Demographic data, presentation, treatment and follow-up
| Patient no. | Sex/age at onset (years) | Symptoms | MRI:* latency (days) – mediotemporal atrophy | CSF: latency (days) – abnormal results | Abs: latency (months) – type with serum concentration or titre as available | Steroid therapy: latency (months) – dose (mg/kg MP-eq) – duration (months) | IVIG therapy: latency (months) – dose (g/kg) | Follow-up MRI: latency (months) – mediotemporal atrophy | Disease duration (months) – outcome |
|---|---|---|---|---|---|---|---|---|---|
| 1 | F/3 | Behavioural change, ataxia | 8 – no | 8 – OCB | 1 – Hu | – | 0.5 – 2 | 8 – no | 9 – ataxia still severe |
| 2 | F/14 | Depression, learning difficulties, TLE, epilepsia partialis continua linqualis, status dystonicus | 69 – no | 175 – OCB | 9 – Ma1, Ma2 | 4 – ? – 13 | 6 – 2 | 8 – bi | 26 – vegetative state |
| 3 | F/8 | TLE | 50 – no | 210 – OCB | 7 – GAD 1:8000 | – | – | 13 – no | 13 – restitution |
| 4 | F/17 | TLE, depression, anxiety, memory difficulties | 0 – no | 479 – OCB | 18 – GAD 1:16000 | 18 – 410 – 23 | 33 – 2.8 | 40 – uni | 41 – memory impairment, TLE |
| 5 | F/13 | Memory deficits, focal seizures, apraxia | 7 – no | 7 – none | 25 – GAD 1:16000, VGKC 157 pM | ? – 90 – 3 | – | 31 – bi | 31 – memory impairment, TLE |
| 6 | F/15 | Concentration difficulties, memory deficits, TLE | 18 – no | 16 – none | 77 – GAD 1:128000, VGKC 310 pM | 5 – ? – ? | 30 – 2 | 67 – uni | 67 – memory impairment, epilepsy |
| 7 | M/3 | Cognition and motor impairment, coma, asystole | 32 – no | 2 – none | 1 – VGKC 143 pM | 1 – ? – 2 | – | 12 – bi | 15 – died |
| 8 | F/16 | TLE, hallucinations, fear, memory impairment | 88 – no | 87 – OCB | 3 – VGKC 202 pM | 4 – 15 – 2 | – | 4 – no | 12 – restitution (some gaps in autobiographic memory) |
| 9 | F/9 | Fever, vigilance loss, coma | 1 – no | 1 – protein 1000 mg/dl | 0 – negative | 0 – 50 – 0.3 | – | 3 – bi | 12 – memory impairment |
| 10 | F/14 | TLE, memory and concentration difficulties | 4 – no | 370 – none | 12, 18 – negative | 12 – 55 – 3 | – | 12 – bi | 15 – massive memory impairment, catamenial TLE |
| Means/summaries | 9 F/11 | 28 – all no | 136 – mean cell count: 4/µl, mean protein 408 mg/dl – OCB in 5 of 7 | 15 | 6 – 124 – 6 | 17 – 2.2 | 20 – 3 no, 2 uni, 5 bi | 24 – 2 recovered, 6 impaired, 1 vegetative state, 1 dead |
↵* All patients had uni- or bilateral increased mediotemporal T2/FLAIR signal.
Abs, autoantibodies; bi, bilateral; CSF, cerebrospinal fluid; F, female; GAD, Abs to glutamic acid decarboxylase; IVIG, intravenous immunoglobulins; m, male; MP-eq, methylprednisolone-equivalent dose (for explanation, see Patients and methods section); OCB, unmatched oligoclonal bands; pos, positive; TLE, temporal lobe epilepsy; uni, unilateral; VGKC-low, Abs to voltage-gated potassium-channels at low concentrations (<100 pM).