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Table 1 Diagnostic criteria for Acquired Demyelinating Syndromes [2, 3]

From: Pediatric versus adult MS: similar or different?

CIS

A first monofocal or multifocal CNS demyelinating event; encephalopathy is absent, unless due to fever

Monophasic ADEM

• A first polyfocal clinical CNS event with presumed inflammatory cause

• Encephalopathy that cannot be explained by fever is present

• MRI typically shows diffuse, poorly demarcated, large, >1-2 cm lesions involving predominantly the cerebral white matter; TI hypointense white matter lesions are rare; Deep grey matter lesions (e.g. thalamus or basal ganglia) can be present

• No new symptoms, signs or MRI findings after 3 months of the incident ADEM

Multiphasic ADEM

• New event of ADEM 3 months or more after the initial event that can be associated with new or re-emergence of prior clinical and MRI findings. Timing in relation to steriods is no longer pertinent.

MS

Any of the following:

• Two or more nonencephalopathic CNS clinical events separated by more than 30 days, involving more than one area of the CNS

• Single clinical event and MRI features rely on 2010 Revised McDonald criteriab for DIS and DIT [4] (but criteria relative for DIT for a single attack and single MRI only apply to children ≥12 years and only apply to cases without an ADEM onset)

• ADEM followed 3 months later by a nonencephalopathic clinical event with new lesions on brain MRI consistent with MS

NMO

All are required:

• Optic neuritis

• Acute myelitis

• At least two of three supportive criteria:

 • Contiguous spinal cord MRI lesion ≥3 vertebral segments

 • Brain MRI not meeting diagnostic criteria for MS

 • Anti-aquaporin-4 lgG seropositive status