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Table 1 PUDDLS Inclusion Criteria

From: Paediatric UK demyelinating disease longitudinal study (PUDDLS)

Condition

Inclusion Criteria

Acute

Disseminated

Encephalo-myelitis

(ADEM)

A polysymptomatic clinical event with acute/subacute onset that must include encephalopathy (behavioural change or altered consciousness). (2) MRI Brain shows f multifocal lesions, predominantly involving white matter.

• Relapsing ADEM: symptoms or signs within 3 months of initial onset of ADEM.

IF a new event occurs ≥ 3 months later and ≥ 1 month after completing steroid treatment, it is defined as:

• Recurrent ADEM: recurrence of initial symptoms without involvement of new clinical areas.

• Multiphasic ADEM: New event, but involving new anatomical areas of the CNS.

Clinically Isolated Syndrome

(CIS)

A first acute-clinical episode of CNS symptoms which may either be monofocal or multifocal, but does not include encephalopathy (except in brainstem syndromes). The MRI will show area of white matter demyelination. These include:

Transverse myelitis: weakness and/or numbness of both legs +/- arms, usually with maximal deficits 1 week after symptom onset supported by demyelination on MRI spine.

Brainstem, cerebellar, and/or hemisphericdysfunction, supported by demyelination on MRI.

Optic Neuritis

(ON)

Acute or subacute loss of vision and ≥ 1 of: relative afferent pupillary defect (unilateral cases), visual field deficit or scotoma, impaired colour vision, optic disc oedema, or abnormal visual evoked potentials. MRI is not necessary for diagnosis.

Neuro-myelitis Optica (NMO)

Three of the following four criteria: i. Optic neuritis ii. Acute myelitis iii. Spinal MRI lesion extends over three or more segments iv. NMO antibody testing is positive.