This clinical trial deals with focal cerebral arteriopathy and childhood stroke, a rare but devastating condition. Focal cerebral arteriopathy (FCA) is an inflammatory vessel wall disease provoked by infection and there is increasing evidence that inflammatory processes play a crucial role in childhood stroke, influencing the outcome of the disease. Analysis of existing data suggests that outcomes are improved and that there is less stroke recurrence in children treated with steroids to reduce the acute inflammatory processes. This clinical trial will be conducted in over 20 hospitals in several countries in order to investigate this. Participants will be randomly separated into two groups. The first group will be treated with standard of care (including aspirin) combined with high dose steroids. The second group will be treated with standard of care (including aspirin) but without steroid treatment. The objective is to investigate if children treated with a combination of high dose steroid and aspirin will have a better and quicker recovery of FCA, better clinical functional outcome, and less recurrence compared to children treated with aspirin alone. This project has been identified by international pediatric stroke experts as the most important topic for a clinical trial in the field and is as well one of the most important research priorities identified by parents. The study results will also provide insight into the evolution of inflammatory vessel disease.
Inclusion Criteria:
1. Informed consent of the legal representative of the trial participant documented by signature
2. Age \> 6 months \& \< 18 years at time of stroke
3. Randomisation possible within 48 hours of diagnosis and maximum 96 hours after stroke onset
4. 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 Central Nervous System (CNS)
5. Unless otherwise defined in the national addendum: Female participants age ≥ 13: Negative pregnancy test (blood or urine)
Exclusion Criteria:
1. Previous stroke
2. Known syndromal disorders, as e.g. Trisomy 21, Neurofibromatosis type 1
3. Known genetic vasculopathies as e.g. posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies and eye anomalies syndrome (PHACES), actin alpha 2 (ACTA II)
4. Moyamoya or sickle cell disease
5. Small vessel cerebral vasculitis (primary CNS vasculitis)
6. Bilateral arteriopathy
7. Arterial dissection(s)
8. Evidence of underlying systemic disorders, as e.g. lupus, rheumatoid problems
9. Secondary CNS angiitis due to infections (meningitis, endocarditis, borreliosis), or generalised angiitis due to rheumatic or other autoimmune problems
10. Progressive large to medium childhood primary angiitis of the CNS (cPACNS ) with 2 of the following 3 criteria:
1. pre-existing progressive neurocognitive dysfunction
2. bilateral MRI lesions/vessel involvement
3. small vessel arterial stenosis
11. On steroid treatment at disease onset
12. Contraindication to steroid treatment as e.g. a congenital or acquired immunodeficiency
13. Inability to follow the procedures of the study, e.g. due to language problems
14. Participation in another interventional study within the 30 days preceding the indication stroke and during the present study