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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