Early-onset XXXX and extraneurological signs: a first reported paediatric series.

@entity1 @entity64 @entity455 @entity51 @entity7025
BACKGROUND: @entity7025 is characterised by an occlusion of the carotid terminations with the development of collateral vessels. Our objective is to describe a series of @entity1 presenting early-onset @entity7025 , which may constitute a distinct entity. METHODS: From a cohort of @entity1 with rare cerebral vascular pathologies, we studied eight @entity1 (28  days-1  year) with early-onset @entity7025 demonstrated by angiography. We retrospectively analysed the patterns on MRI and MRA, as well as all other available data. RESULTS: Median age at diagnosis was 7  months (IQR: 6-8) with arterial @entity64 in the middle cerebral artery territory. All of the @entity1 experienced severe @entity64 recurrence within a median time of 11  months (IQR: 10-12), and all showed extraneurological symptoms. The anterior cerebral circulation was involved in all cases and the posterior circulation was involved in six. Two @entity1 died and all of the other @entity1 suffered permanent @entity51 . CONCLUSIONS: The presence of extraneurological signs in cases of early-onset @entity7025 is suggestive of a newly described systemic @entity455 with predominantly cerebrovascular expression. Given its rapid progression marked by severe recurrent @entity64 and poor clinical outcome, early diagnosis could help in the decision to institute aggressive therapy.
