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28.11.2015 | Paediatric

Early-onset stroke with moyamoya-like syndrome and extraneurological signs: a first reported paediatric series

verfasst von: Bruno Law-ye, Guillaume Saliou, Frédérique Toulgoat, Marc Tardieu, Kumaran Deiva, Catherine Adamsbaum, Béatrice Husson

Erschienen in: European Radiology | Ausgabe 8/2016

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Abstract

Background

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.

Methods

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.

Results

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.

Conclusions

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.

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Titel: Early-onset stroke with moyamoya-like syndrome and extraneurological signs: a first reported paediatric series
verfasst von: Bruno Law-ye
Guillaume Saliou
Frédérique Toulgoat
Marc Tardieu
Kumaran Deiva
Catherine Adamsbaum
Béatrice Husson
Publikationsdatum: 28.11.2015
Verlag: Springer Berlin Heidelberg
Erschienen in: European Radiology / Ausgabe 8/2016
Print ISSN: 0938-7994
Elektronische ISSN: 1432-1084
DOI: https://doi.org/10.1007/s00330-015-4119-z

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Early-onset stroke with moyamoya-like syndrome and extraneurological signs: a first reported paediatric series