(A) Diagnostic classification for patients with pathologically confirmed CBD using criteria proposed by Armstrong et al.1 Patients were classified first according to clinical syndromes: ‘probable CBS’, ‘possible CBS’, ‘FBS’, ‘NAV’, ‘PSPS’, and thereafter according to whether they met diagnostic criteria for ‘probable CBD’ and/or ‘possible CBD’. The diagnostic criteria for CBD incorporate the clinical phenotype, for example, FBS or NAV, so a patient with one of these phenotypes and also meeting criteria for probable CBD would be classified as probable CBD and included in the FBS/NAV number in parentheses. (B) Diagnoses given to patients at presentation and final diagnosis during their lifetime
| At presentation | During lifetime | |
|---|---|---|
| (A) Diagnosis | Primary diagnosis | Primary diagnosis |
| Probable CBD | 9 | 12 |
| Possible CBD | 9 (18 including ‘Probable CBD’) | 1 (13 including ‘Probable CBD’) |
| Probable CBS | 3 | 4 |
| Possible CBS | 4 (7) | 6 (10) |
| Frontal behavioural-spatial (FBS) | 0 (12) | 2 (13) |
| FBS–NAV overlap | 0 | 1 |
| Non-fluent/agrammatic variant (NAV) | 0 (11) | 3 (12) |
| PSP phenotype | 1 (1) | 0 (6) |
| (B) Diagnosis | At presentation | During lifetime |
|---|---|---|
| CBD | 10 | 13 |
| PNFA | 2 | 2 |
| FTD | 4 | 4 |
| PSP | 1 | 0 |
| Other | 2 (1 AD, 1 IPD) | 0 |
AD, Alzheimer's disease; CBD, corticobasal degeneration; FTD, frontotemporal dementia; IPD, idiopathic Parkinson's disease; PNFA, progressive non-fluent aphasia; PSP, progressive supranuclear palsy; PSPS, progressive supranuclear palsy syndrome.