Table 3.
Most common pertinent clinical/examination characteristics/findings of stiff person syndrome spectrum disorders
| Overall (n = 227) | Classic SPS (n = 154) | SPS Plus (n = 48) | PERM (n = 16) | Partial SPS (n = 9) | |
|---|---|---|---|---|---|
| Axial stiffness and spasms, n (%) | 212 (92.5) | 147 (95.5) | 44 (91.7) | 14 (87.5) | 5 (55.6) |
| Appendicular stiffness and spasms, n (%) | 217 (95.6) | 148 (96.1) | 46 (95.8) | 15 (93.8) | 8 (88.9) |
| Hyperlordosis, n (%) | 106 (46.7) | 72 (46.8) | 25 (52.1) | 7 (43.8) | 1 (11.1) |
| Hyperreflexia, n (%) | 165 (72.7) | 108 (70.1) | 34 (70.8) | 12 (75.0) | 7 (77.8) |
| Hypertonia, n (%) | 196 (86.3) | 131 (85.1) | 41 (91.1) | 14 (87.5) | 6 (66.7) |
| Incoordination, n (%) | 121 (51.5) | 63 (40.9) | 36 (80.0) | 10 (62.5) | 3 (33.3) |
| Diplopia, n (%) | 54 (23.8) | 17 (11.0) | 31 (64.6) | 5 (31.2) | 1 (11.1) |
| Vertigo, n (%) | 60 (26.4) | 26 (16.9) | 26 (54.2) | 7 (43.8) | 1 (11.1) |
| Ocular motor dysfunction, n (%) | 64 (28.2) | 15 (9.7) | 35 (72.9) | 13 (81.3) | 1 (11.1) |
| Photosensitivity, n (%) | 57 (25.1) | 30 (19.5) | 20 (41.7) | 6 (37.5) | 1 (11.1) |
| Cognitive symptoms, n (%) | 102 (44.9) | 71 (46.1) | 19 (39.6) | 9 (56.2) | 3 (33.3) |
| Need for ambulation device use, n (%) | 164 (72.2) | 106 (68.8) | 39 (79.2) | 15 (93.8) | 7 (77.8) |
| Unilateral support | 52 (22.9) | 33 (21.4) | 17 (37.8) | 2 (12.5) | 0 |
| Bilateral support | 66 (29.1) | 41 (26.6) | 14 (29.2) | 6 (37.5) | 5 (55.6) |
| Wheelchair | 25 (11.0) | 9 (5.8) | 8 (16.7) | 7 (43.8) | 1 (11.1) |
| Unspecified support device | 7 (3.1) | 23 (14.9) | 0 | 0 | 1 (11.1) |
| Hypersensitivity triggers, n (%) | |||||
| Agoraphobia | 87 (38.3) | 59 (38.3) | 18 (37.5) | 9 (56.2) | 1 (11.1) |
| Startle | 130 (57.3) | 90 (58.4) | 26 (54.2) | 11 (68.8) | 3 (33.3) |
| Cold | 129 (56.8) | 90 (58.4) | 25 (52.1) | 10 (62.5) | 4 (44.4) |
| Open space | 84 (37.0) | 62 (40.3) | 15 (31.2) | 6 (37.5) | 1 (11.1) |
| Stress | 168 (74.0) | 118 (76.6) | 32 (66.7) | 13 (81.2) | 5 (55.6) |
| Noise | 141 (62.1) | 100 (64.9) | 11 (68.8) | 3 (33.3) | |
| EMG findings of co-contraction, continuous motor unit activity, n (%) |
37/158 (23.4) |
27/113 (23.9) |
5/29 (17.2) |
3/10 (30.0) |
2/5 (40.0) |
SPS stiff person syndrome, PERM progressive encephalomyelitis with rigidity and myoclonus, n sample size, EMG electromyography
Included symptoms/signs were based on frequency of occurrence being more than 20% of entire cohort.