Angiografisches Moyamoya bei Glykogenose Typ IA – Management bei einer erwachsenen Patientin
Fallbericht mit Schwerpunkt auf der Behandlung: beidseitige Encephalo-Duro-Arterio-Myo-Synangiose (EDAMS)

Zusammenfassung

Angiografisches Moyamoya ist eine seltene zerebrovaskuläre Erkrankung mit Häufung in Asien. Progrediente Obstruktion der hirnversorgenden Arterien führt zu zerebralen Ischämien, meist bereits im frühen Kindesalter und ist verbunden mit ausgeprägter Kollateralisation, welche in der Angiografie den Aspekt von Rauch (japan.: moya moya) imitiert. Als operative Therapieoptionen existieren direkte und indirekte Anastomoseverfahren, wobei prospektive, randomisierte Studien zur Therapiewahl bislang fehlen. Bei einer 23-jährigen Glykogenose Typ IA-Patientin mit transitorischer ischämischer Attacke wurde die Erstdiagnose eines beidseitigen angiografischen Moyamoya gestellt. Die Koinzidenz beider seltener Erkrankungen wurde bereits in zwei Fallberichten publiziert; es scheint sich hierbei um einen echten Risikofaktor zu handeln. Durch beidseitige Enzephalo-Duro-Arterio-Myo-Synangiose (EDAMS) bei jeweils proximaler ACI-Stenose wurde eine klinisch und radiografisch hervorragendes Ergebnis erzielt.

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).

Schlüsselwörter

Moyamoya - Glykogenose Typ I - v. Gierke-Erkrankung - Glykogenspeicherkrankheit - Risikofaktor

Keywords

moyamoya - glycogenosis type I - Gierkes‘ disease - glycogen storage disease - risk factor

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