Characterization of the binding pattern of human aquaporin-4 autoantibodies in patients with neuromyelitis optica spectrum disorders

Table 5 Clinical, CSF, and demographic characteristics of NMOSD patients with available paired CSF and serum samples

Patient number	29	38	44	45	46	47
Sex	Female	Female	Female	Female	Female	Male
Age (y)	73.7	52.4	41.4	50.1	28.3	68.9
Age at onset (y)	73.7	52.2	26.4	49.6	25.6	68.9
Disease duration (y)	0.00	0.21	14.90	0.50	2.72	0.01
Diagnosis	NMO	ON	NMO	NMO	NMO	NMO
Acute relapse	yes	yes	yes	yes	yes	no
Number of relapses	1	1	5	2	6	1
EDSS	2.0	2.0	6.5	2.5	6.0	5.5
Treatment	NoTh	Pred	RTX	AZA	RTX	PLEX
CSF cells/μl	9	4	5	1	4	73
Albumin quotient	6.59	3.97	6.20	8.92	2.32	18.12
IgG index	0.64	0.55	0.48	0.58	1.20	0.58
CSF OCB	Negative	Negative	Negative	Negative	Positive	Negative
AQP4-IgG CSF^a	6.19	1.23	4.18	2.50	5.04	4.15
AQP4-IgG serum^a	11.59	3.38	15.02	4.47	9.38	7.98
AQP4-IgG quotient	0.53	0.36	0.28	0.56	0.54	0.52
AQP4-IgG pattern CSF	A	A	A	A	A	A
AQP4-IgG pattern serum	B	B	A	B	B	B

AQP4-M23, AQP4-M1 and extracellular loop A, C, and E mutations were expressed in HEK-293A cells and assayed to categorize pattern A and pattern B serum samples as indicated in Fig. 3
AZA azathioprine, NoTh no therapy, PLEX plasma exchange, Pred corticosteroids, RTX rituximab, y years, albumin quotient CSF albumin/serum albumin, IgG index albumin quotient/IgG quotient, OCB oligoclonal bands, AQP4-IgG quotient CSF/serum AQP4-IgG
^aFACS AQP4-IgG binding ratio for AQP4-M23

ISSN: 1742-2094