Weiss C, Witt T N, Fesl G, Geisler J, Tonn J C
Neurochirurgie, LMU München - Grosshadern, 81377 München.
Fortschr Neurol Psychiatr. 2010 Mar;78(3):154-60. doi: 10.1055/s-0029-1245175. Epub 2010 Mar 8.
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).
血管造影显示的烟雾病是一种罕见的脑血管疾病，在亚洲最为常见。其特征是由于颈内动脉分支的渐进性闭塞导致反复缺血性发作。血管造影显示有精细的动脉侧支循环，让人联想到袅袅上升的烟雾（日语中的“烟雾”）。神经外科治疗策略包括直接和间接吻合手术。目前仍缺乏比较临床结果和长期生存率的随机试验。一名23岁的IA型糖原贮积病女性在短暂性脑缺血发作（TIA）后首次被诊断为双侧血管造影显示的烟雾病，伴有双侧颈内动脉近端狭窄。烟雾病和糖原贮积病这两种罕见疾病此前曾在3例病例中同时出现，因此这种代谢功能障碍可能是烟雾病的一个真正危险因素。在我们的病例中，通过双侧连续的脑-硬膜-动脉-肌-联合血管吻合术（EDAMS）取得了出色的血管造影和功能结果。
