TABLE 1.
Neuroinflammatory syndromes with optic neuritis
| Disease | Clinical Features | Laboratory Findings | Neuro-Imaging |
|---|---|---|---|
| Monophasic optic neuritis | 1. Unilateral or bilateral ON (may be asymmetric), and | OCB negative | Brain MRI is normal (outside the affected optic nerves) |
| 2. No signs, symptoms, or examination abnormalities outside the affected eye | |||
| Isolated recurrent optic neuritis | 1. ON and at least 1 relapse, and | OCB positive or negative | Brain MRI is normal (outside the affected optic nerves) |
| 2. No signs, symptoms, or examination abnormalities outside the affected eye | Anti-MOG positive or negative | ||
| Clinically isolated syndrome (CIS) (10) | 1. A first monofocal or polyfocal nonencephalopathic episode typical of MS | OCB may be positive or negative if the MRI has an abnormality typical of MS. | Typically reveals at least 1 or more MS-compatible lesions (diameter >3 mm) outside the optic nerves and chiasm |
| 2. 2010 Revised McDonald criteria are not fulfilled. | If MRI is normal, OCB must be positive to differentiate CIS from isolated optic neuritis. | ||
| Pediatric MS (9,19) | One of the following: | OCB positivity increases with age and is not an absolute criterion for an MS diagnosis if other clinical and radiographic features are met. | Dissemination in time: |
| 1. Two or more nonencephalopathic (e.g., not ADEM-like), clinical CNS events with presumed inflammatory cause, separated by more than 30 days and involving more than 1 area of the CNS | 1. A new T2 and/or gadolinium- enhancing lesion(s) on follow-up MRI, with reference to a baseline scan, irrespective of the timing of the baseline MRI, or | ||
| 2. One nonencephalopathic episode typical of MS which is associated with MRI findings consistent with 2010 Revised McDonald criteria for DIS and in which follow-up MRI shows at least 1 new enhancing or nonenhancing lesion consistent with dissemination in time (DIT) MS criteria | 2. Simultaneous presence of asymptomatic gadolinium-enhancing and nonenhancing lesions at any time | ||
| 3. One ADEM attack followed by a nonencephalopathic clinical event, 3 or more months after symptom onset that is associated with new MRI lesions that fulfill 2010 Revised McDonald DIS criteria | Dissemination in space: | ||
| A first, single, acute event that does not meet ADEM criteria and whose MRI findings are consistent with the 2010 Revised McDonald criteria for DIS and DIT (applies only to children ≥12 years old). | 1. T2 Lesion in at least 2 of 4 areas of the CNS: | ||
| Periventricular | |||
| Juxtacortical | |||
| Infratentorial | |||
| Spinal cord | |||
| 2. Gadolinium enhancement of lesions is not required for DIS. Symptomatic lesions are excluded from the criteria and do not contribute to lesion count. | |||
| Neuromyelitis optica spectrum disorder, NMO-IgG antibody positive | At least 1 core clinical characteristic | NMO-IgG antibody positive | 3. Specific MRI criteria are not required to confirm diagnosis (see NMO-IgG antibody negative for typical MRI findings). |
| 1. Optic neuritis | |||
| 2. Acute myelitis | |||
| 3. Area postrema syndrome (nausea/vomiting/hiccups) | |||
| 4. Other brainstem syndrome | |||
| 5. Symptomatic narcolepsy or acute diencephalic syndrome with MRI lesion(s) | |||
| 6. Symptomatic cerebral syndrome with MRI lesion(s) | |||
| Neuromyelitis optica spectrum disorder, NMO-IgG antibody negative | 1. At least 2 core clinical characteristics all satisfying: 1 of optic neuritis, myelitis, or area postrema syndrome, and | NMO-IgG antibody negative using the best available assay, or testing unavailable | Additional MRI requirements |
| Dissemination in space—isolated recurrent ON or recurrent TM does not qualify. | 1. Area postrema syndrome: dorsal medulla lesion | ||
| 2. Myelitis: longitudinal extensive transverse myelitis | |||
| 3. Optic neuritis: normal brain MRI, or >1/2 length of the optic nerve affected on MRI, or chiasm lesion | |||
| ADEM (19) | 1. A first polyfocal, clinical CNS event with presumed inflammatory demyelinating cause | Typically OCB negative but variability occurs | Typically on brain MRI: |
| 2. Encephalopathy that cannot be explained by fever | 1. Diffuse, poorly demarcated, large (>1–2 cm) lesions involving predominantly the cerebral white matter | ||
| 2. T1-hypointense lesions in the white matter are rare. Deep gray matter lesions (e.g., thalamus or basal ganglia) can be present. | |||
| ADEM followed by optic neuritis (22) | 1. Initial presentation fulfills criteria for ADEM, and | OCBs are not detected in the CSF (a pleocytosis may be present). | 1. MRI reveals typical brain or spinal cord T2 lesions consistent with ADEM initially; however, subsequent imaging shows resolution or near-complete resolution of lesions and new brain or spinal cord lesions do not appear during the ON attacks. |
| 2. ON diagnosed after ADEM with objective evidence of loss of visual function, and | |||
| 3. The ON occurs after a symptom-free interval of | |||
| 4 weeks and not as part of the ADEM or recurrent ADEM, and | |||
| 4. Diagnostic criteria for pediatric MS are not fulfilled. | |||
| Chronic relapsing inflammatory optic neuropathy (34) | 1. ON and at least 1 relapse, and | NMO-IgG seronegative | MRI confirms contrast enhancement of the acutely inflamed optic nerves. |
| 2. Objective evidence for loss of visual function, and | |||
| 3. Response to immunosuppressive treatment and relapse on withdrawal or dose reduction of immunosuppressive treatment |
ADEM, acute disseminated encephalomyelitis; CNS, central nervous system; CSF, cerebrospinal fluid; DIS, disseminated in space; DIT, disseminated in time; MOG, myelin oligodendrocyte glycoprotein; MS, multiple sclerosis; NMO, neuromyelitis optica; OCB, oligoclonal band; TM, tranverse myelitis.