Table 5.
Rates of progression in cognition by phenotype and clinical trial eligibility
| Clinical diagnosis of PSP |
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|---|---|---|---|---|---|---|---|---|---|---|
| Baseline | All patients | RS | Variant | Cortical | Subcortical | RS vs variant | RS vs cortical | RS vs subcortical | Cortical vs subcortical | |
| Mean ± SD | SPE, 95% CI | SPE, 95% CI | SPE, 95% CI | SPE, 95% CI | SPE, 95% CI | |||||
| (n = 227) | (n = 227) | (n = 174) | (n = 53) | (n = 20) | (n = 28) | P-value | P-value | P-value | P-value | |
| ACE-R | ||||||||||
| Attention | 16.3 ± 2.5 | −0.9 (−1.1 to −0.6) | −1.0 (−1.3 to −0.7) | −0.6 (−1.0 to −0.3) | −0.5 (−0.9 to −0.1) | −0.5 (−1.0 to −0.1) | 0.1 | 0.7 | 0.2 | 1 |
| Memory | 20.3 ± 4.7 | −0.6 (−1.0 to −0.3) | −0.7 (−1.2 to −0.3) | −0.5 (−1.0 to 0.1) | −0.5 (−1.3 to 0.3) | −0.4 (−1.2 to 0.4) | 0.3 | 1 | 0.9 | 1 |
| Fluency | 5.0 ± 3.4 | −0.5 (−0.6 to −0.3) | −0.5 (−0.8 to −0.3) | −0.5 (−0.8 to −0.2) | −0.4 (−0.8 to −0.03) | −0.5 (−0.8 to −0.1) | 0.1 | 1 | 0.07 | 0.9 |
| Language | 22.9 ± 3.7 | −0.9 (−1.2 to −0.6) | −1.2 (−1.6 to −0.8) | −0.3 (−0.6 to −0.1) | −0.4 (−0.7 to −0.1) | −0.3 (−0.6 to −0.03) | 0.0003 | 0.05 | 0.1 | 1 |
| Visuospatial | 12.5 ± 3.4 | −1.7 (−1.9 to −1.4) | −2.0 (−2.3 to −1.6) | −0.9 (−1.4 to −0.5) | −1.3 (−2.0 to −0.5) | −0.6 (−1.2 to −0.01) | 0.002 | 0.4 | 0.001 | 0.8 |
| MMSE Total | 26.1 ± 3.8 | −1.5 (−1.9 to −1.1) | −1.8 (−2.4 to −1.2) | −0.9 (−1.3 to −0.4) | −0.8 (−1.3 to −0.2) | −0.8 (−1.4 to −0.2) | 0.005 | 0.2 | 0.03 | 1 |
| ACE-R total | 77.0 ± 13.6 | −4.6 (−5.4 to −3.7) | −5.3 (−6.4 to −4.2) | −3.0 (−4.2 to −1.8) | −2.9 (−4.6 to −1.3) | −2.5 (−4.2 to −0.8) | 0.01 | 0.3 | 0.0007 | 0.6 |
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| Clinical trial eligibility | ||||||||||
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| Baseline | All patients | Trial | Non-trial | Non-trial RS | Non-trial Variant | Trial vs non-trial | Trial vs non-trial RS | Trial vs non-trial variant | Non-trial variant vs non-trial RS | |
| Mean ± SD | SPE, 95% CI | SPE, 95% CI | SPE, 95% CI | SPE, 95% CI | SPE, 95% CI | |||||
| (n = 227) | (n = 227) | (n = 129) | (n = 98) | (n = 45) | (n = 53) | P-value | P-value | P-value | P-value | |
|
| ||||||||||
| ACE-R | ||||||||||
| Attention | 16.3 ± 2.5 | −0.9 (−1.1 to −0.6) | −0.9 (−1.2 to −0.5) | −0.9 (−1.2 to −0.5) | −1.6 (−2.7 to −0.4) | −0.6 (−1.0 to −0.3) | 0.5 | 1 | 0.2 | 0.05 |
| Memory | 20.3 ± 4.7 | −0.6 (−1.0 to −0.3) | −0.7 (−1.2 to −0.2) | −0.6 (−1.0 to −0.2) | −0.9 (−1.8 to −0.03) | −0.5 (−1.0 to 0.1) | 0.9 | 1 | 0.5 | 0.3 |
| Fluency | 5.0 ± 3.4 | −0.5 (−0.6 to −0.3) | −0.6 (−0.9 to −0.3) | −0.5 (−0.7 to −0.3) | −0.5 (−0.8 to −0.1) | −0.5 (−0.8 to −0.2) | 0.1 | 1 | 0.3 | 1 |
| Language | 22.9 ± 3.7 | −0.9 (−1.2 to −0.6) | −1.1 (−1.6 to −0.7) | −0.7 (−1.1 to −0.3) | −1.6 (−2.7 to −0.6) | −0.3 (−0.6 to −0.1) | 0.4 | 0.2 | 0.1 | 0.002 |
| Visuospatial | 12.5 ± 3.4 | −1.7 (−1.9 to −1.4) | −1.8 (−2.2 to −1.5) | −1.4 (−1.9 to −1.0) | −2.5 (−3.3 to −1.7) | −0.9 (−1.4 to −0.5) | 0.8 | 0.04 | 0.04 | 0.001 |
| MMSE total | 26.1 ± 3.8 | −1.5 (−1.9 to −1.1) | −1.6 (−2.1 to −1.0) | −1.3 (−1.9 to −0.8) | −2.8 (−4.8 to −0.8) | −0.9 (−1.3 to −0.4) | 0.5 | 0.7 | 0.08 | 0.01 |
| ACE-R total | 77.0 ± 13.6 | −4.6 (−5.4 to −3.7) | −4.9 (−6.1 to −3.8) | −4.2 (−5.5 to −2.8) | −6.7 (−10.0 to −3.4) | −3.0 (−4.2 to −1.8) | 0.3 | 1 | 0.01 | 0.01 |
ACE-R = revised Addenbrooke’s Cognitive Examination; MMSE = Mini-Mental State Examination; PSP = progressive supranuclear palsy; RS = Richardson’s Syndrome; SD = standard deviation; Trial cases refers to the subset deemed likely eligible to recent Phase II clinical trial; SPE = 1-year progression estimate.