(A) | |||||
---|---|---|---|---|---|
Case number | Age: sex | Clinical features | Time to diagnosis/time to treatment response | Treatment group | Outcome/ΔmRS score* |
2† | 14: F | BC, S, MD, Sp | 6 months/2 days | A | Full recovery no sequelae/(5) |
3 | F | BC, S, MD | 9 months/90 days | D | Cognitive problems/(3) |
4 | 10: F | BC, S | >1 year/no response | A | Cognitive problems/(1) |
16 | 6: M | BC, S, MD | >1 year/7 days | A | Seizures, on treatment/(4) |
20 | 10: F | BC, S, MD | >1 year/no response | A | Significant disability, relapse, leucoencephalopathy on MRI (0) |
28 | 8: M | BC, S, MD | 7 months/3 days | A | One encephalopathic relapse treated with steroids/(2) |
Partial phenotypes | |||||
19 | 10: F | MD, Sp | > 1 yr/28 days | A | Symptom recurrence when unwell (1) |
(B) | |||||
Case number | Age: sex | Clinical features | Time to diagnosis/time to treatment response | Treatment group | Outcome/ΔmRS score† |
1 | 14: F | BC, S, MD | 1 month/90 days | D | Full recovery no sequelae/(3) |
5 | 3: F | BC, S | 1 week/7 days | A | Full recovery no sequelae/(4) |
6 | 5: F | BC, S, MD | 1 month/14 days | C | Antibodies remain high, cognitive problems/(3) |
7 | 9: M | BC, S(EPC),MD | 2 months/90 days | A | Full recovery no sequelae/(3) |
9 | 2: F | BC, S, MD, Sp | 5 weeks/7 days | A | Full recovery no sequelae/(4) |
10 | 14: F | BC, S, MD, | 1 week/29 days | D | One relapse treated with IV/PO steroids and MMF, full eventual recovery no sequelae/(5) |
11 | 2: F | BC, S, MD | <1 week/72 days | D | Full recovery no sequelae/(4) |
12 | 8: F | BC, S, MD | 2 months/60 days | C | Full recovery no sequelae/(3) |
13 | 15: F | BC, S, MD, Sp | A | Lost to follow-up | |
14 | 13: F | BC, S, MD, Sp, NMT | 1 week/14 days | A | Relapse at 1 year treated with AZT and MMF, full eventual recovery/(2) |
15 | 2: F | BC, S, MD | 2 weeks/30 days | A | Ongoing seizures, cognitive problems, antibodies elevated/(3) |
17 | 4: F | BC, S, MD | 2 weeks/30 days | A | Developmental arrest same as predisease stage, cognitive and behavioural problems (3) |
22 | 17: F | BC, S, MD | 3 weeks/3 days | B | Full recovery no sequelae/(5) |
24 | 14: F | BC, S, MD | 1 month/ | A | Cognitive problems and seizures/(2) |
26 | 3: F | BC, S, MD | 1 month/28 days | D | Full recovery no sequelae/(1) |
27 | 11: M | BC, MD | 12 days/8 days | A | Full recovery no sequelae/(5) |
30 | 2: F | BC, MD | 2 weeks/19 days | A | Cognitive and behavioural problems/(2) |
31 | 2: F | BC, MD | 4 weeks/67 days | D | Full recovery no sequelae/(5) |
Partial phenotypes | |||||
8 | 17: M | NPsych, BC, S | 3 weeks/14 days | B | Full recovery no sequelae/(4) |
18 | 5: F | MD, S, Sp | <1 week/7 days | A | Full recovery no sequelae/(3) |
21 | 12: M | MD, NPsych | 8 weeks/7 days | A | Full recovery no sequelae/(1) |
23 | 16: M | NPsych , BC | 1 month/7 days | C | Two relapses before MMF, none since started (1) |
25 | 15: F | NPsych, BC | 2 months/2 days | D | Relapse treated with steroids, cyclophosphamide and rituximab (4) |
29 | 14: M | NPsych, BC | 2 months/5 days | A | Relapse treated with PLEX and second-line immunotherapy; eventual full recovery (0) |
(A) lists patients with late diagnosis; (B) lists patients diagnosed within 8 weeks from symptoms onset. Patients with partial phenotypes were defined based on a lack of encephalopathy as evaluated by clinician.
*Outcome at final follow-up. ΔmRS score from nadir to 12-month postpresentation.
†Denotes patient with ovarian teratoma.
AZT, azathioprine; BC, behavioural change; EPC, epilepsia partialis continua; IV/PO, intravenous/oral; MD, movement disorder; MMF, mycophenolate mofetil; NMT, neuromyotonia; NPsych, neuropsychiatric; PLEX, plasma exchange; S, seizures; Sp, speech dysfunction.