Table 1 Clinical features of pathologically confirmed PSP and MSA patients
Disease | Cases | PSP (a total of 10 patients) | Cases | MSA (a total of 13 patients) |
|---|---|---|---|---|
Gender | 10 | 6M/4F | 13 | 8M/5F, 7P/6C |
AAO | 10 | 64.5±8.6 (46–75 years) | 13 | 66.3±6.9 (56–80 years) |
Duration | 10 | 7.4±3.4 (2–12 years) (6.5±3.5 with down-gaze palsy, 9.3±2.5 w/o it) | 13 | 7.5±2.8 (3.5–12 years) |
Last diagnosis | 10 | 7 (70%) PSP; missed 30% (as MSA, CBD, FTDP, no down-gaze palsy) | 13 | 10 (77%) MSA; missed 23% (as atypical or vascular parkinsonism), |
Onset symptom | 10 | 4 (40%) parkinsonism, 4 (40%) fall, 3 (30%) cognitive/behavioral change | 13 | 6 (46%) parkinsonism (all P), 38% (2P 3C) dysautonomia, 4 (31%) ataxia (all C) |
Falls | 10 | 4 (40%) onset, 5 (50%) in 1 year, 7 (70%) in 2 years, 9 (90%) in 3 years | 13 | 3 (23%, 2C 1P) onset, 5 (38%) in 1 year, 11 (85%) in 5 years |
Down-gaze palsy | 10 | 7 (70%) in the course, 2 (20%) with additional horizontal gaze palsy | 13 | 1 (8%) in the course (1C) |
SWJ | 10 | 9 (90%) in the course | 13 | 3 (23%) in the course (1P 2C) |
Dysautonomia | 8 | 8 (at least 80%), only urogenital dysfunction, no OH, none before MO | 13 | 5 (38%) onset; 46, 62, 77% in 1,3,5 years; 23% before motor; 15% (2P) w/o in life |
Dysarthria | 10 | 10 (100%) in the course | 13 | 10 (100%) in the course |
Dysphagia | 4 | 4 (at least 40%) in the course | 8 | 8 (at least 62%) in the course |
FOG | 5 | 5 (at least 50%) in the course | 9 | 6 (at least 46%) in the course |
Apraxia | 5 | 5 (at least 50%) in the course | 7 | None |
Tremor | 7 | 7 (at least 70%) in the course (3 RT with 2 unilateral RT, 3 AT, 2 PT) | 13 | 9 (70%) in the course (AT 54% all P, PT 23% all P, RT 15% with 1P 1C) |
Dystonia | 6 | 5 (at least 50%) in the course | 7 | 6 (at least 46%; 3P 3C) in the course |
Ataxia | 5 | 5 in the course (at least 50%), on average 4.6 year after MO | 12 | 8 in the course (at least 61%, 6C 2P) |
Pyramidal sign | 9 | 3 in the course (at least 30%) | 11 | 4 in the course (at least 30%, 3P 1C) |
MMSE | 6 | 6 (at least 60%), 23.5/30 at first test average 2.7 years after MO | 13 | 12 normal/mild deficits by history or MMSE/mMMSE average 5.0 years after MO |
Stridor | 0 | Not mentioned | 8 | 4 (at least 30%; 1P 3C), all died 2–5 years later |
Myoclonus | 0 | Not mentioned | 5 | 5 (at least 38%; 3P 2C); 2 of them not on medications (1C 1P) |
MRI | 8 | 8 (at least 80%), normal or mild diffuse atrophy, one midbrain atrophy | 9 | 8 (at least 61%, 6C 2P) cerebellar atrophy, pons atrophy (1C 1P) cross bun (1C) |
L-dopa | 8 | 3 (at least 30%) responsive, all with RT, 450–2000 mg/day for 4–7 years | 12 | 7 (at least 54%, 4P 3C) responsive, 200–1300 mg/day, for 2–10 years |
