Table 1

Predominant symptoms at presentation, and outcomes at final follow-up

(A)
Case numberAge: sexClinical featuresTime to diagnosis/time to treatment responseTreatment groupOutcome/ΔmRS score*
2†14: FBC, S, MD, Sp6 months/2 daysAFull recovery no sequelae/(5)
3FBC, S, MD9 months/90 daysDCognitive problems/(3)
410: FBC, S>1 year/no responseACognitive problems/(1)
166: MBC, S, MD>1 year/7 daysASeizures, on treatment/(4)
2010: FBC, S, MD>1 year/no responseASignificant disability, relapse, leucoencephalopathy on MRI (0)
288: MBC, S, MD7 months/3 daysAOne encephalopathic relapse treated with steroids/(2)
Partial phenotypes
1910: FMD, Sp> 1 yr/28 daysASymptom recurrence when unwell (1)
(B)
Case numberAge: sexClinical featuresTime to diagnosis/time to treatment responseTreatment groupOutcome/ΔmRS score†
114: FBC, S, MD1 month/90 daysDFull recovery no sequelae/(3)
53: FBC, S1 week/7 daysAFull recovery no sequelae/(4)
65: FBC, S, MD1 month/14 daysCAntibodies remain high, cognitive problems/(3)
79: MBC, S(EPC),MD2 months/90 daysAFull recovery no sequelae/(3)
92: FBC, S, MD, Sp5 weeks/7 daysAFull recovery no sequelae/(4)
1014: FBC, S, MD,1 week/29 daysDOne relapse treated with IV/PO steroids and MMF, full eventual recovery no sequelae/(5)
112: FBC, S, MD<1 week/72 daysDFull recovery no sequelae/(4)
128: FBC, S, MD2 months/60 daysCFull recovery no sequelae/(3)
1315: FBC, S, MD, SpALost to follow-up
1413: FBC, S, MD, Sp, NMT1 week/14 daysARelapse at 1 year treated with AZT and MMF, full eventual recovery/(2)
152: FBC, S, MD2 weeks/30 daysAOngoing seizures, cognitive problems, antibodies elevated/(3)
174: FBC, S, MD2 weeks/30 daysADevelopmental arrest same as predisease stage, cognitive and behavioural problems (3)
2217: FBC, S, MD3 weeks/3 daysBFull recovery no sequelae/(5)
2414: FBC, S, MD1 month/ACognitive problems and seizures/(2)
263: FBC, S, MD1 month/28 daysDFull recovery no sequelae/(1)
2711: MBC, MD12 days/8 daysAFull recovery no sequelae/(5)
302: FBC, MD2 weeks/19 daysACognitive and behavioural problems/(2)
312: FBC, MD4 weeks/67 daysDFull recovery no sequelae/(5)
Partial phenotypes
817: MNPsych, BC, S3 weeks/14 daysBFull recovery no sequelae/(4)
185: FMD, S, Sp<1 week/7 daysAFull recovery no sequelae/(3)
2112: MMD, NPsych8 weeks/7 daysAFull recovery no sequelae/(1)
2316: MNPsych , BC1 month/7 daysCTwo relapses before MMF, none since started (1)
2515: FNPsych, BC2 months/2 daysDRelapse treated with steroids, cyclophosphamide and rituximab (4)
2914: MNPsych, BC2 months/5 daysARelapse 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.