Table 1

NMO cohort description and phenotype characteristics compared to control group

AQP4-Ab statusTOTAL NMO cohort n=20Non-NMO demyelination cohort (n=29)
AQP4-Ab negative (n=8)AQP4-Ab positive (n=12)
Sex
 Male:female0:82:102:1813:16
Age at onset in years (range)
 Median (range)10.9 (5.4–16.8)10.3 (2.9–15.0)10.5 (2.9–16.8)13.6 (1.3–15.8)
Follow-up duration in years (range)
 Median (range)5.4 (1.5–12.0)6.4 (2.0–17.8)6.1 (1.5–17.8)1.0
Wingerchuk 2006 criteria positive8/8 (100%)12/12 (100%)0/29
First presentation
 Unilateral ON268 (40%)1 year diagnosis:
 Bilateral ON224 (20%)ON:2
 TM213 (15%)TM:11
 TM and ON213 (15%)ADEM:3
 ADEM022 (10%)MS: 13
CSF OCB positive0/72/102/17 (12%)*10/19 (53%)
Relapsing course6/812/1218/20 (90%)11/29 (38%)
Phenotype of first relapse
 Unilateral ON358
 Bilateral ON000NA
 TM156
 TM and ON011
 CIS/ADEM123
MRI brain abnormalities present5/810/1215/20 (75%)29/29
 Large (>2 cm)2/58/1010/15 (75%)*9/29 (31%)
 Acute lesions mostly resolving on repeat2/56/108/15 (53%)*4/22 (18%)
 T1 hypointense1/55/106/15 (40%)*3/29 (10%)
 Gadolinium enhancing0/53/104/15 (27%)6/17 (35%)
 Barkhof space0/53/103/15 (20%)*11/29 (38%)
 LETM (19/20 all cases)5/510/1015/15 (100%)*11/29 (38%)
MRI brain lesion location
 Centrum semiovale/deep white matter1/57/108/15 (53%)16/29 (55%)
 Periventricular1/56/107/15 (47%)14/29 (48%)
 Brainstem2/53/105/15 (33%)12/29 (41%)
 Juxtacortical1/54/105/15 (33%)14/29 (48%)
 Periaqueductal grey1/54/105/12 (33%)n/a
 Cerebellar1/54/105/15 (33%)7/29 (24%)
 Corpus callosum0/54/104/15 (27%)10/29 (34%)
 Thalamic/basal ganglia1/52/103/15 (20%)5/29 (17%)
 Hypothalamic1/52/103/15 (20%)Not measured
 Cortical2/50/102/15 (13%)4 (14%)
  • *Fisher's exact test p<0.05 (two-tailed) compared to non-NMO cohort.

  • ADEM, acute disseminated encephalomyelitis; CIS, clinically isolated syndrome; CSF, cerebrospinal fluid; LETM, longitudinally extensive transverse myelitis; MS, multiple sclerosis; NMO, neuromyelitis optica; OCB, oligoclonal bands; ON, optic neuritis; TM, transverse myelitis.