| Pathological diagnosis . | MSA . | PD . | PSP . | ||||
|---|---|---|---|---|---|---|---|
| . | Typical MSA . | PD mimic . | PSP mimic . | Othersa . | Typical PD . | Typical PSP (PSP-RS) . | Typical PSP (PSP-P) . |
| Clinical features | |||||||
| Number of patients (%) | 177 (81.2) | 16 (7.3) | 17 (7.8) | 8 (3.7) | 35 | 35 | 35 |
| Male, % (n) | 54.8 (97/177) | 50 (8/16) | 23.5 (4/17) | 37.5 (3/8) | 45.7 (16/35) | 57.1 (20/35) | 80 (28/35) |
| Age at onset, years, mean ± SD | 56.0 ± 9.0 | 61.9 ± 10.7 | 61.7 ± 5.4 | 56.4 ± 8.5 | 56.1 ± 11.7 | 66.1 ± 7.9 | 62.6 ± 10.4 |
| Age at death, years, mean ± SD | 63.6 ± 8.2 | 68.7 ± 9.5 | 67.5 ± 5.4 | 62.5 ± 9.3 | 78.2 ± 8.1 | 74.3 ± 7.2 | 73.1 ± 10.7 |
| Time to final diagnosis, years, mean ± SD | 4.2 ± 2.5 | 1.3 ± 1.5 | 3.9 ± 1.7 | 4.1 ± 2.3 | 1.7 ± 1.8 | 3.6 ± 2.2 | 6.2 ± 2.6 |
| Disease duration to death, years, mean ± SD | 7.6 ± 3.0 | 7.1 ± 4.1 | 6.4 ± 1.8 | 6.1 ± 2.4 | 21.9 ± 6.6 | 8.0 ± 3.0 | 10.5 ± 4.0 |
| Latency between last examination and death, years, mean ± SD | 1.1 ± 1.1 | 0.8 ± 0.9 | 0.8 ± 0.9 | 0.9 ± 1.0 | 2.1 ± 2.7 | 1.2 ± 1.6 | 1.4 ± 1.7 |
| MSA clinical subtypes | |||||||
| MSA-P, % (n) | 62.1 (110/177) | NA | NA | NA | NA | NA | NA |
| MSA-C, % (n) | 37.9 (67/177) | NA | NA | NA | NA | NA | NA |
| Pathological features | |||||||
| MSA pathological subtype | |||||||
| MSA-SND, % (n) | 29.4 (52/177) | 50 (8/16) | 70.6 (12/17) | 50 (4/8) | NA | NA | NA |
| MSA-OPCA, % (n) | 34.5 (61/177) | 18.8 (3/16) | 0 (0/17) | 12.5 (1/8) | NA | NA | NA |
| MSA-mixed, % (n) | 33.9 (61/177) | 31.3 (5/16) | 29.4 (5/17) | 12.5 (1/8) | NA | NA | NA |
| Minimal change, % (n) | 1.7 (3/177) | 0 (0/16) | 0 (0/17) | 25 (2/8) | – | – | – |
| CERAD plaque score, median (25th, 75th percentile) | 0 (0, 0) | 0 (0, 3) | 0 (0, 1) | 0 (0,0) | 0 (0, 1) | 0 (0, 1) | 0 (0,1) |
| NFT stage, median (25th, 75th percentile) | I (0, I) | 0 (0, 0) | I (0.5, I) | 0 (0,0) | II (I, II) | II (0, II) | II (0, II) |
| Lewy body pathology, % (n) | 7.3 (13/177) | 12.5 (2/16) | 5.9 (1/17) | 0 (0/8) | 100 (35/35) | 22.9 (8/35) | 17.1 (6/35) |
| Brainstem-predominant, % (n) | NA | NA | NA | NA | 0 (0/35) | NA | NA |
| Limbic (transitional), % (n) | NA | NA | NA | NA | 14.3 (5/35) | NA | NA |
| Diffuse neocortical, % (n) | NA | NA | NA | NA | 85.7 (30/35) | NA | NA |
| Pathological diagnosis . | MSA . | PD . | PSP . | ||||
|---|---|---|---|---|---|---|---|
| . | Typical MSA . | PD mimic . | PSP mimic . | Othersa . | Typical PD . | Typical PSP (PSP-RS) . | Typical PSP (PSP-P) . |
| Clinical features | |||||||
| Number of patients (%) | 177 (81.2) | 16 (7.3) | 17 (7.8) | 8 (3.7) | 35 | 35 | 35 |
| Male, % (n) | 54.8 (97/177) | 50 (8/16) | 23.5 (4/17) | 37.5 (3/8) | 45.7 (16/35) | 57.1 (20/35) | 80 (28/35) |
| Age at onset, years, mean ± SD | 56.0 ± 9.0 | 61.9 ± 10.7 | 61.7 ± 5.4 | 56.4 ± 8.5 | 56.1 ± 11.7 | 66.1 ± 7.9 | 62.6 ± 10.4 |
| Age at death, years, mean ± SD | 63.6 ± 8.2 | 68.7 ± 9.5 | 67.5 ± 5.4 | 62.5 ± 9.3 | 78.2 ± 8.1 | 74.3 ± 7.2 | 73.1 ± 10.7 |
| Time to final diagnosis, years, mean ± SD | 4.2 ± 2.5 | 1.3 ± 1.5 | 3.9 ± 1.7 | 4.1 ± 2.3 | 1.7 ± 1.8 | 3.6 ± 2.2 | 6.2 ± 2.6 |
| Disease duration to death, years, mean ± SD | 7.6 ± 3.0 | 7.1 ± 4.1 | 6.4 ± 1.8 | 6.1 ± 2.4 | 21.9 ± 6.6 | 8.0 ± 3.0 | 10.5 ± 4.0 |
| Latency between last examination and death, years, mean ± SD | 1.1 ± 1.1 | 0.8 ± 0.9 | 0.8 ± 0.9 | 0.9 ± 1.0 | 2.1 ± 2.7 | 1.2 ± 1.6 | 1.4 ± 1.7 |
| MSA clinical subtypes | |||||||
| MSA-P, % (n) | 62.1 (110/177) | NA | NA | NA | NA | NA | NA |
| MSA-C, % (n) | 37.9 (67/177) | NA | NA | NA | NA | NA | NA |
| Pathological features | |||||||
| MSA pathological subtype | |||||||
| MSA-SND, % (n) | 29.4 (52/177) | 50 (8/16) | 70.6 (12/17) | 50 (4/8) | NA | NA | NA |
| MSA-OPCA, % (n) | 34.5 (61/177) | 18.8 (3/16) | 0 (0/17) | 12.5 (1/8) | NA | NA | NA |
| MSA-mixed, % (n) | 33.9 (61/177) | 31.3 (5/16) | 29.4 (5/17) | 12.5 (1/8) | NA | NA | NA |
| Minimal change, % (n) | 1.7 (3/177) | 0 (0/16) | 0 (0/17) | 25 (2/8) | – | – | – |
| CERAD plaque score, median (25th, 75th percentile) | 0 (0, 0) | 0 (0, 3) | 0 (0, 1) | 0 (0,0) | 0 (0, 1) | 0 (0, 1) | 0 (0,1) |
| NFT stage, median (25th, 75th percentile) | I (0, I) | 0 (0, 0) | I (0.5, I) | 0 (0,0) | II (I, II) | II (0, II) | II (0, II) |
| Lewy body pathology, % (n) | 7.3 (13/177) | 12.5 (2/16) | 5.9 (1/17) | 0 (0/8) | 100 (35/35) | 22.9 (8/35) | 17.1 (6/35) |
| Brainstem-predominant, % (n) | NA | NA | NA | NA | 0 (0/35) | NA | NA |
| Limbic (transitional), % (n) | NA | NA | NA | NA | 14.3 (5/35) | NA | NA |
| Diffuse neocortical, % (n) | NA | NA | NA | NA | 85.7 (30/35) | NA | NA |
CERAD = the Consortium to Establish a Registry for Alzheimer’s Disease; NA = not applicable; NFT = neurofibrillary tangles; PD = Parkinson’s disease; SD = standard deviation.
Autopsy-confirmed MSA cases with other final clinical diagnoses: corticobasal syndrome (n = 2), vascular parkinsonism (n = 2), atypical parkinsonism of uncertain cause (n = 2), pure autonomic failure (n = 1) and undiagnosed (n = 1).
| Pathological diagnosis . | MSA . | PD . | PSP . | ||||
|---|---|---|---|---|---|---|---|
| . | Typical MSA . | PD mimic . | PSP mimic . | Othersa . | Typical PD . | Typical PSP (PSP-RS) . | Typical PSP (PSP-P) . |
| Clinical features | |||||||
| Number of patients (%) | 177 (81.2) | 16 (7.3) | 17 (7.8) | 8 (3.7) | 35 | 35 | 35 |
| Male, % (n) | 54.8 (97/177) | 50 (8/16) | 23.5 (4/17) | 37.5 (3/8) | 45.7 (16/35) | 57.1 (20/35) | 80 (28/35) |
| Age at onset, years, mean ± SD | 56.0 ± 9.0 | 61.9 ± 10.7 | 61.7 ± 5.4 | 56.4 ± 8.5 | 56.1 ± 11.7 | 66.1 ± 7.9 | 62.6 ± 10.4 |
| Age at death, years, mean ± SD | 63.6 ± 8.2 | 68.7 ± 9.5 | 67.5 ± 5.4 | 62.5 ± 9.3 | 78.2 ± 8.1 | 74.3 ± 7.2 | 73.1 ± 10.7 |
| Time to final diagnosis, years, mean ± SD | 4.2 ± 2.5 | 1.3 ± 1.5 | 3.9 ± 1.7 | 4.1 ± 2.3 | 1.7 ± 1.8 | 3.6 ± 2.2 | 6.2 ± 2.6 |
| Disease duration to death, years, mean ± SD | 7.6 ± 3.0 | 7.1 ± 4.1 | 6.4 ± 1.8 | 6.1 ± 2.4 | 21.9 ± 6.6 | 8.0 ± 3.0 | 10.5 ± 4.0 |
| Latency between last examination and death, years, mean ± SD | 1.1 ± 1.1 | 0.8 ± 0.9 | 0.8 ± 0.9 | 0.9 ± 1.0 | 2.1 ± 2.7 | 1.2 ± 1.6 | 1.4 ± 1.7 |
| MSA clinical subtypes | |||||||
| MSA-P, % (n) | 62.1 (110/177) | NA | NA | NA | NA | NA | NA |
| MSA-C, % (n) | 37.9 (67/177) | NA | NA | NA | NA | NA | NA |
| Pathological features | |||||||
| MSA pathological subtype | |||||||
| MSA-SND, % (n) | 29.4 (52/177) | 50 (8/16) | 70.6 (12/17) | 50 (4/8) | NA | NA | NA |
| MSA-OPCA, % (n) | 34.5 (61/177) | 18.8 (3/16) | 0 (0/17) | 12.5 (1/8) | NA | NA | NA |
| MSA-mixed, % (n) | 33.9 (61/177) | 31.3 (5/16) | 29.4 (5/17) | 12.5 (1/8) | NA | NA | NA |
| Minimal change, % (n) | 1.7 (3/177) | 0 (0/16) | 0 (0/17) | 25 (2/8) | – | – | – |
| CERAD plaque score, median (25th, 75th percentile) | 0 (0, 0) | 0 (0, 3) | 0 (0, 1) | 0 (0,0) | 0 (0, 1) | 0 (0, 1) | 0 (0,1) |
| NFT stage, median (25th, 75th percentile) | I (0, I) | 0 (0, 0) | I (0.5, I) | 0 (0,0) | II (I, II) | II (0, II) | II (0, II) |
| Lewy body pathology, % (n) | 7.3 (13/177) | 12.5 (2/16) | 5.9 (1/17) | 0 (0/8) | 100 (35/35) | 22.9 (8/35) | 17.1 (6/35) |
| Brainstem-predominant, % (n) | NA | NA | NA | NA | 0 (0/35) | NA | NA |
| Limbic (transitional), % (n) | NA | NA | NA | NA | 14.3 (5/35) | NA | NA |
| Diffuse neocortical, % (n) | NA | NA | NA | NA | 85.7 (30/35) | NA | NA |
| Pathological diagnosis . | MSA . | PD . | PSP . | ||||
|---|---|---|---|---|---|---|---|
| . | Typical MSA . | PD mimic . | PSP mimic . | Othersa . | Typical PD . | Typical PSP (PSP-RS) . | Typical PSP (PSP-P) . |
| Clinical features | |||||||
| Number of patients (%) | 177 (81.2) | 16 (7.3) | 17 (7.8) | 8 (3.7) | 35 | 35 | 35 |
| Male, % (n) | 54.8 (97/177) | 50 (8/16) | 23.5 (4/17) | 37.5 (3/8) | 45.7 (16/35) | 57.1 (20/35) | 80 (28/35) |
| Age at onset, years, mean ± SD | 56.0 ± 9.0 | 61.9 ± 10.7 | 61.7 ± 5.4 | 56.4 ± 8.5 | 56.1 ± 11.7 | 66.1 ± 7.9 | 62.6 ± 10.4 |
| Age at death, years, mean ± SD | 63.6 ± 8.2 | 68.7 ± 9.5 | 67.5 ± 5.4 | 62.5 ± 9.3 | 78.2 ± 8.1 | 74.3 ± 7.2 | 73.1 ± 10.7 |
| Time to final diagnosis, years, mean ± SD | 4.2 ± 2.5 | 1.3 ± 1.5 | 3.9 ± 1.7 | 4.1 ± 2.3 | 1.7 ± 1.8 | 3.6 ± 2.2 | 6.2 ± 2.6 |
| Disease duration to death, years, mean ± SD | 7.6 ± 3.0 | 7.1 ± 4.1 | 6.4 ± 1.8 | 6.1 ± 2.4 | 21.9 ± 6.6 | 8.0 ± 3.0 | 10.5 ± 4.0 |
| Latency between last examination and death, years, mean ± SD | 1.1 ± 1.1 | 0.8 ± 0.9 | 0.8 ± 0.9 | 0.9 ± 1.0 | 2.1 ± 2.7 | 1.2 ± 1.6 | 1.4 ± 1.7 |
| MSA clinical subtypes | |||||||
| MSA-P, % (n) | 62.1 (110/177) | NA | NA | NA | NA | NA | NA |
| MSA-C, % (n) | 37.9 (67/177) | NA | NA | NA | NA | NA | NA |
| Pathological features | |||||||
| MSA pathological subtype | |||||||
| MSA-SND, % (n) | 29.4 (52/177) | 50 (8/16) | 70.6 (12/17) | 50 (4/8) | NA | NA | NA |
| MSA-OPCA, % (n) | 34.5 (61/177) | 18.8 (3/16) | 0 (0/17) | 12.5 (1/8) | NA | NA | NA |
| MSA-mixed, % (n) | 33.9 (61/177) | 31.3 (5/16) | 29.4 (5/17) | 12.5 (1/8) | NA | NA | NA |
| Minimal change, % (n) | 1.7 (3/177) | 0 (0/16) | 0 (0/17) | 25 (2/8) | – | – | – |
| CERAD plaque score, median (25th, 75th percentile) | 0 (0, 0) | 0 (0, 3) | 0 (0, 1) | 0 (0,0) | 0 (0, 1) | 0 (0, 1) | 0 (0,1) |
| NFT stage, median (25th, 75th percentile) | I (0, I) | 0 (0, 0) | I (0.5, I) | 0 (0,0) | II (I, II) | II (0, II) | II (0, II) |
| Lewy body pathology, % (n) | 7.3 (13/177) | 12.5 (2/16) | 5.9 (1/17) | 0 (0/8) | 100 (35/35) | 22.9 (8/35) | 17.1 (6/35) |
| Brainstem-predominant, % (n) | NA | NA | NA | NA | 0 (0/35) | NA | NA |
| Limbic (transitional), % (n) | NA | NA | NA | NA | 14.3 (5/35) | NA | NA |
| Diffuse neocortical, % (n) | NA | NA | NA | NA | 85.7 (30/35) | NA | NA |
CERAD = the Consortium to Establish a Registry for Alzheimer’s Disease; NA = not applicable; NFT = neurofibrillary tangles; PD = Parkinson’s disease; SD = standard deviation.
Autopsy-confirmed MSA cases with other final clinical diagnoses: corticobasal syndrome (n = 2), vascular parkinsonism (n = 2), atypical parkinsonism of uncertain cause (n = 2), pure autonomic failure (n = 1) and undiagnosed (n = 1).
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