Table 1.
Presenting clinical syndromes.
| Clinical Syndrome | Features | ||
|---|---|---|---|
| Optic Neuritis (ON) | Most common presentation in adults (50%) | Often bilateral Long lesions within the anterior optic pathway |
Good recovery, but with excessive optic disc edema and RNFL thinning |
| Transverse Myelitis | Initial presentation in 30% of adults | Motor, sensory, or autonomic symptoms | Significant predictor of disability, but more steroid responsive than NMOSD and MS |
| ADEM | Initial presentation in 50% of children. Very rare in adults (5%) | Presenting symptom is seizures in 40% of the children with ADEM and MOGAD | Increased risk of post-ADEM epilepsy |
| FLAMES | Unilateral cortical hyperintensities in the FLAIR sequence with associated seizures | Focal seizures which can progress to generalized, headache, encephalopathy | Meningeal involvement can be present |
| MOGAD & NMDAR overlapping syndrome | MOG antibodies and NMDAR-antibodies co-existence | Patients present with atypical NMDAR encephalitis or atypical MOGAD | Requires more aggressive treatment than MOGAD alone and cancer screening |
| Multifocal CNS attack | Can involve the optic nerve, spinal cord, brainstem and cerebellum | Vision loss, motor/sensory symptoms, ataxia, diplopia | |
| NMOSD syndrome | ON, LETM | Rarely intractable nausea and vomiting | AQP4 seronegative |
ON: optic neuritis; LETM: longitudinal extensive transverse myelitis, ADEM: acute disseminated encephalomyelitis; FLAMES: FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures; NMDAR: N-methyl-D-aspartate-receptor; NMOSD: neuromyelitis optica spectrum disorder; CNS: central nervous system.