Clinical And Imaging Analysis Of Adult Moyamoya Disease

ObjectiveIn order to improve our understanding of moyamoya disease and provide some reference for clinical diagnosis and treatment, a retrospective study of adult moyamoya disease which encompasses epidemiology, pathology, clinical manifestations, imaging features, treatment, prognosis and so on has been done.Methods1. I collected clinical and imaging data of53adult moyamoya disease patients who hospitalized in Tianjin Huanhu hospital during April2003～April2013.2. Patients with ischemic cerebral vascular events were classified as ischemia group and hemorrhagic patients as hemorrhage group. Then I compared epidemiology, related factors, clinical manifestations, pathological vessels, cerebral aneurysms and functional outcomes when discharge from hospital between the two groups.3. I summarized brain CT and MRI findings and analyzed the value of MRA when diagnosing adult moyamoya disease.4. I assessed the effectiveness of revascularization surgery.5. All data were processed by spss19.0statistical software. All statistics were used in two-sided test, P<0.05was considered statistically significant.Results1. The average age of patients was36.42±10.85years. Male to female ratio was1:1.48. All the patients were Han Chinese and from areas near Tianjin, with no family pattern. The average age, age composition, gender, season distribution were not statistically significant between ischemia group and hemorrhage group. Smoking proportion in ischemia group were statistically higher than hemorrhage group. There were5.3%patients with special medical records and10.5%patients with obvious incentives in front of cerebral vascular event attacks.2. Pathology:1case revealed that superficial temporal artery became thickened and narrowed, calcium deposition increased and CD34, anti-smooth muscle antibody were positive by immunohistochemistry;1case showed intimal hyperplasia and desquamation in endocranium arteriole, bleeding and expanded veins;1case showed endocranium and a little pia mater thickened.3. The most common clinical manifestation of all the patients was headache and dizziness, followed by movement disorder of limbs, visual abnormalities, abnormal sensation of limbs, speech disorder and unconsciousness. The proportion of visual abnormalities and movement disorder of limbs in ischemia group were higher than hemorrhage group. The proportion of headache and dizziness, unconsciousness in hemorrhage group were higher than ischemia group. In addition, Both admission NIHSS score and the highest NIHSS score of hemorrhage group were higher than ischemia group. Difference between the two groups was statistically significant.4. Test about rheumatism immunity of6patients were normal on the whole.5. In ischemia group,30.4%of patients had encephalatrophy. For cerebral infarction patients,93.1%of them had infarcts on one side of brain hemisphere,37.9%of them had posterior circulation infarcts and69.0%of them with multiple and punctate infarcts, which did not meet common vascular distribution. In hemorrhage group,13.0%of patients with encephalatrophy,21.7%of patients with multiple lacunar infarcts and encephalomalacia foci. For hemorrhage site,56.5%of patients had brain parenchyma involvement,52.2%of patients had ventricle involvement and30.4%of patients were subarachnoid space involvement.6. The proportion of stenosis or occlusion of posterior cerebral artery (PCA) in ischemia group was higher than hemorrhage group and the difference in PCA stage between the two groups was statistically significant. There was no statistically significant difference in Suzuki stage between ischemia group and hemorrhage group. The proportion of cerebral aneurysms in hemorrhage group was statistically higher than ischemia group. In addition, cerebral aneurysm is a risk factor of adult hemorrhagic moyamoya disease.7. The positive predictive value of MRA in diagnosing stenosis or occlusion of internal carotid artery C1, anterior cerebral artery A1, middle cerebral artery M1and PCA P1was55.8%-90.1%, negative predictive value was45.9%-88.5%, sensitivity was75.3%-97.2%, specificity was40.0%-76.8%, and accuracy rate was78.6%-93.8%. MRA level1patients were at DSA level Ⅰ and Ⅱ, MRA level2corresponded to DSA level II, III or IV. MRA level3corresponded to DSA level III and IV, MRA level4corresponded to DSA level V and VI. In terms of diagnosing compensatory vessels, for posterior choroidal artery, posterior pericallosal artery, ophthalmic artery, MRA was similar to DSA and for small vessels, MRA often showed unclearly.8. The difference in patients’average discharge NIHSS score between ischemia group and hemorrhage group was not statistically significant. Patients’average mRS scores assessed in6months later after revascularization operations was statistically lower than pre-operation. A few days to20months later after the operation, MRA or DSA showed external carotid artery system had established traffic branches to support internal carotid artery system for all the21patients.Conclusion1. Our data showed that adult moyamoya disease was more common in younger adult patients. Male to female ratio was1:1.48;2. Cerebral aneurysm was a risk factor of adult hemorrhagic moyamoya disease;3. The most common clinical manifestation of all the patients was headache and dizziness, followed by movement disorder of limbs, visual abnormalities, abnormal sensation of limbs. Ischemic moyamoya disease patients were vulnerable to movement disorders of limbs and visual disabilities.4. For adult moyamoya disease patients, infarcts were often multiple, not meeting common vascular distribution and Hemorrhage usually affected the lateral ventricle. The diagnosis relies on vascular imaging examination. Besides DSA, MRA could be used as diagnostic standard.5. In addition to the general medical treatment, patients could consider revascularization operations.