TABLE 1.
Demographic data—Cortical degeneration
| AD | FTLD | DLB | |
|---|---|---|---|
| Description of the population | |||
| Sample size, n | 41 | 10 | 16 |
| Male, n/n tot (%) | 22/41 (53.7%) | 7/10 (70.0%) | 12/16 (75.0%) |
| Age at symptom onset (years), mean ± SD |
62.0 ± 11.5 n tot = 33 |
62.5 ± 11.4 n tot = 10 |
66.6 ± 9.3 n tot = 14 |
| Duration of disease (years), mean ± SD |
8.4 ± 5.1 n tot = 33 |
6.5 ± 4.4 n tot = 10 |
6.5 ± 4.0 n tot = 14 |
| Age at death (years), mean ± SD |
71.9 ± 11.6 n tot = 41 |
69.0 ± 12.6 n tot = 10 |
74.5 ± 8.5 n tot = 16 |
| Clinical data | |||
| Memory disorders, n/n tot (%) | 27/33 (81.8%) | 5/10 (50.0%) | 9/15 (60.0%) |
| Phasic disorders, n/n tot (%) | 23/33 (69.7%) | 8/10 (80.0%) | 7/15 (46.7%) |
| Praxis disorders, n/n tot (%) | 22/33 (66.7%) | 7/10 (70.0%) | 9/15 (60.0%) |
| Executive disorders, n/n tot (%) | 20/33 (60.6%) | 8/10 (80.0%) | 8/15 (53.3%) |
| Anxio‐depressive disorders, n/n tot (%) | 14/33 (42.4%) | 3/10 (30.0%) | 2/15 (13.3%) |
| Behavioral disorders, n/n tot (%) | 15/33 (45.4%) | 7/10 (70.0%) | 10/15 (66.7%) |
| Hallucinations/delirium, n/n tot (%) | 3/33 (9.1%) | 0/10 (0%) | 11/15 (73.3%) |
| Extrapyramidal syndrome, n/n tot (%) | 4/33 (12.1%) | 1/10 (10.0%) | 13/15 (86.7%) |
| Paraclinical data | |||
| Biomarker CSF, n/n tot (%) | |||
| A+T+ | 6/10 (60.0%) | 0 | – |
| A−T+ | 2/10 (20.0%) | 1/3 (33.3%) | – |
| A+T− | 2/10 (20.0%) | 0 | – |
| A−T− | 0 | 2/3 (66.7%) | – |
| CT‐scan/ MRI abnormalities, n/n tot (%) | 29/31 (93.5%) | 9/10 (90.0%) | 10/10 (100.0%) |
| Neuropsychological tests abnormalities, n/n tot (%) | 25/25 (100.0%) | 7/7 (100.0%) | 8/8 (100.0%) |
| PET‐scan abnormalities, n/n tot (%) | 13/14 (92.9%) | 4/4 (100.0%) | 5/5 (100.0%) |
| DAT‐scan abnormalities, n/n tot (%) | – | – | 5/5 (100.0%) |
| Genetic mutations | One PSEN1 gene mutation | One C9orf72 gene mutation | – |
| Neuropathological data | |||
| Immediate macroscopic analysis | |||
| Brain weight (kg), mean ± SD |
1.23 ± 0.19 n tot = 39 |
1.12 ± 0.13 n tot = 10 |
1.29 ± 0.13 n tot = 15 |
Note: For each subgroup, the total number of headcount (n tot) is specified in case of missing data. Cerebrospinal fluid biomarkers profiles: biomarkers of Aβ plaques (labeled “A”) are low CSF Aβ42 or Aβ42/Aβ40 ratio; biomarkers of fibrillar Tau (labeled “T”) are elevated CSF phosphorylated tau (p‐Tau). Binarizing each of the two biomarker groups into normal/abnormal (−/+) results in four possible biomarker profiles: A+T+, A−T+, A+T−, A−T [20].
Abbreviations: AD, Alzheimer disease; FTLD, frontotemporal lobar degeneration; DLB, dementia with Lewy dodies; CSF, cerebro spinal fluid; CT, computed tomography; MRI, magnetic resonance imaging; PET, positron emission tomography; IHC, immunohistochemistry.