Table 1.

Clinical and radiographic characteristics, anti-AQP4 antibody status, type and duration and immunosuppressive therapy in SS NMOSD patients.

Patient	Clinical and Radiographic Findings	Anti- AQP4 Ever	Months Between FACS & IHC	Paired Anti- AQP4 Resultsa FACS IHC		Prior Therapy	Duration of immunosuppressive therapy prior to sera collection, FACS (months)	Duration of immunosuppressive therapy prior to sera collection, IHC (months)
1	Monophasic LETM, associated with brainstem and hypothalamic lesions	Yes	26	Yes	Yes	Methotrexate	68	42
2	Recurrent LETM × 2	Yes	58	Yes	Yesc	Azathioprine	42	0
3	Monophasic, unilateral ON with altitudinal deficit	Yes	21	Yes	Yesc	Methotrexate	15	0
4	Recurrent LETM × 4, unilateral monophasic ON, brainstem lesion	Yes	7	Yes	Yes	Azathioprine	127	120
5	Recurrent LETM × 5	Yes	30	Yes	Yes	Methotrexate	80	50
6	Recurrent LETM × 7, monophasic bilateral ON, brainstem lesion	Yesb	0	No	No	Mycophenolate, Cyclophosphamide, Rituximab	95	95
7	Monophasic LETM	Yesb	6	No	No	Plasma Exchange, Mycophenolate	7	1
8	Recurrent LETM × 3	Yes	16	No	Yes	Mycophenolate	21	5
9	Recurrent LETM × 2	Yes	2	Yes	Noc	Mycophenolate	1	0
10	Recurrent LETM × 5	Yes	13	Yes	No	Mycophenolate	13	0
11	Recurrent bilateral ON × 2	Yes	0	Yes	No	Mycophenolate	38	38

Legend: FACS = Fluorescence Activated Cell Sorting; IHC = Immunohistochemistry; LETM = Longitudinally Extensive Transverse Myelitis; NMOSD = Neuromyelitis Optica Spectrum Disorder; ON = Optic Neuritis; Prior Therapy = Immunosuppressive therapy excluding prednisone; 0 months = patient is treatment naïve

^aRefers to paired results of anti-AQP4 assays, the one by IHC being performed closest in time to that by FACS.

^bPatient initially had anti-AQP4 antibodies by IHC, but did not have detectable anti-AQP4 antibodies by IHC performed closest in time to FACS assay.

^cIn patients 2, 3, and 9, despite detection of anti-AQP4 antibodies by IHC, the decision by outside treating physicians was not to initiate immunosuppressive therapy even when patients were treatment-naïve. In these patients, the duration between onset of immunosuppressive therapy and sera collection for FACS assay could be less than the interval between paired sera collection.