The clinical characteristics and angiographic image analysis in fifteen adult patients with moyamoya disease

Objective To explore the clinical characteristics and angiographic finding in adult patients presented with Moyamoya disease. Methods Fifteen adult patients diagnosed using digital subtraction angiography( DSA) as Moyamoya disease were reviewed retrospectively. Their clinical appearance and angiographic findings were summarized. Results In this group of adult patients,male to female ratio was 1∶ 1. 5. The age distribution ranged from 21 ~ 64 years-old( average 42. 8 ± 11. 28 years-old). Cerebral hemorrhage or intraventricular hemorrhage were observed in 7 patients( 46. 7%),brain infarction or transient ischemic attack in 4 patients( 26. 7%),recurrent headache or dizziness in 3 patients( 20. 0%),and epilepsy attack in one patient( 6. 7%). According to Moyamoya staging( Suzuki),one patient was classified as stage I,two patients as stage II,ten patients as stage III,and 2 patients as stage IV. Collaterals supply from lenticulostriate arteries in 14 patients,from pial collateral in 14 patients,from the ophthalmic artery and the posterior and anterior ethmoidal arteries in 7 patients,and from dural collateral of external carotid artery in 6 patients. Conclusions Female patients were prone to have Moyamoya disease. Intracranial hemorrhage is the predominant clinical presentation( 46. 7%). Most patients were classified as Suzuki grade III. Bilateral stenosis or occlusion is consistently found in the anterior circulation except two patients with posterior circulation lesions. The exact pathophysiologic mechanism of these differences,which causes the highly selective involvement in the anterior circulator artery,needs more studies to explain these findings. During the disease process,collateral supplies come not only from lenticulostriate and pial collaterals,but also from dural or ophthalmic arteries. It is advised to study patients with stroke,epilepsy of unknown cause,frequent headache and dizziness using MRA or CTA. DSA,as the gold standard for the diagnosis of Moyamoya disease,can demonstrate the pathology and route for collaterals. It also provides valuable information and guidance in the medication treatment and surgical revascularization procedures.