- Monophasic or relapsing acute optic neuritis, myelitis, brainstem encephalitis, encephalitis, or any combination thereof, and
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- Radiologic or, only in patients with a history of optic neuritis, electrophysiologic (VEP) findings compatible with CNS demyelination, and
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- At least one of the following findings:
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MRI - Longitudinally extensive spinal cord lesion (≥3 VS, contiguous, so-called LETM)
- Longitudinally extensive spinal cord atrophy (≥3 VS, contiguous) in patients with a history compatible with acute myelitis
- Conus medullaris lesions, especially if present at onset
- Longitudinally extensive optic nerve lesion (eg, >1/2 of the length of the pre-chiasmal optic nerve, T2 or T1/Gd)
- Perioptic Gd enhancement during acute ON
- Normal supratentorial MRI in patients with acute ON, myelitis and/or brainstem encephalitis
- Brain MRI abnormal but no lesion adjacent to a lateral ventricle that is ovoid/round or associated with an inferior temporal lobe lesion and no Dawson finger-type or juxtacortical U fiber lesion
- Large, confluent T2 brain lesions suggestive of ADEM
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Funduscopy - Prominent papilledema/papillitis/optic disc swelling during acute ON
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CSF - Neutrophilic CSF pleocytosis or CSF white cell count >50/microL
- No CSF-restricted OCB as detected by IEF at first or any follow-up examination (applies to continental European patients only)
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Histopathology - Primary demyelination with intralesional complement and IgG deposits
- Previous diagnosis of "pattern II MS"
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Clinical findings - Simultaneous bilateral acute ON
- Unusually high ON frequency or disease mainly characterized by recurrent ON
- Particularly severe visual deficit/blindness in one or both eyes during or after acute ON
- Particularly severe or frequent episodes of acute myelitis or brainstem encephalitis
- Permanent sphincter and/or erectile disorder after myelitis
- Patients diagnosed with "ADEM," "recurrent ADEM," "multiphasic ADEM," or "ADEM-ON"
- Acute respiratory insufficiency, disturbance of consciousness, behavioral changes, or epileptic seizures (radiological signs of demyelination required)
- Disease started within four days to approximately four weeks after vaccination
- Otherwise unexplained intractable nausea and vomiting or intractable hiccups (compatible with area postrema syndrome)
- Coexisting teratoma or NMDAR encephalitis (low evidence)
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Treatment response - Frequent flare-ups after intravenous methylprednisolone, or steroid-dependent symptoms (including CRION)
- Clear increase in relapse rate following treatment with interferon-beta or natalizumab in patients diagnosed with MS (low evidence)
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