Clinical Analysis Of 21 Cases With Moyamoya Disease

Introduction:Moyamoya disease is a chronic cerebrovascular disorder defined by progressive occlusion of the intracranial vessels and willis circle.A collateral network of capillaries develops at the base of the brain,giving rise to the characteristic reticulate ("puff of smoke") on angiography. In 1969, Suzuki define it six processes:1.narrowing of the carotid siphon; 2 initiation of moyamoya vessels; 3intensification of moyamoya vessels; 4 minimization of moyamoya vessels; 5 reduction of moyamoya vessels ;6 disappearance of moyamoya vessels. The stenosis begins with the intracranial carotid arteries and may progress to involve anterior, middle cerebral, and posterior cerebral arteries, developing collateral vessels, including intracranial carotid arteries, extracranial carotid arteries and vertebrel-basilar arteries.when these vessels are narrow or occlusion, it induce collateral vessels open, such as PCA-MCA, PCA-ACA. In Asian populations, moyamoya disease has a well-defined phenotype. especially Japan, Korea and China. The researchs show the majority of patients were women. The rate of men and women was 1:1.8. With the development of accessory examination, the patients without symptom is more and more. In order to further realize it, 21 MMD patients diagnosed at the First Hosptial of Jinlin University form march 2007 to march 2010 were enroller in this study. The study of onset age, sex, clinical manifestions, results of the imaging examinations and prognosis were reviewed in all cases.Objective:To analyze the clinical features and diagnostic value of transcranial Doppler(TCD), magnetic resonance imaging(MRI), magnetic resonance angiography(MRA)and digital subtraction angiography(DSA).Methods:Totally 21 MMD patients diagnosed at the First Affiliated Hospital of JiLin University from March 2007 to May 2010 were enrolled in this study. The initial clinical manifestions and results of the imaging examinations were reviewed in all cases.Results:①This study was composed of 21 patients,ranging in age from 5 to 67years old, of which 15 cases were in the adult group(>15 years) and 6 cases were in the juvenile group(≤15 years).The average age was 31 years old and peak incidence age was 5-15 years old and 35-45 years old. The male is 9 cases and female is 12 cases, the rate is 3:4.②The first symptom was cerebral infarction with a total of 7 cases, while transient ischemic attack was 7 cases(<35 years) and cerebral hemorrhage was 7 cases(>35 years).③All patients were examined by DSA or MRA, 5 cases had bilateral stenosis of middle cerebral arteries, 5 cases had stenosis of a middle cerebral artery and a internal carotid artery, 11 cases had bilateral stenosis of the distal internal carotid arteries. All patients were found collateral networks of capillaries at the base of the brains in varying degrees.④All patients were screened by using TCD at first and diagnosed by using MRA or DSA finally.Conclusion:①Ischemic stroke is the major clinical manifestation in childern MMD patients. childern clinical manifestation is less in degree than imaging examinations.②With the development of accessory examination,the rate of the patients with ischemic stroke is higher and higher.③When a patient has recurrent stroke without high blood pressure, diabetes and somke, MMD should be considered and the combine of TCD and MRA is available.