| BACKGROUD:In1957, moyamoya disease was discovered in Japan for the first time. In1997, the diagnosis guideline on moyamoya disease is established by the Japanese Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare with the elimination of the related concomitant diseases. The other medical conditions are defined as moyamoya syndrome. Some researchers gave a new definition that moyamoya angiopathy includes moyamoya disease and moyamoya syndrome. However that opinion hadn’t been made as a guide and the moyamoya was still used in this study consisting of moyamoya disease and moyamoya syndrome. The International Classification of Diseases recognizes "moyamoya" as the specific name for this condition. Digital subtraction angiography is still the gold standard to diagnose the moyamoya. The cases on moyamoya were reported in Asia, Europe and America, and the incidence of those was highest in Japan with0.35-0.94per100000, and the higher incidence was in South Korea, China and other Southeast Asian countries, the incidence in Europe and America was one-tenth of that in Japan.The etiology on moyamoya remains not explicit. A number of studies presented that special angiopathy resulted from the upper respiratory infection, leptospirosis, and autoimmune diseases. The moyamoya accompanied with some autoimmune diseases possibly suggested the relationship between the special angiopathy and the concomitant autoimmune diseases. The moyamoya autopsy cases reported the positive expressions of the IgG and IgM on the intima of the internal lesion blood vessels and the middle meningeal arteries, and the discovery perhaps suggested those were related to intracranial arteries lesion.Just now no effective drug and treatment can regulate and reverse the moyamoya process. But the extra-intracranial revascularization could compensate partly the ischemic brain, and improve the clinical syndromes resulting from the ischemia for a long time. In general, the revascularization generally consisted of the direct and indirect treatments. However, some researchers combined the foregoing methods (STA-MCA combined with EMS) to treat moyamoya. It was necessary to diagnose the special angiopathy early and introduce the surgical treatment. The radiological methods can present the bypass, value the brain vascular reserve capacity, evaluate the cerebral perfusion and predict the possibility of the postsurgical complication that are of great significance to guide the diagnosis and treatment on moyamoya. In addition, the clinical survey outcomes can reflect the postsurgical neurocognitive function, body rehabilitation and the improvement of the life, study and work ability, as well as the survey can be designed to reflect the rehabilitation state based on the operator’s perspective.AIM:1. To make a descriptive analysis of the moyamoya patients and present the surgical efficacy and low risk of the rebleeding and dead after revascularization.2. To explore the role of the autoimmunity in the moyamoya development.3. To value the change of the brain perfusion after revascularization by mean of CT perfusion. METHODS:1. Clinical follow-up and statistical analysisDuring January2005and October2011, total158moyamoya patients were reviewed in Jinling hospital who were diagnosed by mean of CTA (13cases) and Digital Subtraction Angiography (DSA)(145cases). The descriptive analysis on moyamoya was conducted based on the age and clinical presentation-age distribution, while concomitant aneurysm on moyamoya and anamnesis being presented.From January2005to January2011,135moyamoya patients were reviewed by mean of the survival analysis. Both of the accepted revascularization and unaccepted revascularization groups were analyzed on the risk of the rebleeding and mortality. The follow up was by phone and outpatient service, and the deadline was post-discharge2years and3months later. The follow-up content consisted of the result of the rebleeding and death, radiological reviews, and rehabilitation (including body function, intelligence, memory, the work and life state, and the discomfort). According to the clinical note and follow-up, the FIM survey was completed.The statistical analysis was conducted with IBM SPSS19.0. The measurement data was showed with number and mean±standard deviations (x±s). Age frequency distribution was illustrated with15-class-range and5-class-width. The statistical survival analysis was by mean of the Kaplan-Meier analysis (P＜0.05).2. The pathological immunohistochemistry of the specimens on moyamoyaFrom February2009to September2012, the total25specimens were collected based on the moyamoya guideline and elimination. The total25specimens (VCAM-1:21specimens) and the5control specimens were fixed in the Neutral buffered formaldehyde for20hours, then the paraffin block was embedded and cut into slices in4-μm-thick. The S-P method was adopted to conduct the pathological immunohistochemistry on the expression of the IgG, IgM, complement C3and VCAM-1. The positive outcome was judged with the brown intima of the dura blood vessels and analyzed by mean of the semi-quantitative analysis. The statistical analysis was conducted with IBM SPSS19.0. The Wilcoxon rank sum test was used (P＜0.05(*)及P＜0.01(**)).3. The evaluation of the revascularization with CT perfusionDuring June2012and January2013,14moyamoya patients received CTA and CT perfusion before revascularization and postsurgical7days later. The horizontal slice of the basal ganglia region was chosen to analyze. The regions of interest (ROI) of the anterior cerebral artery, middle cerebral artery and posterior cerebral artery were marked out by the expert in the radiology department and the contralateral side was mark with mirror image function. The absolute results of the regions of interest (ROI) of the anterior cerebral artery, middle cerebral artery and posterior cerebral artery were analyzed with the method of pair-sample T test in the groups of combined and indirect revascularization.The9moyamoya patients with bilateral angiopathy were reviewed from the foregoing14moyamoya patients owing to comparing the CT perfusion difference before and after revascularization on the contralateral side as well as comparing relative value of the surgical side. The relative value was the surgical side dividing the contralateral side. The absolute data and relative data of each ROI were analyzed with pair-sample T test. The CT perfusion data from the ROI of the brain side without surgery of the bilateral intracranial arteries lesion was still by the method of pair-sample t test. The statistical analysis was conducted with IBM SPSS19.0. The analysis of the pair-sample t test was adopted (P＜0.05(*)及P＜0.01(**)).RESULT: 1. Clinical follow-up and statistical analysisFrom January2005to January2011, total158moyamoya patients were in Jinling hospital. According to the residence for more than5years, the158moyamoya patients were from Anhui Province (68cases,43.0%), Jiangsu Province (89cases,56.3%) and Hubei Province (1case,0.7%). The ages of all the moyamoya patients were from4to71(39.4±12.6) years. And the patients consisted of female (85cases,53.8%) and male (73cases,46.2%). In addition, the both peaks of the total moyamoya incidence included16～20years and36～40years while the female being same to the peaks of the total incidence but the male mainly during the36to45years. The moyamoya clinical representation comprised hemorrhage (120cases,75.9%) and ischemia (38cases,24.1%), and the hemorrhage representation-age,distribution remained from36to40years but ischemia-age distribution presented gently. There were34moyamoya patients accompanying with aneurysm including with hemorrhage representation (28cases,82.4%) and ischemic representation (6cases,17.6%). Owing to the117moyamoya patients with intracranial angiopathy representation based on the radiological data and medical history, there were unilateral lesion (27cases,23.1%) and bilateral lesion (90cases,76.9%). What’s more, the117moyamoya patients can be divided secondary lesion (22cases,18.8%), third lesion (89cases,76.1%) and forth lesion (6cases,5.1%) according to the Suzuki classification guide. The anamnesis of the total158moyamoya patients included hypertension (21cases), diabetes mellitus (1case), nephritis (2cases), psoriasis (1case), allergic history (5cases) and tobacco and alcohol addiction (23cases).The total135moyamoya patients were reviewed by mean of the survival analysis. The results of both the accepted revascularization and unaccepted revascularization groups consisted of rebleeding cases(1accepted and3unaccepted revascularization). To the deadline, the mean survival time were26.1months (95%CI:25.3～26.9months) and20.8months (95%CI:19.5～22.0months). The survival analysis presented statistical significance (P=0.000). During the deadline,135moyamoya patients comprised of3dead (1accepted and2unaccepted revascularization). The survival analysis suggested the statistical significance (P=0.000).The70follow-up was by phone and outpatient service, and the deadline was post-discharge2years and3months later. The functional independent measure consisted of the functional scores (91points), neurocognitive score (35points) and total score of the both. The comparison of the both revascularizations suggested no difference on the long time functional rehabilitation. However the revascularization statistically differed from the conservation based on the analysis outcomes.2. The pathological immunohistochemistry of the specimensThe cell nucleus of the endothelial cells showed the anachromasis and protuberance to blood lumen in the HE staining. The positive expression incidences were respectively92%,84%, and84%on the IgG, IgM, and C3on the intima of the dura blood vessels on moyamoya. The results were20%,20%, and40%in the control group. The analysis suggested significant difference. The positive expression of VCAM-1on moyamoya was14%with none of the positive expression in the control group and no statistical difference.According to the Wilcoxon rank sum test outcomes, the expressions of the IgG, IgM and complement C3on the intima of the dura blood vessels on moyamoya were significantly different. However the expression of the VCAM-1was positive in3cases without a statistical difference. The4superficial temporal artery specimens from the foregoing moyamoya patients presented an uneven incrassation of the intima but no significantly change on the medial intima. What’s more, the cell nucleus of the endothelial cells showed the anachromasis and protuberance to blood lumen in the HE staining and disorganized fibre. And there were3cases to present the positive expressions of the IgG, IgM, and C3on the intima of the moyamoya specimens.3. The value of the revascularization with CT perfusionFrom the14moyamoya patients, the12patients received the combined revascularizations. The comparison of the data of the CT perfusion on the region of the cortex of the cerebral middle artery before revascularization and postsurgical7days later showed the statistical difference on the absolute value on MTT (pre-operation4.5±0.6s, post-operation4.7±0.6s, P=0.045). There were3patients (4sides) receiving indirect revascularization, but the statistical analysis presented no significant difference.To exclude influence of the bilateral revascularization on the results and analyze the absolute and relative value in each region of the cerebral arteries, the9patients with bilateral angiopathy underwent the unilateral combined revascularization were reviewed. The comparison of the CT perfusion data before revascularization and postsurgical7days later presented significant difference of the TTP value on the middle cerebral artery ROI of the surgical side (pre-operation11.5±2.0s, post-operation10.5±1.9s, P=0.016), and the MTT value illustrated the apparent difference (pre-operation4.4±0.6s, post-operation4.7±0.7s, P=0.009). Viewing the anterior cerebral artery ROI and the posterior cerebral artery ROI, the comparison of the CT perfusion data before revascularization and postsurgical7days later presented TTP value (pre-operation11.9±2.0s, post-operation10.9±2.2s, P=0.046) (pre-operation10.7±1.4s, post-operation9.6±1.6s, P=0.024). With regard to the mirror image of the anterior cerebral artery ROI and the posterior cerebral artery ROI, the comparison of the CT perfusion data before revascularization and postsurgical7days later presented the TTP value (pre-operation11.3±2.0s, post-operation10.3±2.0s, P=0.027)(pre-operation10.9±1.9s, post-operationl0.0±2.2s,P=0.039). Nonetheless, the contralateral middle cerebral artery ROI presented no statistical difference.However there was only the rMTT relative value approached statistical difference (pre-operation1.0±0.2s, post-operation1.1±0.3s, P=0.052).Some moyamoya patients received CT perfusion review3months later, the comparison of the review data and the preoperational CT perfusion and post operational review for7days later presented the improving compensation of the ROI.CONCLUSION:1. The moyamoya present special epidemiological character and the survival analysis presented the lower rebleeding and death risk while moyamoya patients receiving revascularization.In this study, female morbidity was more than male. It was possible that some female specialty might relate to the incidence risk of the moyamoya. The adults hemorrhage representation was much more comparing with the ischemia for the ischemic representation hiding or compensatory through the collateral vessels. The moyamoya patients accompanying with aneurysm suggested that might result in hemorrhage as one of the risks. Whether The other concomitant diseases related to the moyamoya could not be illustrated.The survival analysis of the both of accepted and unaccepted revascularization showed the surgery could decrease the risk of the rebleeding and dead. The functional independent measure outcomes also presented the revascularization can improve the brain ischemic state, clinical syndromes, functional rehabilitation, social neurocognitive ability and the quality of moyamoya patients’ life. The collateral vessels developed for a long time, but the long follow-up illustrated the indirect revascularization effective. For the moyamoya patients accompanying wit aneurysm, the only treatment to the aneurysm cannot improve the clinical syndrome and survival incidence.2. The immunoglobulin and the activated complement taking part in the intima lesion of the blood vesselThe study mainly discussed the relationship between the blood vessels lesion of the dura and the positive autoimmune proteins on moyamoya disease. The results illustrated the positive expression of the IgG, IgM and complement C3comparing with the control group. The complement system possibly was activated by the immune globulins during the process of the blood vessels lesion.3. The post operational CT perfusion presented the value of the revascularization of moyamoya patientsAs a result, the combined method can improve the cerebral perfusion. The change of cerebral perfusion was significant in the region of the cortex of the cerebral middle artery on the surgical side. What’s more, there was some the influence to the region the both cerebral anterior artery and cerebral posterior artery through the collateral vessels indirectly. The results suggested the revascularization played an important role in improving the cerebral ischemia. |
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