Management of moyamoya syndrome in patients with Noonan syndrome

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
Mihir GuptaGary K Steinberg

Abstract

A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. We report four cases of Noonan syndrome patients presenting with headaches and/or sensorimotor strokes in childhood that caused unilateral sensorimotor impairment. Cerebral angiography and MRI revealed bilateral moyamoya syndrome. All patients underwent successful bilateral extracranial-to-intracranial revascularization. The first patient was a 10-year-old girl who presented following a hemorrhagic stroke and recovered well after indirect bypass. The second patient was an adult with a history of childhood stroke whose symptoms progressed in adulthood. She underwent a direct bypass and improved, but continued to experience TIA at her 4 year follow-up. The third patient was a 7-year-old girl with headaches and a new onset TIA who failed pharmacological therapy and subsequently underwent bilateral indirect bypass. The fourth patient was a 24-year-old...Continue Reading

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Citations

Mar 28, 2017·Journal of Medical Genetics·Stéphanie GueyManoelle Kossorotoff
Nov 23, 2018·International Journal of Molecular Sciences·Sciacca Francesca LuisaBersano Anna
Aug 14, 2018·Phytopathology·José A Gutiérrez-BarranqueroAntonio de Vicente
Jan 25, 2021·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·Fouad MitriMarkus Kraemer

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