Bruno Law-Ye1, Guillaume Saliou2,3,4, Frédérique Toulgoat5, Marc Tardieu6,7, Kumaran Deiva7, Catherine Adamsbaum8,9,10, Béatrice Husson1,4. 1. Pediatric Radiology Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France. 2. Neuroradiology Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France. 3. National Referral Center for Neurovascular Malformation in Children, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France. 4. French Center for Paediatric Stroke, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France. 5. CHU Nantes, Nord Laennec Hospital, Diagnostic and Interventional Neuroradiology, Bd J Monod St Herblain, 44093, Nantes Cedex 1, France. 6. Faculty of Medicine, Paris Sud University, 63 rue Gabriel Péri, 94276, Le Kremlin Bicêtre Cedex, France. 7. Neuropediatry Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France. 8. Pediatric Radiology Department, AP-HP, Bicêtre Hospital, 78 rue du Général Leclerc, 94275, Le Kremlin Bicêtre Cedex, France. adamsbaum.catherine@gmail.com. 9. Faculty of Medicine, Paris Sud University, 63 rue Gabriel Péri, 94276, Le Kremlin Bicêtre Cedex, France. adamsbaum.catherine@gmail.com. 10. LTCI, CNRS, Télécom ParisTech, Université Paris-Saclay, Paris, France. adamsbaum.catherine@gmail.com.
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.
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.
Entities:
Keywords:
Digital subtraction angiography; Ischaemic stroke; MRA; MRI; Moyamoya syndrome
Authors: Catharina M Legierse; Marijke R Canninga-Van Dijk; Carla A F M Bruijnzeel-Koomen; Veronica C M Kuck-Koot Journal: Eur J Dermatol Date: 2008-05-13 Impact factor: 3.328
Authors: Amélie Pinard; Maximillian D J Fiander; Alana C Cecchi; Andrea L Rideout; Mohamed Azouz; Stuart M Fraser; P Daniel McNeely; Simon Walling; Sarah C Novara; Anna C E Hurst; Dongchuan Guo; Sandhya Parkash; Michael J Bamshad; Deborah A Nickerson; Anthony M Vandersteen; Dianna M Milewicz Journal: Neurology Date: 2021-02-10 Impact factor: 9.910