title: Early-onset stroke with moyamoya-like syndrome and extraneurological signs: a first reported paediatric series.
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.
authors.affiliation: Pediatric Radiology Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Law-Ye
authors.affiliation: Neuroradiology Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France.|National Referral Center for Neurovascular Malformation in Children, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France.|French Center for Paediatric Stroke, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Saliou
authors.affiliation: CHU Nantes, Nord Laennec Hospital, Diagnostic and Interventional Neuroradiology, Bd J Monod St Herblain, 44093, Nantes Cedex 1, France.
authors.forename: Frédérique
authors.initials: F
authors.lastname: Toulgoat
authors.affiliation: Faculty of Medicine, Paris Sud University, 63 rue Gabriel Péri, 94276, Le Kremlin Bicêtre Cedex, France.|Neuropediatry Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Tardieu
authors.affiliation: Neuropediatry Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France.
authors.forename: Kumaran
authors.initials: K
authors.lastname: Deiva
authors.affiliation: Pediatric Radiology Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France. adamsbaum.catherine@gmail.com.|Faculty of Medicine, Paris Sud University, 63 rue Gabriel Péri, 94276, Le Kremlin Bicêtre Cedex, France. adamsbaum.catherine@gmail.com.|LTCI, CNRS, Télécom ParisTech, Université Paris-Saclay, Paris, France. adamsbaum.catherine@gmail.com.
authors.forename: Catherine
authors.initials: C
authors.lastname: Adamsbaum
authors.affiliation: Pediatric Radiology Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France.|French Center for Paediatric Stroke, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France.
authors.forename: Béatrice
authors.initials: B
authors.lastname: Husson
mesh_terms: D002533:Cerebral Angiography / Q000379:methods*; D002560:Cerebrovascular Circulation / Q000502:physiology*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D020768:Middle Cerebral Artery / Q000000981:diagnostic imaging*; D009072:Moyamoya Disease / Q000150:complications* / Q000175:diagnosis; D012189:Retrospective Studies; D020521:Stroke / Q000175:diagnosis / Q000209:etiology*
keywords: Digital subtraction angiography; Ischaemic stroke; MRA; MRI; Moyamoya syndrome