Table of Content

20 July 2008, Volume 3 Issue 07

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论著

Clinical Features of Moyamoya Disease in Adults

2008, 3(07):  472-476. 

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Objective To explore the clinical features in adult patients with moyamoya disease. Methods The age of onset, gender and clinical features of 244 Chinese adult patients with moyamoya disease were analyzed and compared with the data of the patients with moyamoya disease in Korea, Japan and North America. Results In the 244 patients, the age was from 18 to 72 years old. The mean age was 36 years. The male and female ratio was 1:1.03, which 120 were females and 124 were males. The initial symptom was cerebral ischemia in 187 patients and cerebral hemorrhage in 57 patients. All the patients came from 28 different provinces. Patients from Henan and Hebei province were 34 and 30 cases, which the proportion was the most. All the patients had digital subtraction angiography (DSA)/ magnetic resonance angiography(MRA) examination, and according to Suzuki stage, the I to VI stage was in 24, 96, 112, 152, 80 and 24 hemispheres respectively. In our cases, 244 patients underwent encephaloduro- arterio-synangiosis (EDAS) operation. After the operation, 57 cases that the initial symptom was hemorrhage had no rebleeding, and 161 cases in 187 patients that the initial symptom was ischemia had relief obviously, 26 cases no marked change. Conclusion Adult patients in China with moyamoya disease present with different clinical features compared with other countries. These observations may indicate the various mechanisms involved in moyamoya disease in China. EDAS is an effective method for the adult patients with moyamoya disease.

Moyamoya Syndrome in Graves’ Disease

2008, 3(07):  477-480. 

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Objective To analyze the clinical manifestation and intracranial lesions in patients concomitant with Graves’ disease (GD) and moyamoya syndrome(MMS) screened by transcranial Doppler (TCD). Methods We screened MMS by TCD for patients with ischemic stroke from 2000 to 2007 and these patients were diagnosed for MMS by digital subtract angiography (DSA) or magnetic resonance angiography(MRA). All the patients were diagnosed for GD with high level of thyroglobulin antibody(TGAb) and thyroid peroxidase autoantibody(TPOAb) at the same time. Results A total of 13 GD patients (1 male and 12 female) concomitant with MMS were screened during 7 years. Eleven patients had ischemic stroke (two patients accompanied with chorea), while one had dizziness and one had chorea only. Eleven patients had stenosis/occlusion on bilateral terminal internal carotid arteries (TICAs) /middle cerebral arteries(MCAs) /anterior cerebral arteries(ACAs), one patient with unilateral siphon of internal carotid artery(ICA) lesions, and the remaining patient had occlusion of proximal ICA in both sides. In all the patients as followup, we found vascular lesions aggravated by TCD in one patient with GD uncontrolled and another patient with improvement of cerebral ischemic symptom and stable thyroid function. Four patients underwent bilateral superficial temporal artery(STA)-MCA anastomosis after normalization of her hormonal conditions in 307 hospital of people’s liberation army(PLA). The neurological condition was improved since revascularization procedure. Conclusion According to the TCD screening, we find MMS associated with GD may not rare. Thyroid function test should be performed for the patients who have MMS without risk factors of atherosclerosis. Revascularization is thought to be an appropriate management for prevention further ischemic stroke.

Hemodynamic Change of Childhood Moyamoya Disease after Multiple Burr-hole Operation

2008, 3(07):  481-486. 

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Objective Using multicolor ultraphonic power Doppler to study the hemodynamic change of childhood moyamoya disease after multiple burr-hole operation, to know the effectiveness of the multiple burr-hole operation and to choose the best device of follow-up. Methods Eight children diagnosed with moyamoya disease underwent multiple burr-hole operations. And we used multicolor ultraphonic power Doppler assisting with single photon emission computed tomography (SPECT) to observe the hemodynamic change before and after the operations. Results Beginning at one week after operation, angiograms showed neovascularization, and 3 months after operation showed rich neovascularization. SPECT showed that the regional cerebral blood flow(rCBF) of homolateral hemisphere was improved at 3 months after burr-hole operation, but rCBF of opposite hemisphere decreased when compared with preoperation. Ischemic symptoms of all the patients improved after burr-operation. Conclusion After burr-hole operation, we can find rich new vessels and lateral branches through the holes. The burr-hole operation can improve the ischemia of childhood moyamoya disease; Doppler imaging is a valid noninvasive alternative to carotid artery angiography in evaluating direct and indirect revascularization.