Table 1.
Characteristic | Value |
---|---|
Male, % (n/n) | 67% (33/49) |
Age at onset, median [IQR], years | 56.1 [50.5 – 60.9] |
Age at death, median [IQR], years | 65.7 [58.9 – 70.4] |
Disease duration, median [IQR], years | 8.6 [5.7 – 12.9] |
Predominant motor phenotype | |
MSA-P, % (n/n) | 65 (32/49) |
MAS-C, % (n/n) | 35 (17/49) |
Initial manifestation | |
Autonomic, % (n/n) | 50 (23/47) |
Parkinsonism, % (n/n) | 30 (14/47) |
Cerebellar, % (n/n) | 20 (10/47) |
Early motor disability | |
Frequent falls (≤ 3 years from onset), n/n | 11/35 |
Aid-requiring walking (≤ 3 years from onset), n/n | 7/28 |
Early respiratory impairment | |
Stridor (≤ 3 years from onset), n/n | 4/6 |
Early autonomic failure | |
Orthostatism (≤ 1 year from onset), n/n | 13/31 |
Bladder symptoms (≤ 1 year from onset), n/n | 15/37 |
Bladder catheterization (≤ 3 years from onset), n/n | 8/22 |
TST anhidrosis ≥ 40% (≤ 3 years from onset), n/n | 8/15 |
CASS ≥ 6 (≤ 3 years from onset), n/n | 13/21 |
Age at disease onset was available in 46 out of 49 patients. Frequent falls were defined as at least 2 falls per year or documentation of “frequent”, “several”, or “consistent” falls not attributed to orthostatism. Orthostatism was defined as history of presyncope/ syncope or evidence of orthostatic hypotension. CASS, composite autonomic severity score (CASS ≥ 6 indicates laboratory evidence of severe generalized autonomic failure); MSA-P, parkinsonian type MSA; MSA-C, cerebellar type MSA; TST, thermoregulatory sweat test (anhidrosis is reported as percentage of body surface not sweating; TST anhidrosis ≥ 40% indicates moderate-to-severe anhidrosis)