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

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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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Abbreviations

ACA:

Cerebral anterior artery

MCA:

Middle cerebral artery

PCA:

Posterior cerebral artery

ICA:

Internal carotid artery

DSA:

Digital subtraction angiography

MMS:

Moyamoya syndrome

MMD:

Moyamoya disease

MRA:

Magnetic resonance angiography

mRS:

Modified Rankin Scale

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Acknowledgments

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.

Author information

Authors and Affiliations

  1. Pediatric Radiology Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France

    Bruno Law-ye, Catherine Adamsbaum & Béatrice Husson

  2. Neuroradiology Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France

    Guillaume Saliou

  3. National Referral Center for Neurovascular Malformation in Children, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France

    Guillaume Saliou

  4. French Center for Paediatric Stroke, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France

    Guillaume Saliou & Béatrice Husson

  5. CHU Nantes, Nord Laennec Hospital, Diagnostic and Interventional Neuroradiology, Bd J Monod St Herblain, 44093, Nantes Cedex 1, France

    Frédérique Toulgoat

  6. Faculty of Medicine, Paris Sud University, 63 rue Gabriel Péri, 94276, Le Kremlin Bicêtre Cedex, France

    Marc Tardieu & Catherine Adamsbaum

  7. Neuropediatry Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France

    Marc Tardieu & Kumaran Deiva

  8. LTCI, CNRS, Télécom ParisTech, Université Paris-Saclay, Paris, France

    Catherine Adamsbaum

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  1. Bruno Law-ye
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  2. Guillaume Saliou
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  3. Frédérique Toulgoat
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  4. Marc Tardieu
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  5. Kumaran Deiva
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  6. Catherine Adamsbaum
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  7. Béatrice Husson
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Corresponding author

Correspondence to Catherine Adamsbaum.

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Law-ye, B., Saliou, G., Toulgoat, F. et al. Early-onset stroke with moyamoya-like syndrome and extraneurological signs: a first reported paediatric series. Eur Radiol 26, 2853–2862 (2016). https://doi.org/10.1007/s00330-015-4119-z

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  • DOI: https://doi.org/10.1007/s00330-015-4119-z

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