Logistic regression analysis assessing predictors of poor outcome (MRS >2) at last clinical follow-up in 212 patients with GAD65 neurological autoimmunity
| Variable | Median (range) or frequency (%) | OR (95% CI) | Univariate P value | Multivariate P value |
| Age at symptom onset in years | 46 (5–83) | 1.04 (1.02 to 1.06) | <0.0001 | – |
| Male | 49/212 (23) | 0.65 (0.34 to 1.24) | 0.19 | – |
| Acute immunotherapy* | 151/212 (71) | 1.72 (0.94 to 3.13) | 0.08 | – |
| Second-line acute immunotherapy† | 23/152 (15) | 1.6 (0.6 to 4) | 0.3 | – |
| Maintenance immunotherapy‡ | 90/212 (42) | 1.33 (0.77 to 2.32) | 0.31 | – |
| Time from symptom onset to first immunotherapy in months | 21 (1–540) | 0.987 (0.979 to 0.995) | <0.0001 | – |
| Epilepsy (with or without LE) | 62/212 (29) | 0.17 (0.09 to 0.32) | <0.0001 | – |
| Cerebellar ataxia | 91/212 (43) | 4.58 (2.49 to 8.39) | <0.0001 | 0.016 |
| Stiff-person spectrum disorder | 107/212 (50) | 1.35 (0.78 to 2.34) | 0.28 | – |
| Overlap syndrome | 44/212 (21) | 1.41 (0.71 to 2.8) | 0.32 | – |
| Serum GAD65 titre in nmol/L | 534 (20.1–7558) | 1 (0.99 to 1) | 0.32 | – |
| Serum GAD65 titre >500 nmol/L | 110/212 (52) | 1.89 (1.09 to 3.28) | 0.02 | 0.007 |
| Diabetes mellitus | 87/212 (41) | 1.21 (0.69 to 2.1) | 0.51 | – |
| mRS at first Mayo Clinic evaluation (range) | 3 (0–6) | 5.95 (3.74 to 9.45) | <0.0001 | <0.0001 |
| Total symptom duration recorded in months | 74 (3–636) | 0.996 (0.993 to 0.999) | 0.006 | – |
| mRS at last follow-up | 3 (0–6) | – | – | – |
| mRS >2 at last follow-up | 121/212 (57) | – | – | – |
*Acute immunotherapies used for treatment of GAD65 neurological autoimmunity included corticosteroids, intravenous immunoglobulin, plasma exchange, rituximab, cyclophosphamide and/or autologous stem cell transplantation; frequency stratified by core disease manifestation is reported in table 3.
†Second-line acute immunotherapy refers to use of rituximab and/or cyclophosphamide among patients who received acute immunotherapy.
‡Maintenance immunotherapies used for chronic immunomodulation included mycophenolate mofetil (n=37), azathioprine (n=27), intravenous IG (n=27), rituximab (n=19), corticosteroids (n=7), methotrexate (n=2), tacrolimus (n=1) and sirolimus (n=1).
GAD65, glutamic acid decarboxylase-65; LE, limbic encephalitis; mRS, modified Rankin Scale.