Clinical Characteristics of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD)

Major Clinical Phenotypes of MOGAD

Optic neuritis often presents with severe visual deficit or blindness during or after acute episodes, and frequently shows perioptic gadolinium enhancement and longitudinally extensive optic nerve lesions on MRI, and may present as chronic relapsing inflammatory optic neuropathy (CRION) 1
Transverse myelitis can present as longitudinally extensive transverse myelitis (LETM) affecting ≥3 vertebral segments, and may cause permanent sphincter and/or erectile disorders, with conus medullaris lesions being particularly characteristic 1
Acute disseminated encephalomyelitis (ADEM) is characterized by large, confluent T2 brain lesions, disturbance of consciousness, behavioral changes, or epileptic seizures, and can occur as "recurrent ADEM," "multiphasic ADEM" or "ADEM-ON" (ADEM with recurrent optic neuritis) 1
Brainstem encephalitis can involve area postrema syndrome with intractable nausea, vomiting, or hiccups, and acute respiratory insufficiency in severe cases 1
Cortical encephalitis is characterized by cortical/subcortical white matter lesions and can present with seizures 2

Post-vaccination MOGAD disease onset can occur within 4 days to approximately 4 weeks after vaccination, and can present with any of the above phenotypes 1
MOGAD can co-exist with teratoma or NMDAR encephalitis, though evidence is limited 1

Steroid-dependent course is characterized by frequent flare-ups after intravenous methylprednisolone withdrawal 1

CSF typically shows neutrophilic pleocytosis or white cell count >50/μl, and usually lacks CSF-restricted oligoclonal bands (particularly in continental European patients) 1
MOGAD is significantly more frequent among young children with acquired demyelinating disease (up to 70%) than adults (≤1% in Western countries, ≤5% in Asian countries) 1

Progressive disease course is very atypical for MOGAD and should prompt consideration of alternative diagnoses 3

The American Academy of Neurology and other medical guidelines suggest that MOGAD-associated optic neuritis characteristically causes severe central visual deficit or complete blindness during acute episodes, with bilateral involvement being common 4
The optic nerve involvement is typically longitudinally extensive, affecting more than 50% of the pre-chiasmal optic nerve length or involving the optic chiasm, according to the American Academy of Ophthalmology 5, 4

Prominent papilledema, papillitis, or optic disc swelling is the hallmark finding on fundoscopy, not peripheral field changes, as stated by the American Academy of Neurology 4
Perioptic gadolinium enhancement (optic nerve sheath involvement) occurs frequently, which is a key diagnostic feature, as noted by the National Institute of Neurological Disorders and Stroke 5, 4
Bilateral simultaneous optic neuritis is common, affecting central rather than peripheral vision, according to the American Academy of Ophthalmology 4

MOGAD targets the optic nerve in a longitudinally extensive pattern that primarily affects central visual pathways, as described by the American Academy of Neurology 5, 4

Unlike glaucoma or other conditions causing peripheral vision loss, MOGAD presents with acute central vision loss and optic disc swelling, which is a key distinguishing feature, as stated by the American Academy of Ophthalmology 4
The absence of peripheral field defects helps distinguish MOGAD from conditions like primary angle-closure disease, which would show peripheral vision loss first, according to the American Academy of Ophthalmology 6