Table 2.
Demographic and clinical characteristics of cases in the autopsy cohort
| Later onset | Usual Onset | P value | |
|---|---|---|---|
| Number of Patients | 9 | 184 | |
| Male, % (n) | 44.4 (4/9) | 52.7 (97/184) | 0.74 |
| Age at onset (years, mean ± SD) | 80.8 ± 4.5 | 57.7 ± 7.7 | < 0.0001 |
| Initial symptoms, % (n) | |||
| Gait difficulty | 44.4 (4/9) | 22.8 (42/184) | 0.22 |
| Bradykinesia | 11.1 (1/9) | 12.0 (22/184) | > 0.99 |
| Hand clumsiness | 0 (0/9) | 7.1 (13/184) | > 0.99 |
| Tremor | 0 (0/9) | 6.0 (11/184) | > 0.99 |
| Falls | 33.3 (3/9) | 4.9 (9/184) | 0.013 |
| Gait ataxia | 0 (0/9) | 10.3 (19/184) | 0.60 |
| Speech problem | 0 (0/9) | 3.8 (7/184) | > 0.99 |
| Dystonia | 11.1 (1/9) | 0.5 (1/184) | 0.091 |
| Apraxia | 0 (0/9) | 0.5 (1/184) | > 0.99 |
| Cramps | 0 (0/9) | 0.5 (1/184) | > 0.99 |
| Urinary incontinence | 0 (0/9) | 11.4 (21/184) | 0.60 |
| Urinary retention | 0 (0/9) | 4.3 (8/184) | > 0.99 |
| Frequent urination | 0 (0/9) | 1.1 (2/184) | > 0.99 |
| Orthostatic hypotension | 0 (0/9) | 2.2 (4/184) | > 0.99 |
| Dizziness | 0 (0/9) | 6.5 (12/184) | > 0.99 |
| Erectile dysfunction | 0 (0/4) | 23.7 (23/97) | 0.57 |
| Cognitive impairment | 0 (0/9) | 1.6 (3/184) | > 0.99 |
| Paraesthesia | 0 (0/9) | 1.1 (2/184) | > 0.99 |
| Stridor | 0 (0/9) | 0.5 (1/184) | > 0.99 |
| Pathological subtypes | |||
| MSA-SND | 88.9 (8/9) | 46.7 (86/184) | 0.065 |
| MSA-OPCA | 11.1 (1/9) | 20.7 (38/184) | > 0.99 |
| MSA-SND/OPCA | 0 (0/9) | 31.0 (57/184) | 0.24 |
| Minimal change MSA | 0 (0/9) | 1.6 (3/184) | > 0.99 |
| Pathological features | |||
| Brain weight (g) | 1143 ± 150 | 1222 ± 144 | 0.11 |
| Braak NFT stage | 2.4 ± 1.6 | 1.5 ± 1.1 | 0.017 |
| Thal amyloid phase | 2.0 ± 1.4 | 0.9 ± 1.3 | 0.017 |
| Presence of vascular pathology, % (n) | 11.1 (1/9) | 10.3 (19/184) | > 0.99 |
MSA, multiple system atrophy; SND, striatonigral degeneration; OPCA, olivopontocerebellar atrophy; NFT, neurofibrillary tangle