Stroke in childhood due to inflammatory narrowing of blood vessels: Treatment with steroids

Prior treatment

• Previous stroke
• Contraindication to steroid treatment as e.g. a congenital or acquired immunodeficiency

Other illness

• On steroid treatment at disease onset
• Participation in another interventional study within the 30 days preceding the indication stroke and during the present study
• Known syndromal disorders, as e.g. Trisomy 21, Neurofibromatosis type 1
• Known genetic vasculopathies as e.g. PHACES syndrome, ACTA II
• Moyamoya or sickle cell disease
• Evidence of underlying systemic disorders, as e.g. lupus, rheumatoid problems
• Secondary CNS angiitis due to infections (meningitis, endocarditis, borreliosis), or generalised angiitis due to rheumatic or other autoimmune problems

Other

• Progressive large to medium childhood primary angiitis of the CNS (cPACNS ) with 2 of the following 3 criteria: a. pre-existing progressive neurocognitive dysfunction b. bilateral MRI lesions/vessel involvement c. small vessel arterial stenosis
• Inability to follow the procedures of the study, e.g. due to language problems
• Small vessel cerebral vasculitis (primary CNS vasculitis)
• Bilateral arteriopathy
• Arterial dissection(s)

• Informed consent of the legal representative of the trial participant documented by signature
• Age > 6 months & < 18 years at time of stroke
• Randomisation possible within 48 hours of diagnosis and maximum 96 hours after stroke onset
• Unilateral arteriopathy according to the following criteria: • Newly acquired neurologic deficits • Specific neuroimaging (MRA) features of either - unilateral stenosis, or - unilateral vessel irregularities within the CNS
• Unless otherwise defined in the national addendum: Female participants age ≥ 13: Negative pregnancy test (blood or urine)