Table 1 Clinical characteristics of six GAD65 positive-AE patients from our center
| Â | Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 |
|---|---|---|---|---|---|---|
Age/gender | 35/F | 35/M | 29/F | 49/F | 27/F | 33/F |
Past history | Unremarkable | Excision of epileptogenic focus in 2015 | Unremarkable | Unremarkable | Excision of thymoma in 2013 | L Thyroidectomy |
Family history | Unremarkable | Hyperthyroidism of his mother | Unremarkable | Unremarkable | Unremarkable | Unremarkable |
Symptom onset | 2018/08 | 2000/? | 2016/06 | 2000/? | 2009/? | 2014/? |
Initial symptoms | Seizure | Seizure | Seizure | Seizure | Seizure | Seizure |
Seizure types | SPS; CPS; sGTCS | sGTCS; CPS | SPS | CPS; sGTCS | CPS; sGTCS | CPS |
Cognition/MoCA | Impaired/22 | Impaired/22 | Impaired/NA | Impaired/19 | Impaired/23 | Impaired/23 |
Other Symptoms | CI | CI, SPS*, CA, NBC | CI | CI, SPS* | CI | CI |
AEDs | LEV, 1000Â mg/day | LEV, 1000Â mg/day; CBZ, 600Â mg/day; CZP, 1Â mg/day | CBZ, 500Â mg/day | CZP, 4Â mg/q8h; CBZ, 400Â mg/qd | OXC, 300Â mg/q12h; LEV, 250Â mg/q12h | LEV, 625Â mg/q12h; discontinuation by herself later |
Seizure Frequency at the time of Immunotherapy | CPS, 2–3/day; sGTCS, 1–2/month | sGTCS, zero; CPS, 1/day | SPS, 2–6/week | CPS, 2–4/week; sGTCS, 1–2/month | CPS, 1–2/day; sGTCS, 1–2/year | CPS, 2–13/day |
MRI lesions | R MTL, Amy, Hipp | Atrophy of L MTL, Hipp and B cerebellum | B FL | B MTL | B Hipp | L Hipp and Amy |
18F-FDG-PET/CT | Hypometabolism of R TL | NA | Hypometabolism of R Tha, L Hipp and TL | Hypometabolism of L TL | Hypometabolism of L TL | Hypometabolism of L TL |
Others | Thymus residue | No | Thymus residue | No | Thymoma | No |
Immunotherapy | 2018/11 | 2018/11 | 2018/02 | 2019/03 | 2019/07 | 2019/04 |
Comorbidities | No | Hyperthyroidism, LADA | No | No | MG; hyperthyroidism | No |
Serum examination showing positive Abs | No | ANA, anti-mitochondrial M2 subtype abs, anti-Ro-52 abs, TPA, TGA, anti-cardiolipin abs | TPA, TGA | No | TPA | No |
Anti-GAD65 Abs, serum | 1:10 positive | 1:100 positive | NA, positive | 1:320 positive | 1:100 positive | 1:100 positive |
Anti-GAD65 Abs, CSF | 1:100 positive | 1:100 positive | NA, positive | 1:320 positive | 1:320 positive | 1:100 positive |
Immunotherapy | IVMP and prednisone tapered; IVIG | IVIG twice, and MF | IVIG and prednisone tapered | IVMP and IVIG, medrol tapered | IVMP and IVIG, prednisone tapered | IVMP and IVIG, prednisone tapered |
Outcome (2019.07) | CPS, 2–3/month | CPS, 3–4/month | NA | no more seizures; SPS* improved | no more CPS and sGTCS | CPS, 1–2/day |