Vascular responses in cerebrovascular "Moyamoya" disease--evaluated by positron emission tomography

We investigated cerebral blood flow and metabolism, and cerebral vascular response in 9 patients with cerebrovascular Moyamoya disease or unilateral Moyamoya phenomenon using positron emission tomography (PET). The subjects consisted of 5 men and 4 women, and were from 9 to 60 years old. Five patients had bilateral occlusion in the carotid fork with Moyamoya vessels (fulfilled the criteria of cerebrovascular Moyamoya disease), and four patients had unilateral Moyamoya phenomenon. The PET scanner used was the HEADTOME III, of which spatial resolution in clinical use was 10 mm full width at half-maximum (FWHM) in the image plane. Cerebral blood flow (CBF), cerebral metabolic rate of oxygen (CMRO2), cerebral oxygen extraction fraction (OEF), and cerebral blood volume (CBV) were measured in resting state by the 15O-labelled gases steady state method in every patient and 22 normal controls (17 men and 5 women, and from 26 to 64 years old). Consecutively cerebral vascular responses were measured by H215O autoradiographic method in resting state, hypercapnia, hypocapnia, and hypertension. Forced hypercapnia, hypocapnia, and hypertension were achieved by 7% CO2 inhalation, hyperventilation, and venous infusion of angiotensin II, respectively. CMRO2 of the whole brain was significantly lower in patients than in normal controls (p less than 0.05), and CBV of the lentiform nucleus significantly increased in patients (p less than 0.01). This reflected Moyamoya vessels in the basal ganglionic regions. In 3 of 5 patients with bilateral Moyamoya vessels, CBF and CMRO2 in the symptomatic cerebral hemisphere were lower than that in the nonsymptomatic hemisphere.(ABSTRACT TRUNCATED AT 250 WORDS)     

甲状腺功能亢进合并烟雾病发病机制的研究进展

烟雾病是一种以双侧颈内动脉末端及大脑前动脉、大脑中动脉起始部慢性进行性狭窄或闭塞为特征，并继发颅底异常血管网形成的脑血管疾病。甲状腺功能亢进继发烟雾病的发病机制尚不清楚，可能与甲状腺功能、免疫与炎症、遗传因素等有关。免疫和炎症相关的分子变化对血管内皮细胞和平滑肌细胞的影响，可能在颅内大动脉狭窄、闭塞和烟雾血管的形成中起着重要作用。本文对甲状腺功能亢进继发烟雾病的可能病理生理学机制、免疫与炎症、遗传因素等发病机制进行综述。     

连续40例出血型烟雾病的DSA影像学分析

目的探讨烟雾病患者的DSA影像学诊断特征。方法回顾性分析40例烟雾病病例的DSA影像学表现,40例患者均经CT诊断为颅内出血,均经DSA确诊。结果双颈内动脉末段狭窄闭塞22例,一侧颈内动脉末段狭窄闭塞8例,一侧大脑中动脉狭窄闭塞3例,双侧大脑前动脉和一侧大脑中动脉狭窄闭塞1例,双侧大脑前动脉狭窄闭塞3例,双侧大脑中动脉狭窄闭塞1例,一侧大脑中动脉及对侧大脑前动脉狭窄闭塞2例,均伴颅底异常血管网形成。结论DSA为诊断烟雾病的金标准,DSA检查可清楚显示烟雾病血管狭窄闭塞的部位、侧支循环情况及是否合并动脉瘤,据其表现可指导进一步治疗。     

Occlusive cerebrovascular disease in young adults

13 patients under 40 years of age had cerebral infarction and angiographic evidence of arterial stenosis or occlusion. None of them had cardiac disorders prone to cerebral embolism. Five patients had occlusion at the origin of the internal carotid artery, one had occlusion of the common carotid artery and one each had stenosis at the origin of the internal carotid and common carotid arteries, respectively. Two patients had unilateral occlusion of the supraclinoid portion of the carotid artery with basal collaterals that had some resemblance to the Moyamoya disease. Three patients demonstrated stenosis or occlusion of the middle and/or anterior cerebral arteries. Three patients had hypercholesterolemia, one of whom was hypertensive. None had confirmed diabetes mellitus. One female, who died, had taken oral contraceptives for 3 years. A male, with internal carotid artery occlusion, had serological evidence for syphilis.     

An attempt to treat cerebrovascular 'Moyamoya' disease in children.

Moyamoya formations at the base of the brain are not congenital vascular malformations but represent collateral pathways associated with chronic progressive stenosis of the carotid fork. The authors have studied 44 personal cases, 18 children under 15 years of age, and 26 adults. In children the Moyamoya vessels change through six stages: (1) carotid fork stenosis; (2) progressive carotid stenosis with initial Moyamoya collaterals and dilatations of cerebral arteries; (3) dilatation of Moyamoya collaterals and disappearance of anterior and middle cerebral arteries; (4) thinning of Moyamoya; (5) contraction of Moyamoya and disappearance of posterior cerebral arteries; (6) intracerebral vessels perfused from the external carotid and/or vertebrae. These six stages are not observed in adults. Bilateral cervical perivascular sympathectomy (PVS) was performed in 9 children and superior cervical ganglionectomy (SCG) was added unilaterally in 4 and bilaterally in 3 cases. Angiographic follow-up studies were carried out 1-7 years following surgery. Improvement was observed in most of the cases examined within the first 2 months after surgery. This was not the case in arteriograms performed more than 6 months postoperatively. It would seem that PVS and SCG can improve the progress of Moyamoya vessels but only for a short period of time. Clinical symptoms, however, seem to continue improving over a long period of time. Cerebral blood flow improved 5 weeks following surgery in a 13-year-old boy.     

烟雾病的诊断与鉴别诊断

目的 评价烟雾病的诊断方法，讨论其鉴别诊断。方法 回顾性分析11例烟雾病的CT、MRI及MRA资料，分析脑实质及脑血管的形态学改变。结果 CT及MRI表现为脑实质改变。脑出血3例，脑梗化6例，脑萎缩2例；MRA均见有不同程度的颈内动脉分叉以上狭窄或闭塞，其中双侧颈内动脉狭窄6例，单侧颈内动脉狭窄5例；大脑中动脉狭窄20支，大脑前动脉狭窄18支，大脑后动脉狭窄14支；9例同时显示异常血管网。结论 烟雾病主要靠放射学诊断，MRA可很好地显示烟雾病异常血管，可作为筛台烟雾病首选方法。诊断时，要排除脑中风、动脉瘤和动静脉畸形出血。     

Disappearance of aneurysms associated with moyamoya disease after STA-MCA anastomosis with encephaloduro myosynangiosis

Moyamoya disease is a rare cerebrovascular disease characterized by steno-occlusive vasculopathy affecting the terminal internal carotid arteries. Although the effect of direct arterial bypass on the prevention of recurrent haemorrhage or ischemic events in patients with hemorrhagic moyamoya disease has been demonstrated, disappearance of aneurysms associated with moyamoya disease has rarely been reported. In this study, we present two patients with aneurysms associated with moyamoya disease. After superficial temporal artery to middle cerebral artery anastomosis combined with encephaloduro myosynangiosis, the aneurysms on the moyamoya vessels disappeared, which was confirmed by follow-up angiography.     

出血型烟雾病的临床特点及治疗现状

烟雾病是一种慢性闭塞性脑血管病,表现为双侧颈内动脉末端的进行性狭窄和闭塞,伴随脑底异常血管网生成。脑血管重建术通过改善颅内供血,对治疗缺血型烟雾病有效。而对于出血型烟雾病的治疗,目前仍未找到确实有效的治疗方法,外科手术治疗仍存在争议。本文总结概括了出血型烟雾病的临床特点和治疗现状。     

A case of unilateral moyamoya disease presenting with hemichorea

We reported a 12-year-old boy with unilateral moyamoya disease whose initial and predominant manifestation was hemichorea. Neurological examinations revealed chorea in his left upper extremity and muscle hypotonia in his left upper and lower extremities. Cranial MRI showed moyamoya vessels only in the right basal ganglia and infarction in the white matter of the right frontal lobe. Right carotid angiography revealed stenosis in the distal part of internal carotid artery, and in the proximal part of anterior and middle cerebral arteries with moyamoya vessels. Left carotid angiography showed normal findings. He was diagnosed as a suspected case of moyamoya disease (unilateral moyamoya disease) according to the diagnostic criteria proposed by the Research Committee on Moyamoya Disease of the Ministry of Health and Welfare of Japan. His chorea responded to haloperidol but encephalo-duro-arterio-synangiosis on the right side improved all symptoms. Chorea occurs in some patients with moyamoya disease. Hypofunction of the striatal indirect pathway is suggested as the cause of chorea. In this case an ischemic lesion in the right striatum may have caused hypofunction of the pathway and developed chorea and hypotonia.     

Ultrastructural studies of cerebral arteries and collateral vessels in moyamoya disease

Moyamoya disease was originally defined as a characteristic syndrome of recurrent headaches, occlusion of the distal internal carotid arteries and the foggy (moyamoya) clusters of collateral vessels at the base of the brain as demonstrated by cerebral angiography. The etiology is unknown and pathobiology is poorly understood. We examined the intracranial arteries in 3 patients to demonstrate characteristic changes and to obtain a better understanding of the basis mechanisms of the disease. Controls were obtained from 3 normotensive patients who died as a result of cancer. Occluded internal carotid arteries were characterized by severe thickening of the intima with a dense luminal array of smooth muscle cells, a deeper less cellular zone, pronounced tortuosity of the internal elastica and thinning of the media. Collateral vessels were arterial in structure and were affected by similar proliferative changes in the intima, thinning of the media, and contorted internal elastica. Stainable lipids were not part of the typical components. Severe contortion of the internal elastica, medial damage and intimal proliferation may result from recurrent and sustained spasticity of the cerebral arteries. The distal lenticulostriate arteries showed severe medial damage similar to what is termed as a moth-eaten change in hypertensive patients dying of massive cerebral hemorrhage.     

Inheritance of moyamoya disease in a Caucasian family

Background and purpose: Moyamoya disease is a very rare occlusive cerebrovascular disorder characterized by progressive stenosis or occlusion of the intracranial portion of the internal carotid artery and proximal cerebral arteries with an extensive network of fine collaterals. The aetiology and genetic susceptibility of moyamoya disease, especially in Caucasians, still remains unclear. Methods and results: We describe the cases of affected German father, daughter and son with juvenile stroke because of idiopathic moyamoya disease. The rare existing literature is reviewed and discussed. Conclusions: This is the first report on a father‐to‐child inheritance pattern in Caucasian patients with idiopathic Moyamoya disease (MMD). Our cases indicate possible genetic risk factors for the genesis of Caucasian Moyamoya disease.     

Moyamoya病的临床表现与影像学特点

目的 回顾分析12例Moyamoya病的临床和影像学特征，探讨Moyamoya病的影像学诊断价值。方法 分析12例Moyamoya病患的临床资料及数字减影血管造影(DSA)、磁共振血管造影(MRA)、磁共振成像(MRI)和CT结果。结果 所有病例均表现有颈内动脉或其分支不同程度的狭窄或闭塞和颅底异常血管网(MMD血管)；其中病变呈双侧8例．单侧4例。12例中头颅CT表现有梗塞灶4例，脑出血5例，其余3例表现正常。结论 除DsA外．MRI和MRA是两种可以很好评价Moyamoya病的影像学方法。若儿童或青壮年发生脑血管病，反复出现脑梗死或出现脑室出血、脑叶出血或蛛网膜下腔出血(SAH)则要考虑Moyamoya病的可能。     

Stroke risk factors and development of collateral flow in carotid occlusive disease

Background and purpose – Blood flow through collateral vessels compensates for reduced blood flow through stenotic or occluded extracranial carotid arteries. Previous studies have shown that extent of collateral flow influences likelihood of stroke and its outcome. Here we analyzed the relationship between stroke risk factors (hypertension, coronary artery disease, diabetes mellitus, tobacco smoking and hypercholesterolemia) and number of patent intracranial collaterals detected by transcranial Doppler ultrasonography. Subjects and methods – We studied 182 patients with various degrees of angiography proven unilateral stenosis of the internal carotid artery. Contribution of the anterior and posterior communicating arteries to the perfusion of the cerebral hemisphere on the side of the stenosis or occlusion was evaluated by a series of compression tests performed during continuous insonation of the middle cerebral artery. The number of detected collateral vessels was correlated with analyzed stroke risk factors. Results – Subjects with stenosis more than 75% or occlusion of the internal carotid artery had a higher frequency of two major intracranial collateral vessels ( P 0.01 and P 0.001, respectively). Hypertensive patients with stenosis more than 75% or total carotid occlusion were more likely to have only a single collateral vessel than patients without hypertension ( P 0.01 and P 0.05, respectively). Other risk factors did not influence the patency of preformed collateral vessels. Conclusions – Hypertension hindered the development of preformed intracranial collateral vessels in our patients with carotid occlusive disease.     

出血型烟雾病的研究进展

【摘要】 烟雾病是一种以颈内动脉末端狭窄、闭塞并伴有颅底异常血管网形成为特征的脑血管病,出血型烟雾病是其中的一个重要类型,也是目前的研究热点,本文就出血型烟雾病的流行病学特征、病因及发病机制、影像学特征及预测出血的指标、治疗和疗效评价等做一综述。     

Cerebrovascular disease in sickle cell anemia: a clinical, pathological and radiological correlation

An opportunity to study cerebrovascular changes in sickle cell anemia (SCA) presented itself when a black child with this disorder died of bihemispheric strokes. Angiography demonstrated severe occlusive vascular disease involving primarily the circle of Willis and major bifurcations of both internal carotid arteries. Collateral circulation to the distal branches of the internal carotid arteries occurred through transdural anastomoses from the external carotid system and via the leptomeningeal route. Perfusion of the basal ganglia was accomplished by vessels arising from the proximal internal carotid arteries. These changes resembled those of Moyamoya disease. Autopsy showed old and recent cerebral infarcts. Two vascular processes were responsible for the arterial occlusions: (1) exuberant intimal hyperplasia, and (2) old and recent thrombi with partial recanalization. The former has been described only once before in SCA. Small vessels in the basal ganglia were exceptionally numerous and dilated. We conclude that intimal hyperplasia within large cerebral arteries may be responsible for infarction and small vessel prliferation in basal ganglia in patients with SCA.     

Cerebral blood flow asymmetry in the detection of extracranial cerebrovascular disease

Regional cerebral blood flow was measured by the 133Xe inhalation technique in patients with unilateral carotid occlusion, unilateral carotid occlusion and contralateral carotid stenosis, bilateral carotid occlusion, or normal arteriograms. After adjusting for age, sex, and history of stroke, hemispheric blood flow asymmetry was shown to be a predictor of unilateral carotid occlusion with a sensitivity of 80.6% and a specificity of 80.5%. Asymmetry of regional cerebral blood flow is useful in the assessment of patients with extracranial cerebrovascular disease.     

Sickle cell anemia with moyamoya disease: outcomes after EDAS procedure

Moyamoya disease is a relatively uncommon neurovascular complication of sickle cell anemia. We report a case series of six patients with sickle cell anemia who developed moyamoya disease and underwent encephaloduroarteriosynangiosis procedures. These six patients presented with either cerebrovascular accidents, transient ischemic attacks, or seizures, and subsequent magnetic resonance imaging scans were suggestive of moyamoya-like changes in the cerebral vasculature. Conventional cerebral angiography was used to confirm the diagnosis in all six patients. Four of six patients manifested a cerebrovascular accident before surgery, and two of these patients were compliant on a transfusion protocol at the time of their cerebrovascular accident. Bilateral (n = 4) or unilateral (n = 2) encephaloduroarteriosynangiosis procedures were performed without any complications. The patient who was stroke-free preoperatively had a cerebrovascular accident 2 weeks after the procedure; otherwise, all patients have remained free of neurovascular complications with an average follow-up of 33 months. Collateral anastomoses between external and internal carotid arteries were established by magnetic resonance angiography in three patients. The encephaloduroarteriosynangiosis procedure is a safe and effective treatment option in patients with sickle cell anemia who develop moyamoya disease.     

DSCTA联合早期疗效的评价CTPI对颅内外动脉吻合术后

目的联合应用数字减影CT血管造影(DSCTA)和CT灌注成像(CTPI)对颅内外动脉吻合术(EIAB)后早期疗效进行评价。方法因脑血管疾病经受了EIAB的患者18例,其中9例行术后DSCTA检查,13例行手术前后CTPI检查,其中4例同时接受手术前后CTPI检查和术后DSCTA检查。结果 CTPI显示8例术后脑血供明显改善,表现为原异常灌注区域范围减小,部分吻合血管周围脑组织呈过度灌注状态,脑血容量、脑血流量明显增加,平均通过时间明显缩短(P0.05);3例术后脑血供无明显变化;2例脑血供减少。术后DSCTA显示搭桥血管通畅7条,闭塞3条。DSCTA可清楚显示旁路血管全程及吻合口的部位、大小或宽度。多平面重组(MPR)和去骨结构容积再现重建均可清楚显示搭桥血管及吻合口,但MRP更有助于测量吻合口的宽度。结论联合应用DSCTA和CTPI成像技术可对EIAB后早期疗效进行无创性定量评估,具有良好的临床应用前景。     

成年型孪生子Moyamoya病

目的报道一个成年型孪生子Moyamoya病。方法孪生姊妹之一的先证者为31岁,1年前出现全身乏力,检查发现糖尿病。半年前出现性格改变和抑郁,5个月前出现发作性右上肢麻木无力,2个月前出现左上肢活动不灵活和双下肢一过性无力,6d前出现大小便失禁和精神行为异常。查体显示轻度痴呆,左侧鼻唇沟浅,四肢肌力级,肌张力高,四肢锥体束征阳性。头部CT显示大脑半球多发性脑梗死。另一孪生妹妹在2年前亦发现糖尿病,曾经有过头晕发作。对先证者进行TCD和脑血管造影检查,另一孪生妹妹和无症状的2个哥哥及一对孪生女儿进行TCD检查。结果先证者DSA检查示双侧颈内动脉闭塞,双侧大脑前、中动脉主干不显影,颅底可见异常增生的血管网。TCD检查显示先证者和孪生姊妹的双侧颈内动脉末端闭塞,后循环代偿性血流增快,其孪生女儿之一存在双侧大脑中动脉起始段狭窄,无症状的2个哥哥血管无异常。结论孪生子Moyamoya病可以在成年起病,以缺血性损害为主要临床表现,合并糖尿病提示该类型Moyamoya病的发生可能和糖尿病相关基因的异常有关。当双胞胎之一出现Moyamoya病时,对另一个成员应当常规进行TCD筛查以确定其血管是否存在异常。     

Ischaemia-induced vascular vulnerability resulting in intracerebral haemorrhage with ipsilateral internal carotid artery occlusion

Intracerebral haemorrhage accompanied with cervical internal carotid artery (ICA) occlusion on the same side without moyamoya-like vessels is rare. A 73-year-old man with left ICA occlusion and no presence of moyamoya disease criteria underwent xenon-enhanced computed tomography with acetazolamide challenge test. The findings showed hypoperfusion and no vasoreactivity in the territory of the left middle cerebral artery. During follow-up he suffered bleeding in the left frontoparietal lobe. Cerebral angiography showed left ICA occlusion and cross flow via the anterior communicating artery without moyamoya vessels. Long-term ischaemia would make perforating or anastomotic arteries vulnerable. These arteries were easily ruptured by hypertension, resulting in intracerebral haemorrhage.