title: Moyamoya disease associated with hereditary spherocytosis.
abstract: A 5-year-old girl with hereditary spherocytosis presented with two episodes of transient ischemic attacks within a month. Cranial magnetic resonance imaging angiography revealed a left internal carotid artery and middle cerebral artery stenosis, with an extensive vascular mesh in the thalamic area indicative of moyamoya disease. Treatment consisted of supporting cerebral perfusion with blood transfusions, and splenectomy to prevent recurrence. Moyamoya disease is a very unusual cerebrovascular disorder in childhood and its association with hereditary spherocytosis is rarely reported.
authors.affiliation: Department of Pediatrics, University Hospital Center Reims, Reims, France. pvovan@wanadoo.fr
authors.forename: Philippe
authors.initials: P
authors.lastname: Vo Van
authors.forename: Pascal
authors.initials: P
authors.lastname: Sabouraud
authors.forename: Gratiela
authors.initials: G
authors.lastname: Mac
authors.forename: Michel
authors.initials: M
authors.lastname: Abely
authors.forename: Nathalie
authors.initials: N
authors.lastname: Bednarek
mesh_terms: D000740:Anemia / Q000188:drug therapy / Q000209:etiology; D001803:Blood Transfusion; D002545:Brain Ischemia / Q000209:etiology; D016893:Carotid Stenosis / Q000150:complications; D002533:Cerebral Angiography; D002560:Cerebrovascular Circulation; D002675:Child, Preschool; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007361:Intelligence Tests; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D020768:Middle Cerebral Artery / Q000473:pathology; D009072:Moyamoya Disease / Q000150:complications*; D013103:Spherocytosis, Hereditary / Q000150:complications* / Q000628:therapy; D013156:Splenectomy; D020521:Stroke / Q000209:etiology; D014888:Wechsler Scales
chemical_list: D006454:Hemoglobins