Early-onset stroke with moyamoya-like syndrome and extraneurological signs: a first reported paediatric series
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Moyamoya syndrome is characterised by an occlusion of the carotid terminations with the development of collateral vessels. Our objective is to describe a series of infants presenting early-onset moyamoya-like syndrome, which may constitute a distinct entity.
From a cohort of children with rare cerebral vascular pathologies, we studied eight infants (28 days–1 year) with early-onset moyamoya-like syndrome demonstrated by angiography. We retrospectively analysed the patterns on MRI and MRA, as well as all other available data.
Median age at diagnosis was 7 months (IQR: 6–8) with arterial ischaemic stroke in the middle cerebral artery territory. All of the children experienced severe stroke 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 children died and all of the other children suffered permanent neurological deficits.
The presence of extraneurological signs in cases of early-onset moyamoya syndrome is suggestive of a newly described systemic vasculopathy with predominantly cerebrovascular expression. Given its rapid progression marked by severe recurrent strokes and poor clinical outcome, early diagnosis could help in the decision to institute aggressive therapy.
KeywordsMoyamoya syndrome Ischaemic stroke Digital subtraction angiography MRI MRA
Cerebral anterior artery
Middle cerebral artery
Posterior cerebral artery
Internal carotid artery
Digital subtraction angiography
Magnetic resonance angiography
Modified Rankin Scale
The scientific guarantor of this publication is Catherine Adamsbaum. The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article. The authors state that this work has not received any funding. No complex statistical methods were necessary for this paper. Institutional Review Board approval was not required in accordance with French and European ethical guidelines (standard care). Written informed consent was not required for this study because this was a non-interventional retrospective study. Methodology: retrospective, observational, multicentre study.
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