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Table 1 Epidemiological and clinical features according to diagnosis at last follow-up

From: Evaluation of treatment response in adults with relapsing MOG-Ab-associated disease

  Total population
n = 125
Relapsing
ON
n = 61
NMOSD-like phenotype
n = 41
bRelapsing
TM
n = 11
cMADEM/brainstem S
n = 5
dMS-like/optico-spinal phenotype
n = 7
Females, n (%) 69 (55.2) 33 (54.1) 24 (58.5) 5 (45.5) 3 (60) 4 (57.1)
Age at onset, years, median (range) 34.1 (18.0–67.1) 36.0 (18.0–67.1) 34.6 (18.0–62.5) 33.7 (18.0–42.1) 45.7 (31.3–60.7) 22.7 (19.4–53.7)
Caucasian, n (%) 120 (96) 58 (95.1) 40 (97.6) 11 (100) 5 (100) 6 (85.7)
Follow-up, years, median (range) 4.5 (0.2–47) 1.4 (0.4–47) 5.7 (0.2–47) 10.9 (2.1–21.2) 2.5 (0.56–4.0) 5.5 (0.2–19.3)
Phenotype at onset, n (%)
 ON 82 (65.6) 61 (100) 16 (39) 0 0 5 (71.4)
 Myelitis 25 (20) 0 12 (29.3) 11 (100) 0 2 (28.6)
 ON and myelitis 9 (7.2) 0 9 (22) 0 0 0
 Encephalopathic/brainstem S. 9 (7.2) 0 4 (9.7) 0 5 (100) 0
EDSS at onset, median (range) 3 (0–9) 2.5 (0–4) 3.25 (0–7.5) 3 (1–6) 4.5 (3.5–9) 3.5 (3–8)
 EDSS 0–2.5 47 (38.2) 30 (50) 22 (55) 4 (36.6) 3 (60) 5 (71.4)
 EDSS 3–5.5 64 (52) 30 (50) 32 (55) 4 (36.6) 3 (60) 5 (71.3)
 EDSS ≥ 6.0 12 (9.8) 0 6 (15) 2 (18.2) 2 (40) 2 (28.6)
aARR mean (SD) 0.79 (0.91) 0.80 (0.76) 0.64 (0.76) 0.46 (0.41) 1.13 (1.06) 1.78 (2.19)
Acute treatment MTP/PLEX/IVIG), n (%) 120 (96) 61 (100) 39 (95.1) 10 (90.9) 3 (60) 7 (100)
Paraclinical features, n (%)
 CSF OCB 10/107 (9.4) 1/49 (2.04) 4/36 (11.1) 3/11 (27.3) 0/5 (0) 2/6 (33.3)
 CSF pleiocytosis 41/98 (41.8) 7/46 (15.2) 21/31 (67.7) 6/10 (60) 4/5 (80) 3/6 (50)
 Abnormal brain MRI,
 at onset
28/74 (37.8) 7/38 (18.4) 11/23 (47.8) 3/6 (50) 4/4 (100) 3/3 (100)
EDSS at the last follow-up, median (range) 2 (0–7) 1.0 (0–4) 2 (0–7) 2 (0–4) 2.5 (1–3.5) 3.5 (0–6.5)
 EDSS 0–2.5 86 (69.9) 48 (80) 26 (65) 7 (63.6) 3 (60) 2 (28.6)
 EDSS 3–5.5 31 (25.2) 12 (20) 11 (27.5) 4 (36.6) 2 (40) 2 (28.6)
 EDSS ≥ 6.0 6 (4.9) 0 3 (7.5) 0 0 3 (42.9)
VA at the last follow-up
 VA ≥ 0.7 61/100 (61) 36/61 (59) 18/31 (58.1) 3/3 (100) 4/5 (80)
 VA > 0.2–0.6 21/100 (21) 13/61 (21.3) 8/31 (25.8) 0 0
 VA ≤ 0.2 18/100 (18) 12/61 (19.7) 5/31 (16.1) 0 1/5 (20)
  1. aFor ARR (SD), index event was excluded
  2. bFive patients had an extensive transverse myelitis
  3. cThree patients had multiphasic-ADEM with further ON relapses (ADEM-ON)
  4. dOptico-spinal phenotypes in 4, multiple sclerosis-like phenotype in 3 patients
  5. ON optic neuritis, NMOSD neuromyelitis optica spectrum disorder, TM transverse myelitis, ADEM-ON acute disseminated encephalomyelitis-optic neuritis, Brainstem S brainstem syndrome, MS multiple sclerosis, Optico-spinal optico-spinal phenotype, EDSS Expanded Disability Status Scale, ARR annualised relapse ratio, SD standard deviation, MTP methylprednisolone, PLEX plasma exchange, IVIG intravenous immunoglobulins, CSF cerebrospinal fluid, OCB oligoclonal bands, MRI magnetic resonance imaging, VA visual acuity