TABLE 3–1.

Demographics and Clinical Presentation of Optic Neuritis

Diagnosis	Demographics	Onset of Vision Loss	Pain	Eye Findings	Associated Systemic/Neurologic Disease
Multiple sclerosis	Young adults, female predominance	Acute or subacute	Yes	Normal or mild disc edemaa	Signs or symptoms of multiple sclerosis
Neuromyelitis optica (NMO) spectrum disorder	Older adults, strong female predominance	Acute or subacute	Yes	Normal or mild disc edemaa	Longitudinally extensive transverse myelitis, area postrema syndrome, SIADH
MOG-IgG	Pediatric and adult, no sex predilection	Acute or subacute	Yes	Frequent disc edema; often moderate or severea	Myelitis, ADEM
Seronegative (AON, RION, CRION)	Young adults, female predominance	Acute or subacute	Yes	Normal or mild edema; rare uveitisa	Autoimmune serology, steroid dependence
Granulomatous (sarcoidosis, granulomatosis with polyangiitis)	All age groups, sarcoidosis more common in African and Caribbean ethnicity, granulomatosis with polyangiitis peaks at older age	Subacute	Variable	Normal or edematous disc Sarcoid: uveitis, vitreitis, periphlebitis, episcleritis Granulomatosis with polyangiitis: scleritis, conjunctivitis, uveitis, vitreitis, vasculitis, orbital inflammation	Sarcoidosis: hilar adenopathy, lung fibrosis, cardiac symptoms, erythema nodosum Granulomatosis with polyangiitis: sinus disease, otitis, nasal ulcers, glomerulonephritis, systemic vasculitis, pleural effusion
Autoimmune (Sjögren syndrome, systemic lupus erythematosus)	Young adults, strong female predominance	Acute or subacute	Variable	Normal or edematousa Sjögren syndrome: dry eye	Sjögren syndrome: xerostomia, dental disease, pancreatitis Systemic lupus erythematosus: malaise, arthritis, malar rash, renal disease, thrombosis
GFAP-IgG	Early to late adulthood, no sex predilection	Not reported	No	Disc edema, normal intracranial pressure	Encephalitis, meningoencephalitis, myelitis, seizure
Paraneoplastic (CRMP-5)	Older adults, no sex predilection	Subacute	No	Disc edema, vitreitis, retinal vascular leak	Malignancy
Neuroretinitis	No age predilection, no sex predilection	Subacute	No	Disc edema with macular star	Viral prodrome, infection, catscratch disease
Syphilis	Concurrent HIV, high-risk behavior	Acute, subacute or chronic	Variable	Frequent severe disc edema, neuroretinitis, episcleritis, uveitis	Meningitis, encephalitis, cranial nerve palsies
Lyme disease	Lyme-endemic region	Acute or subacute	Rarely reported	Disc edema, neuroretinitis	Myalgia, arthralgia, erythema migrans
Tuberculosis	Tuberculosis-endemic region, Immunocompromise	Acute or subacute	Infrequent	Normal or edema, uveitis, orbital apex syndrome	Pulmonary disease, meningitis, lymphadenopathy
Viral infection	Viral-endemic regions, zoster reactivation, immunocompromise	Acute or subacute	Variable	Normal or edema, retinal necrosis, chorioretinitis	Zoster, fever, rash, lymphadenopathy, immunocompromise

ADEM = acute disseminated encephalomyelitis; AON = autoimmune optic neuropathy; CRION = chronic relapsing inflammatory optic neuropathy; CRMP-5 = collapsin response mediator protein-5; GFAP-IgG = glial fibrillary acidic protein immunoglobulin G; HIV = human immunodeficiency virus; MOG-IgG = myelin oligodendrocyte glycoprotein immunoglobulin G; RION = relapsing isolated optic neuritis; SIADH = syndrome of inappropriate secretion of antidiuretic hormone.

^aOptic disc pallor if recurrent disease.