Demographic and clinical characteristics of patients with pathologically proven MSA
| Clinical features . | MSA patients with NC . | MSA patients with FSD . | MSA patients with MI . | MSA patients with MI + FSD . | P . |
|---|---|---|---|---|---|
| Number of patients (%) | 118 (79.7) | 12 (8.1) | 6 (4.1) | 12 (8.1) | |
| Male, % (n) | 50.8 (60/118) | 58.3 (7/12) | 33.3 (2/6) | 58.3 (7/12) | NS |
| Age of onset, mean ± SD (range) | 57.8 ± 9.2 (41–78) | 56.6 ± 5.9 (46–66) | 55.8 ± 7.6 (44–64) | 57.0 ± 5.9 (44–64) | NS |
| Age at death, mean ± SD (range) | 65.0 ± 8.5 (44–83) | 63.9 ± 6.0 (53–73) | 64.7 ± 4.8 (57–70) | 65.1 ± 5.7 (51–71) | NS |
| Disease duration, years, mean ± SD (range) | 7.2 ± 3.0 (3–18) | 7.3 ± 1.8 (5–11) | 8.8 ± 6.9 (3–22) | 8.1 ± 6.9 (3–13) | NS |
| Diagnostic accuracy, % (n) | 88.1 (104/118) | 75 (9/12) | 83.3 (5/6) | 83.3 (10/12) | NS |
| Onset of cognitive impairment (years), mean ± SD (range) | NA | 3.7 ± 2.7 (0–9) | 4.9 ± 4.9 (0–14) | 3.7 ± 2.5 (0–9) | NS |
| Family history of cognitive impairment, % (n) | 5.1 (6/118) | 8.3 (1/12) | 33.3 (2/6) | 8.3 (1/12) | NS |
| MMSE, mean ± SD (number of patients who had MMSE) | 28.3 ± 1.3 (9) | 26.8 ± 1.3 (6) | 29 (1) | 25.5 ± 2.6 (4) | NA |
| Final diagnosis of misdiagnosed cases | PSP (9); CBS (1); vascular parkinsonism (1); undiagnosed (1) | PSP (2); typical parkinsonism (1) | Parkinson's disease (1) | CBS (1); pure autonomic failure (1) | |
| Clinical subtype | |||||
| MSA-P, % (n) | 59.6 (62/104) | 22.2 (2/9) | 40 (2/5) | 50 (5/10) | NS |
| MSA-C, % (n) | 40.4 (42/104) | 77.8 (7/9) | 60 (3/5) | 50 (5/10) | NS |
| Depression, % (n) | 43.2 (51/118) | 50 (6/12) | 33.3 (2/6) | 25 (3/12) | NS |
| Hallucination, % (n) | 7.6 (9/118) | 8.3 (1/12) | 0 (0/6) | 16.7 (2/12) | NS |
| REM sleep behaviour disorder, % (n) | 50.8 (60/118) | 50 (6/12) | 33.3 (2/6) | 50 (6/12) | NS |
| Urinary incontinence, % (n) | 73.7 (87/118) | 75 (9/12) | 100 (6/6) | 75 (9/12) | NS |
| Orthostatic hypotension, % (n) | 49.2 (58/118) | 66.7 (8/12) | 66.7 (4/6) | 75 (9/12) | NS |
| Pathological features | |||||
| Brain weight, g, mean ± SD | 1343.3 ± 152.8 | 1310.6 ± 133.4 | 1204 ± 211.6 | 1309.8 ± 174.9 | NS |
| MSA pathological subgroup | |||||
| MSA-SND, % (n) | 38.1 (45/118) | 16.7 (2/12) | 0 (0/6) | 25 (3/12) | NS |
| MSA-OPCA, % (n) | 28.8 (34/118) | 50 (6/12) | 50 (3/6) | 66.7 (8/12) | NS |
| MSA-mixed, % (n) | 33.1 (39/118) | 33.3 (4/12) | 50 (3/6) | 8.3 (1/12) | NS |
| CERAD plaque score, median (25th, 75th %tile) | 0 (0, 0.25) | 0 (0, 0) | 0 (0, 0.5) | 0 (0, 0.75) | NS |
| Braak NFT stage, median (25th, 75th %tile) | I (0, I) | I (0, I) | 0 (0, II) | I (0, I) | NS |
| Lewy body pathology, % (n) | 7.6 (9/118) | 0 (0/12) | 16.7 (1/6) | 8.3 (1/12) | NS |
| Cerebral vascular diseasea | 3.7 (4/118) | 0 (0/12) | 0 (0/6) | 0 (0/12) | NS |
| Clinical features . | MSA patients with NC . | MSA patients with FSD . | MSA patients with MI . | MSA patients with MI + FSD . | P . |
|---|---|---|---|---|---|
| Number of patients (%) | 118 (79.7) | 12 (8.1) | 6 (4.1) | 12 (8.1) | |
| Male, % (n) | 50.8 (60/118) | 58.3 (7/12) | 33.3 (2/6) | 58.3 (7/12) | NS |
| Age of onset, mean ± SD (range) | 57.8 ± 9.2 (41–78) | 56.6 ± 5.9 (46–66) | 55.8 ± 7.6 (44–64) | 57.0 ± 5.9 (44–64) | NS |
| Age at death, mean ± SD (range) | 65.0 ± 8.5 (44–83) | 63.9 ± 6.0 (53–73) | 64.7 ± 4.8 (57–70) | 65.1 ± 5.7 (51–71) | NS |
| Disease duration, years, mean ± SD (range) | 7.2 ± 3.0 (3–18) | 7.3 ± 1.8 (5–11) | 8.8 ± 6.9 (3–22) | 8.1 ± 6.9 (3–13) | NS |
| Diagnostic accuracy, % (n) | 88.1 (104/118) | 75 (9/12) | 83.3 (5/6) | 83.3 (10/12) | NS |
| Onset of cognitive impairment (years), mean ± SD (range) | NA | 3.7 ± 2.7 (0–9) | 4.9 ± 4.9 (0–14) | 3.7 ± 2.5 (0–9) | NS |
| Family history of cognitive impairment, % (n) | 5.1 (6/118) | 8.3 (1/12) | 33.3 (2/6) | 8.3 (1/12) | NS |
| MMSE, mean ± SD (number of patients who had MMSE) | 28.3 ± 1.3 (9) | 26.8 ± 1.3 (6) | 29 (1) | 25.5 ± 2.6 (4) | NA |
| Final diagnosis of misdiagnosed cases | PSP (9); CBS (1); vascular parkinsonism (1); undiagnosed (1) | PSP (2); typical parkinsonism (1) | Parkinson's disease (1) | CBS (1); pure autonomic failure (1) | |
| Clinical subtype | |||||
| MSA-P, % (n) | 59.6 (62/104) | 22.2 (2/9) | 40 (2/5) | 50 (5/10) | NS |
| MSA-C, % (n) | 40.4 (42/104) | 77.8 (7/9) | 60 (3/5) | 50 (5/10) | NS |
| Depression, % (n) | 43.2 (51/118) | 50 (6/12) | 33.3 (2/6) | 25 (3/12) | NS |
| Hallucination, % (n) | 7.6 (9/118) | 8.3 (1/12) | 0 (0/6) | 16.7 (2/12) | NS |
| REM sleep behaviour disorder, % (n) | 50.8 (60/118) | 50 (6/12) | 33.3 (2/6) | 50 (6/12) | NS |
| Urinary incontinence, % (n) | 73.7 (87/118) | 75 (9/12) | 100 (6/6) | 75 (9/12) | NS |
| Orthostatic hypotension, % (n) | 49.2 (58/118) | 66.7 (8/12) | 66.7 (4/6) | 75 (9/12) | NS |
| Pathological features | |||||
| Brain weight, g, mean ± SD | 1343.3 ± 152.8 | 1310.6 ± 133.4 | 1204 ± 211.6 | 1309.8 ± 174.9 | NS |
| MSA pathological subgroup | |||||
| MSA-SND, % (n) | 38.1 (45/118) | 16.7 (2/12) | 0 (0/6) | 25 (3/12) | NS |
| MSA-OPCA, % (n) | 28.8 (34/118) | 50 (6/12) | 50 (3/6) | 66.7 (8/12) | NS |
| MSA-mixed, % (n) | 33.1 (39/118) | 33.3 (4/12) | 50 (3/6) | 8.3 (1/12) | NS |
| CERAD plaque score, median (25th, 75th %tile) | 0 (0, 0.25) | 0 (0, 0) | 0 (0, 0.5) | 0 (0, 0.75) | NS |
| Braak NFT stage, median (25th, 75th %tile) | I (0, I) | I (0, I) | 0 (0, II) | I (0, I) | NS |
| Lewy body pathology, % (n) | 7.6 (9/118) | 0 (0/12) | 16.7 (1/6) | 8.3 (1/12) | NS |
| Cerebral vascular diseasea | 3.7 (4/118) | 0 (0/12) | 0 (0/6) | 0 (0/12) | NS |
CBS = corticobasal syndrome; CERAD = the Consortium to Establish a Registry for Alzheimer’s Disease; MI = memory impairment; MMSE = Mini-Mental State Examination; MSA-C = MSA with predominant cerebellar ataxia; MSA-P = MSA with predominant parkinsonism; NA = not available; NC = normal cognition; NFT = neurofibrillary tangle; NS = not significant; PSP = progressive supranuclear palsy; RBD = REM sleep behaviour disorder; SD = standard deviation.
Moderate or severe risk of contributing to cognitive impairment.
Demographic and clinical characteristics of patients with pathologically proven MSA
| Clinical features . | MSA patients with NC . | MSA patients with FSD . | MSA patients with MI . | MSA patients with MI + FSD . | P . |
|---|---|---|---|---|---|
| Number of patients (%) | 118 (79.7) | 12 (8.1) | 6 (4.1) | 12 (8.1) | |
| Male, % (n) | 50.8 (60/118) | 58.3 (7/12) | 33.3 (2/6) | 58.3 (7/12) | NS |
| Age of onset, mean ± SD (range) | 57.8 ± 9.2 (41–78) | 56.6 ± 5.9 (46–66) | 55.8 ± 7.6 (44–64) | 57.0 ± 5.9 (44–64) | NS |
| Age at death, mean ± SD (range) | 65.0 ± 8.5 (44–83) | 63.9 ± 6.0 (53–73) | 64.7 ± 4.8 (57–70) | 65.1 ± 5.7 (51–71) | NS |
| Disease duration, years, mean ± SD (range) | 7.2 ± 3.0 (3–18) | 7.3 ± 1.8 (5–11) | 8.8 ± 6.9 (3–22) | 8.1 ± 6.9 (3–13) | NS |
| Diagnostic accuracy, % (n) | 88.1 (104/118) | 75 (9/12) | 83.3 (5/6) | 83.3 (10/12) | NS |
| Onset of cognitive impairment (years), mean ± SD (range) | NA | 3.7 ± 2.7 (0–9) | 4.9 ± 4.9 (0–14) | 3.7 ± 2.5 (0–9) | NS |
| Family history of cognitive impairment, % (n) | 5.1 (6/118) | 8.3 (1/12) | 33.3 (2/6) | 8.3 (1/12) | NS |
| MMSE, mean ± SD (number of patients who had MMSE) | 28.3 ± 1.3 (9) | 26.8 ± 1.3 (6) | 29 (1) | 25.5 ± 2.6 (4) | NA |
| Final diagnosis of misdiagnosed cases | PSP (9); CBS (1); vascular parkinsonism (1); undiagnosed (1) | PSP (2); typical parkinsonism (1) | Parkinson's disease (1) | CBS (1); pure autonomic failure (1) | |
| Clinical subtype | |||||
| MSA-P, % (n) | 59.6 (62/104) | 22.2 (2/9) | 40 (2/5) | 50 (5/10) | NS |
| MSA-C, % (n) | 40.4 (42/104) | 77.8 (7/9) | 60 (3/5) | 50 (5/10) | NS |
| Depression, % (n) | 43.2 (51/118) | 50 (6/12) | 33.3 (2/6) | 25 (3/12) | NS |
| Hallucination, % (n) | 7.6 (9/118) | 8.3 (1/12) | 0 (0/6) | 16.7 (2/12) | NS |
| REM sleep behaviour disorder, % (n) | 50.8 (60/118) | 50 (6/12) | 33.3 (2/6) | 50 (6/12) | NS |
| Urinary incontinence, % (n) | 73.7 (87/118) | 75 (9/12) | 100 (6/6) | 75 (9/12) | NS |
| Orthostatic hypotension, % (n) | 49.2 (58/118) | 66.7 (8/12) | 66.7 (4/6) | 75 (9/12) | NS |
| Pathological features | |||||
| Brain weight, g, mean ± SD | 1343.3 ± 152.8 | 1310.6 ± 133.4 | 1204 ± 211.6 | 1309.8 ± 174.9 | NS |
| MSA pathological subgroup | |||||
| MSA-SND, % (n) | 38.1 (45/118) | 16.7 (2/12) | 0 (0/6) | 25 (3/12) | NS |
| MSA-OPCA, % (n) | 28.8 (34/118) | 50 (6/12) | 50 (3/6) | 66.7 (8/12) | NS |
| MSA-mixed, % (n) | 33.1 (39/118) | 33.3 (4/12) | 50 (3/6) | 8.3 (1/12) | NS |
| CERAD plaque score, median (25th, 75th %tile) | 0 (0, 0.25) | 0 (0, 0) | 0 (0, 0.5) | 0 (0, 0.75) | NS |
| Braak NFT stage, median (25th, 75th %tile) | I (0, I) | I (0, I) | 0 (0, II) | I (0, I) | NS |
| Lewy body pathology, % (n) | 7.6 (9/118) | 0 (0/12) | 16.7 (1/6) | 8.3 (1/12) | NS |
| Cerebral vascular diseasea | 3.7 (4/118) | 0 (0/12) | 0 (0/6) | 0 (0/12) | NS |
| Clinical features . | MSA patients with NC . | MSA patients with FSD . | MSA patients with MI . | MSA patients with MI + FSD . | P . |
|---|---|---|---|---|---|
| Number of patients (%) | 118 (79.7) | 12 (8.1) | 6 (4.1) | 12 (8.1) | |
| Male, % (n) | 50.8 (60/118) | 58.3 (7/12) | 33.3 (2/6) | 58.3 (7/12) | NS |
| Age of onset, mean ± SD (range) | 57.8 ± 9.2 (41–78) | 56.6 ± 5.9 (46–66) | 55.8 ± 7.6 (44–64) | 57.0 ± 5.9 (44–64) | NS |
| Age at death, mean ± SD (range) | 65.0 ± 8.5 (44–83) | 63.9 ± 6.0 (53–73) | 64.7 ± 4.8 (57–70) | 65.1 ± 5.7 (51–71) | NS |
| Disease duration, years, mean ± SD (range) | 7.2 ± 3.0 (3–18) | 7.3 ± 1.8 (5–11) | 8.8 ± 6.9 (3–22) | 8.1 ± 6.9 (3–13) | NS |
| Diagnostic accuracy, % (n) | 88.1 (104/118) | 75 (9/12) | 83.3 (5/6) | 83.3 (10/12) | NS |
| Onset of cognitive impairment (years), mean ± SD (range) | NA | 3.7 ± 2.7 (0–9) | 4.9 ± 4.9 (0–14) | 3.7 ± 2.5 (0–9) | NS |
| Family history of cognitive impairment, % (n) | 5.1 (6/118) | 8.3 (1/12) | 33.3 (2/6) | 8.3 (1/12) | NS |
| MMSE, mean ± SD (number of patients who had MMSE) | 28.3 ± 1.3 (9) | 26.8 ± 1.3 (6) | 29 (1) | 25.5 ± 2.6 (4) | NA |
| Final diagnosis of misdiagnosed cases | PSP (9); CBS (1); vascular parkinsonism (1); undiagnosed (1) | PSP (2); typical parkinsonism (1) | Parkinson's disease (1) | CBS (1); pure autonomic failure (1) | |
| Clinical subtype | |||||
| MSA-P, % (n) | 59.6 (62/104) | 22.2 (2/9) | 40 (2/5) | 50 (5/10) | NS |
| MSA-C, % (n) | 40.4 (42/104) | 77.8 (7/9) | 60 (3/5) | 50 (5/10) | NS |
| Depression, % (n) | 43.2 (51/118) | 50 (6/12) | 33.3 (2/6) | 25 (3/12) | NS |
| Hallucination, % (n) | 7.6 (9/118) | 8.3 (1/12) | 0 (0/6) | 16.7 (2/12) | NS |
| REM sleep behaviour disorder, % (n) | 50.8 (60/118) | 50 (6/12) | 33.3 (2/6) | 50 (6/12) | NS |
| Urinary incontinence, % (n) | 73.7 (87/118) | 75 (9/12) | 100 (6/6) | 75 (9/12) | NS |
| Orthostatic hypotension, % (n) | 49.2 (58/118) | 66.7 (8/12) | 66.7 (4/6) | 75 (9/12) | NS |
| Pathological features | |||||
| Brain weight, g, mean ± SD | 1343.3 ± 152.8 | 1310.6 ± 133.4 | 1204 ± 211.6 | 1309.8 ± 174.9 | NS |
| MSA pathological subgroup | |||||
| MSA-SND, % (n) | 38.1 (45/118) | 16.7 (2/12) | 0 (0/6) | 25 (3/12) | NS |
| MSA-OPCA, % (n) | 28.8 (34/118) | 50 (6/12) | 50 (3/6) | 66.7 (8/12) | NS |
| MSA-mixed, % (n) | 33.1 (39/118) | 33.3 (4/12) | 50 (3/6) | 8.3 (1/12) | NS |
| CERAD plaque score, median (25th, 75th %tile) | 0 (0, 0.25) | 0 (0, 0) | 0 (0, 0.5) | 0 (0, 0.75) | NS |
| Braak NFT stage, median (25th, 75th %tile) | I (0, I) | I (0, I) | 0 (0, II) | I (0, I) | NS |
| Lewy body pathology, % (n) | 7.6 (9/118) | 0 (0/12) | 16.7 (1/6) | 8.3 (1/12) | NS |
| Cerebral vascular diseasea | 3.7 (4/118) | 0 (0/12) | 0 (0/6) | 0 (0/12) | NS |
CBS = corticobasal syndrome; CERAD = the Consortium to Establish a Registry for Alzheimer’s Disease; MI = memory impairment; MMSE = Mini-Mental State Examination; MSA-C = MSA with predominant cerebellar ataxia; MSA-P = MSA with predominant parkinsonism; NA = not available; NC = normal cognition; NFT = neurofibrillary tangle; NS = not significant; PSP = progressive supranuclear palsy; RBD = REM sleep behaviour disorder; SD = standard deviation.
Moderate or severe risk of contributing to cognitive impairment.
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