Late-onset neuromyelitis optica spectrum disorder in AQP4-seropositive patients in a Chinese population

BMC Neurology

Table 1 Comparison of clinical features in patients with late-onset and early-onset AQP4-seropositive NMO/NMOSD

Feature	Late-onset AQP4-Ab Positive (n = 30)	Early-onset AQP4-Ab Positive (n = 30)	p-value
Total disease duration, months	24 (13–120)	25.5 (13–122)	0.958
Follow-up, median, mo	16 (12–84)	19 (12–88)	0.45
Female	26 (86.7)	27 (90)	0.5
Age at onset, median, y	57.5 (50–70)	31 (13–49)	<0.001
Onset episode, No.
ON only	13 (43.3)	13 (43.3)	0.603
TM only	15 (50)	13 (43.3)	0.398
ON + TM	1 (3)	3 (10)	0.306
Brain/brainstem	0	0	-
Mixed e.g. ON + brain	1 (3)	1 (3)	0.754
Median first interattack interval, months	7.5 (1–34)	6 (0.5–56)	0.58
Median interval between first ON and LETM, months	13 (0–51)	4 (0–54)	0.022
Median time from first symptoms to diagnosis of NMO	17 (0.5–52)	7 (0.5–54)	0.009
Monophasic	2 (6.6)	2 (6.6)	0.694
Total n of attacks	2.5 (1–11)	3 (1–7)	0.571
ARR	1.37 (0.5–2.9)	1.50 (0.5–2.99)	0.398
Coexisting autoimmunity, No.	11 (36.7)	7 (23.3)	0.20
Coexisting autoimmune disorders^a	4	2	0.57
Autoantibodies (number of positive tested/number tested)	7	5	0.57
Comorbidities, No.	20 (66.7)	8 (26.7)	0.002
Arterial thrombotic events	1 (3)	0 (0)	0.5
Venous thrombotic events	2 (6.7)	1 (3)	0.5
Diabetes mellitus	5 (16.7)	3 (10)	0.35
Hypertension	8 (26.7)	1 (3)	0.01
Osteoporosis	5 (16.7)	2 (6.6)	0.21
^bImmunosuppressors	27 (90)	28 (93.3)	0.5
Corticosteroids	13	16	0.35
Azathioprine + corticosteroids	12	9	0.25
Tacrolimus + corticosteroids	2	3	0.52
Nadir EDSS score	6.75 (3–8.5)	5 (3–8)	0.007
Time to EDSS 4	8 (0–39)	13.5 (0–54)	0.026
EDSS score at last visit	5.25 (1.5–10)	4 (0.5–7.5)	0.002

Values are presented as frequencies (percentages) and medians (minimum-maximum)
ON optic neuritis, LETM longitudinally extensive transverse myelitis, ARR annualized relapse rate, EDSS Expanded Disability Status Scale
^aCoexisting autoimmune disorders with or without autoantibodies included: Late-onset AQP4-Ab positive group: Sjögren syndrome (3 cases), Rheumatoid arthritis (1 case); early-onset AQP4-Ab positive group: Sjögren syndrome (1 case), Graves’ disease (1 case)
^bAutoantibodies (number of positive tested/number tested) included: Late-onset AQP4-Ab Positive group: anti-deoxyribo-nuclease B antibody (1 case), anti-nuclear antibody (2 cases), anti-histone antibody (2 cases), anti-keratin antibody (1 case), anti-cyclic citrullinated peptide antibody (1 case); Early-onset AQP4-Ab Positive group: anti-double-stranded DNA antibody (1 case), anti-nuclear antibody (2 cases), anti-histone antibody (2 cases)

ISSN: 1471-2377