Table 1.
Clinical characteristic | Low-titer GAD65 Antibodies (n = 6) |
High-titer GAD65 Antibodies (n = 24) |
p Value |
Female (%) | 3 (50) | 20 (83) | 0.12 |
Age at onset, y, median (range) | 43.5 (26 to 83) | 52.5 (23 to 71) | 0.42 |
Time from symptom onset to diagnosis, mo, median (range) | 2.5 (2 to 48) | 13 (1 to 96) | 0.01 |
Clinical phenotype (%) | 1.00 | ||
Classic SPS | 4 (67) | 17 (71) | |
SLS | 2 (33) | 7 (29) | |
PERM | 0 (0) | 0 (0) | |
Type 1 DM (%) | 3 (50) | 10 (42) | 0.53 |
Other autoimmune diseases (%) | 1 (17) | 10 (42) | 0.26 |
Malignancy (%) | 2 (33) | 2 (8) | 0.17 |
Lack of antagonist inhibition on EMGa | 5/5 (100) | 8/10 (80) | 0.43 |
CSF pleocytosis | 0/6 (0) | 2/16 (13) | 0.52 |
Elevated CSF protein | 2/5 (29) | 3/15 (20) | 0.37 |
mRS at baseline, median (range) | 3.5 (2 to 4) | 4 (1 to 5) | 0.15 |
Follow-up period, m, median (range) | 47 (2 to 120) | 44 (15 to 192) | 0.98 |
mRS at the last follow-up, median (range) | 2.5 (0 to 6) | 2 (0 to 4) | 0.56 |
Change in mRS, median (range) | 0.5 (−2 to 2) | 2 (0 to 5) | 0.09 |
Abbreviations: DM = diabetes mellitus; GAD65 = glutamic acid decarboxylase 65; mRS = modified Rankin Scale; PERM = progressive encephalomyelitis with rigidity and myoclonus; SLS = stiff-limb syndrome.
Lack of antagonist inhibition on EMG is defined by the continuous co-contraction of agonist and antagonist muscles (with inability to relax) as confirmed by EMG.