NMOSD | 1 per 100,000 Caucasians2 3.5-4.1 per 100,000 Asians2 1.8-10 per 100,000 Blacks1,2 Onset 30-40 years1,2 Onset 28-33 years for Blacks2 Onset 35-40 years for Asians2 Onset 44 years for Caucasians2 3 : 1-9 : 1 female/male1 | LETM1,2 ON1,2 Area postrema syndrome1,2 Diencephalic syndromes1,2 Cerebral syndromes1,2 Brainstem syndromes1,2 | Negative impact on quality of life:2 depression, neuropathic pain, bowel/bladder dysfunction, sexual dysfunction, inability to work, cognitive impairment Within 5 years untreated:1 50% blind and wheelchair-bound, 33% will have died Better recovery:2 young adults, early treatment (<14 days) Greater recurrence/disability/death:2 age >60, age <12, Asian, Black, longer length of myelitis lesions, ON at disease onset, increased severity of initial attack 5 : 1-10 : 1 female/male recurrence2 |
AQP4-seropositive NMOSD | 1/4 have coexistent autoimmune disease1,2 Uncommon in children2 | Inflammation of posterior optic nerve segments (optic tracts & chiasm)1,2 Unilateral/chiasmal ON involving >1/2 optic nerve2 Myelitis involving >3 vertebral segments in 85%, especially cervical and thoracic cords1,2 | Relapses in 90% involving ON and/or LETM2 Annual relapse rate 0.77 despite therapy2 More visual & motor disability2 |
MOG-seropositive (MOGAD) | Onset mid-30 s2 1 : 1 female/male2 1.6/million overall2 1.3/million adults2 3.1/million children2 More common in children & elderly1,2 No racial preponderance2 | Acute disseminated encephalomyelitis for age <72 ON for age >72 Inflammation of anterior optic nerve segments with increased edema2 Bilateral ON more common2 Lumbosacral myelitis2 Myelitis <3 vertebral segments2 | Better visual recovery from ON1,2 Better motor recovery2 Relapses in 44-83%2 Can be monophasic without relapse1,2 58% have residual disability2 |