title: [Angiographic moyamoya in a female with glycogenosis type IA - a case report focusing therapeutic management: bilateral encephalo-duro-arterio-myo-synangiosis (EDAMS)].
abstract: Angiographic Moyamoya is a rare cerebrovascular disease most frequent in asia. Its characateristics are recurrent ischemic attacks due to progressive occlusion of ICA branches. Angiography reveals fine arterial collateralisation reminding of ascending smoke ("moyamoya" in japanese). Neurosurgical treatment strategies include direct and indirect reanastomosation procedures. Randomised trials for comparison of clinical outcome and long term survival remain missing. A 23 years old female with glycogenosis type IA was first diagnosed bilateral angiographic moyamoya with bilateral proximal stenosis of ICA after transient ischemic attack (TIA). Coincidence of both rare diseases moyamoya and glycogenosis has previously been reported in three cases, so that this metabolic dysfunction presumably is a true risk factor for moyamoya. In our case, excellent angiographic and functional results were achieved by bilateral, consecutive Enzephalo-Duro-Arterio-Myo-Synangiosis (EDAMS).
authors.affiliation: Neurochirurgie, LMU München - Grosshadern, 81377 München. carolin.weiss@uk-koeln.de
authors.forename: C
authors.initials: C
authors.lastname: Weiss
authors.forename: T N
authors.initials: TN
authors.lastname: Witt
authors.forename: G
authors.initials: G
authors.lastname: Fesl
authors.forename: J
authors.initials: J
authors.lastname: Geisler
authors.forename: J C
authors.initials: JC
authors.lastname: Tonn
mesh_terms: D002343:Carotid Artery, Internal / Q000473:pathology; D016893:Carotid Stenosis / Q000473:pathology; D002533:Cerebral Angiography; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D006801:Humans; D002546:Ischemic Attack, Transient / Q000150:complications / Q000473:pathology; D018810:Magnetic Resonance Angiography; D009072:Moyamoya Disease / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D009389:Neovascularization, Pathologic / Q000473:pathology / Q000628:therapy; D019635:Neurosurgical Procedures; D014656:Vascular Surgical Procedures; D055815:Young Adult