n = 4 |
60.3 (34-82), n = 19 |
59.8 (50-70), n = 4 |
Sex, M:F (% male) | 58:56 (50.9%) | 28:25 (53%) | 14:19 (42%) | 2:2 (50%) | 10:9 (53%) | 4:1 (80%) |
Education (range), yr | 15.3 (8-23), n = 85 |
15.3 (10-21), n = 36 |
15.2 (10-23), n = 25 |
16.0 (15-17), n = 3 |
15.8 (12-19), n = 18 |
12.0 (8-16), n = 3 |
Disease duration, (range), mo | 89.1 (17-300), n = 109b |
90.8 (24-300), n = 49 |
81.5 (20-264), n = 32 |
82.8 (36-120), n = 4 |
107.0 (24-204), n = 19 |
57.0 (17-151), n = 5 |
Family historyc | Probable: 17.0% Possible: 30.0% |
Probable: 22.4% Possible: 22.4% |
Probable: 19.2% Possible: 30.8% |
Possible: 75.0% | Probable: 6.3% Possible: 43.8% |
Possible: 20.0% |
Six patients with frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) pathology were diagnosed clinically with motor neuron disease (MND) at some point after presentation. Mean age of onset = 51.3 years; mean education = 17.3 years; mean disease duration = 49.3 months.
Information regarding age at onset of dementia was not available in three patients.
Family history was coded according to the following criteria: definite = family history consistent with autosomal dominant pattern of inheritance (ie, at least three affected first-degree relatives in multiple generations) and/or identification of mutations in MAPT or valosin-containing protein genes; probable = two first-degree relatives with an FTLD diagnosis or related dementia; possible = one first-degree relative or two second-degree relatives with a diagnosis of FTLD or related dementia; none = no family history of FTLD or related condition. Information regarding family history was available in only 100 of the 114 patients.
DLDH = dementia lacking distinctive histopathology; AD = Alzheimer’s disease.