Table 2.
Immunological profiles of individuals with stiff person syndrome spectrum disorders
| Overall (n = 227) |
Classic SPS (n = 154) |
SPS Plus (n = 48) |
PERM (n = 16) |
Partial SPS (n = 9) |
|
|---|---|---|---|---|---|
| Serum Anti-GAD65 + , n (%) | 197 (86.8) | 130 (84.4) | 45 (93.8) | 15 (93.8) | 7 (77.8) |
| Quest and Hopkins*, n (%) | 175 | 114 | 42 | 15 | 4 |
| < 10,000 IU/mL | 80 (45.7) | 58 (50.9) | 12 (28.6) | 6 (40.0) | 2 (50.0) |
| > = 10,000 IU/mL | 95 (54.3) | 56 (49.1) | 28 (71.4) | 9 (60.0) | 2 (50.0) |
| Mayo Labs^, n (%) | 29 | 22 | 5 | 1 | 1 |
| < 20 nmol/L | 10 (34.5) | 9 (40.9) | 0 | 1 (100) | 0 |
| >= 20 nmol/L | 19 (65.5) | 13 (59.1) | 5 (100) | 0 | 1 (100) |
| Quest and Hopkins*, n (%) | 175 | 114 | 42 | 15 | 4 |
| 1st tertile (<117) | 27 (15.4) | 20 (17.5) | 5 (11.9) | 1 (6.7) | 1 (25.0) |
| 2nd tertile (117–24,576) | 61 (34.9) | 45 (39.5) | 10 (23.8) | 5 (33.3) | 1 (25.0) |
| 3rd tertile (24,576–2,281,100) | 87 (49.7) | 49 (43.0) | 27 (64.3) | 9 (60.0) | 2 (50.0) |
| Mayo Labs^, n (%) | 29 | 22 | 5 | 1 | 1 |
| 1st tertile (< 44.7) | 11 (37.9) | 9 (40.9) | 0 | 1 (100) | 1 (100) |
| 2nd tertile (44.7–289.7) | 8 (27.6) | 6 (27.3) | 2 (40.0) | 0 | 0 |
| 3rd tertile (289.7–1,587) | 10 (34.5) | 7 (31.8) | 3 (60.0) | 0 | 0 |
| CSF anti-GAD65 + , n (%) | 45/72 (62.5) | 27/50 (54.0) | 13/14 (92.9) | 3/4 (75.0) | 2/4 (50.0) |
| Anti-amphiphysin, n (%) | 6 (2.6) | 6 (3.9) | 0 | 0 | 0 |
| Anti-glycine receptor, n (%) | 11 (4.8) | 9 (5.8) | 1 (2.1) | 1 (6.2) | 0 |
| CSF restricted oligoclonal banding, n (%) | 7/99 (7.1) | 3/65 (4.6) | 1/24 (4.2) | 2/6 (33.3) | 1/4 (25.0) |
| CSF elevated IgG index, n (%) | 8/54 (14.8) | 6/36 (16.7) | 1/11 (9.1) | 0/3 | 1/4 (25.0) |
SPS stiff person syndrome, PERM progressive encephalomyelitis with rigidity and myoclonus, GAD glutamic acid decarboxylase, CSF cerebrospinal fluid, Ig immunoglobulin, n sample size
*Enzyme Linked Immunoassay (ELISA), units International Units (IU)/mL
^Radioimmunoassay (RIA), units nmol/mL.