Novel magnetic resonance angiography stage grading for moyamoya disease

BACKGROUND: Magnetic resonance angiography (MRA) has been acknowledged as a noninvasive diagnostic modality for moyamoya disease. However, in terms of staging of moyamoya disease, conventional angiography is still the gold standard. Therefore, the purpose of this study was to establish MRA grades for moyamoya disease as an alternative to conventional angiography. METHODS: Twenty-two patients (44 sides) with moyamoya disease diagnosed by conventional angiography were evaluated by MRA during the past 5 years. MRA scores were assigned based on the severity of occlusive changes of the internal carotid artery, the horizontal portion of the middle cerebral artery, the anterior and the posterior cerebral arteries and the signals of the distal branches of these arteries. Total points ranged from 0 (normal) to 10 (most severe). RESULTS: MRA scores (0-10) were significantly consistent with the conventional angiographic staging. Four grades based on this novel MRA scores correlated well with Suzuki's stages, with high sensitivity and specificity. CONCLUSIONS: These novel MRA grades can be a reliable alternative to conventional staging. By employing these novel MRA grades, the use of conventional angiography can be avoided for the purpose of evaluation of the stages of moyamoya disease.     

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。     

Moyamoya病代偿血流的评价

目的　研究 Moyamoya 病不同侧支循环的血流动力学特点。方法　DSA诊断的 Moyamoya 病患者,依据有无大脑后动脉和颈外动脉代偿进行分组,对比大脑后动脉和颈外动脉的峰值血流速度和脉动指数或阻力指数。通过受试者工作特征曲线分析,确定TCD评估标准。结果　有大脑后动脉参与代偿时,大脑后动脉 Vp较高, PI较低 (P < 0.001)。有颈外动脉参与代偿时, RI 较低 (P <0.001)。结论　TCD可以帮助临床评价Moyamoya 病的代偿情况。     

肢体抖动性短暂性脑缺血发作临床分析

目的 通过3例典型患者的病史和辅助检查，结合文献复习，探讨肢体抖动性短暂性脑缺血发作的临床表现及诊断，以提高诊断的准确率。方法 采用动态脑电图、超声多普勒、磁共振成像、磁共振动脉血管造影、全脑血管造影等辅助检查，对3例肢体抖动性短暂性脑缺血发作患者进行临床表现、辅助检查及影像学资料分析。结果 3例患者均有肢体抖动的临床表现，其中2例曾被误诊为继发性癫痫，给予抗癫痫治疗无效，行全脑血管造影、脑电图等检查。全脑血管造影提示3例颈内动脉系统均存在严重闭塞或狭窄性病变，而发作期脑电图检查未见癫痫波释放。给予抗血小板聚集等药物治疗，但仍偶有发作。2例施行血管内支架植入术后发作完全停止，1例由于血管迂曲明显，手术未获成功，经内科保守治疗症状缓解出院。结论 肢体抖动性短暂性脑缺血发作为临床少见短暂性脑缺血发作，其发作形式常表现为发作性、无意识的一侧肢体无力及抖动，和局灶性运动性癫痫发作相似，易误诊为局灶性癫痫。通过脑电图及全脑血管造影等检查可以明确诊断，从而提高对该病诊断的准确率，防止漏诊及误诊。     

Moyamoya disease: importance of the MRI-MRA combination and difficulties in management and follow-up in 7 cases

PURPOSE: To point out the importance of both MRI and MR angiography for the diagnosis and follow-up of moyamoya disease. Materials and methods: We report seven cases. MRI was performed in the initial phase in three cases. MR angiography without conventional angiography led to the diagnosis in one case. Conventional angiography was performed in six cases. Follow-up MRI and MR angiography were obtained in two. RESULTS: The initial MRI revealed ischemic lesions in the sylvian territory in two cases, and a cerebral malformation in one, after-effect lesions in two, and cerebral atrophy and ventricular dilatation in one. Conventional angiography provided the diagnosis of moyamoya disease in all cases, associated with cerebral arteriovenous malformation in two. CONCLUSION: MRI and MRA should be performed for the diagnosis and follow-up of moyamoya disease.     

The significance of duplex ultrasonography examination in the acute cardioembolic stroke patients--with special attention to evaluation of internal carotid arteries]

The aim of this study is to clarify whether it is possible to diagnose occlusions of internal carotid arteries in the acute cardioembolic stroke patients by observing internal carotid arteries with duplex ultrasonography. Twenty-two consecutive patients underwent both duplex ultrasonography examination and cerebral angiography within 24 hours from the onset. Two cases who had no flow velocity and 8 cases who had systolic flow velocity but no anterograde diastolic flow velocity were all diagnosed as having internal carotid occlusions by cerebral angiography. Eleven of 22 cases who had anterograde diastolic flow velocity were diagnosed as having occlusions at distal side of M 1 portion or no occluded lesions. Besides, among 11 cases who were diagnosed as having occlusions of internal carotid arteries by cerebral angiography, five had end diastolic ratios (ED ratio) of common carotid arteries that were less than 4.0s. It is useful to evaluate the existence of anterograde diastolic flow velocity by duplex ultrasonography for the diagnosis of internal carotid occlusions in the acute cardioembolic stroke patients.     

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.     

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。     

磁共振血管成像、经颅多普勒及颈动脉超声对颈动脉系统短暂性脑缺血发作的诊断价值

目的 探讨磁共振血管成像（MRA）、经颅多普勒（TCD）和颈动脉超声对颈动脉系统短暂性脑缺血发作（TIA）的诊断价值。方法 对42例颈动脉系统TIA患者进行MRA、TCD和颈动脉超声检测,观察其异常情况。结果 颈动脉MRA异常12例（28．6％）,脑MRA异常32例（76．2％）,其中8例二者均异常,大多为颈动脉轻～重度狭窄;TCD异常30例（71．4％）（此30例脑MRA均异常）;颈动脉超声异常16例（38．1％）（其中颈动脉MRA异常12例）。结论 MRA、TCD和颈动脉超声3种方法联合应用,可对颈动脉系统TIA的病因及诊断作出客观的评价。     

Moyamoya syndrome in a patient with congenital human immunodeficiency virus infection.

A 10-year-old boy with congenital human immunodeficiency virus (HIV) infection developed recurrent episodes of left hemiparesis at age 7 years. The progression of his disease was followed by computed tomography, magnetic resonance imaging, magnetic resonance angiography, and cerebral angiography. The series of images showed progressive stenosis of both carotid arteries at the suprasellar origin with involvement of his anterior and middle cerebral arteries, while prominent collateral vessels developed from his external carotid supply through ophthalmic and middle meningeal arteries. The pattern of cerebrovascular disease is consistent with moyamoya syndrome. We suggest that further studies on the pathophysiology of cerebrovascular disease in patients with HIV could be helpful in understanding the cause of moyamoya disease as well. Also, with the various advances in treatment of HIV, neurovascular complications could be seen more frequently as the long-term survival in these patients improves.     

A 10-year-old girl with coexistent moyamoya disease and Graves' disease

There are rare reports of young women with moyamoya disease associated with Graves' disease; we are unaware of any previous reports of this association in prepubescent girls. We report a 10-year-old girl who presented with multiple bilateral strokes. Cerebral angiography demonstrated moderate to severe stenosis of her bilateral distal internal carotid arteries and proximal anterior and middle cerebral arteries, which was greater on the right. Thyroid function tests demonstrated suppressed thyroid-stimulating hormone and elevated thyroid hormone levels. Serum antiphospholipid antibody screen demonstrated mild elevations of antiocardiolipin IgG. Possible mechanisms predisposing individuals to concurrent moyamoya and Graves' disease are discussed.     

成人烟雾病17例临床表现和影像学特点分析

目的探讨成人烟雾病的临床和影像学改变特点。方法回顾性分析17例经常规数字减影血管造影(DSA)、磁共振血管造影(MRA)和/或MRI检查确诊的成人烟雾病患者的临床和影像学资料。结果 17例患者中表现为缺血性脑卒中12例(70.6%),主要表现为偏瘫、失语、头痛及智能减退,还可表现为短暂性脑缺血发作;出血性脑卒中5例(29.4%),主要表现为头痛、意识障碍、偏瘫及抽搐。MRI检查发现缺血性脑卒中累及额叶8例,底节区3例,深部脑白质3例,枕叶3例,颞叶1例;累及单侧半球4例,双侧半球8例;脑萎缩6例。环池内烟雾血管影1例,双侧大脑中动脉累及10例。出血性脑卒中患者中单纯脑室出血2例,蛛网膜下腔出血1例,脑室出血并脑干出血1例,脑室出血并丘脑出血1例。MRA均发现双侧颈内动脉或其分支狭窄或闭塞。全脑DSA检查7例,其中双侧颈内动脉狭窄、闭塞5例,双侧大脑前动脉狭窄或闭塞4例,双侧大脑中动脉狭窄或闭塞3例。结论成人烟雾病临床表现多样,临床特点与责任病灶及支配血管相关;MRI和/或MRA有助于烟雾病的早期诊断,确诊需行全脑DSA检查。     

The Evaluation of Symptomatic Carotid Artery Disease

Severe, symptomatic carotid artery disease is most often diagnosed by conventional cerebral angiography. Noninvasive tests are commonly used to identify candidates for angiography and endarterectomy. The purpose of this study, a mail and telephone survey of academic and community neurologists, neurosurgeons, and vascular surgeons in Rhode Island in 1994, was to determine which noninvasive tests physicians used to evaluate these patients and how the test results were used to select patients for angiography. One hundred (86%) of a possible 116 responses were collected. Seventy-six percent of physicians chose carotid duplex ultrasound as the first diagnostic test, a percentage significantly higher than that for any other test (p < 0.0001). Fourteen percent chose angiography without a prior screening test, 3% chose magnetic resonance angiography (MRA) prior to angiography, 6% chose carotid duplex ultrasound plus MRA prior to angiography, and 1 % chose MRA without angiography. The specific noninvasive test results required for angiography referral were surprisingly variable, both within and across the three physician specialties. Vascular surgeons and neurosurgeons were more likely to choose angiography without prior screening tests than were neurologists. In conclusion, the majority of specialty physicians in the state of Rhode Island chose carotid ultrasound as the screening test of choice for the evaluation of a potential endarterectomy candidate. Few responding physicians chose MRA in combination with carotid duplex ultrasound prior to angiography, or MRA prior to angiography or MRA alone. Awareness of the actual practice patterns of specialists may lead to programs of education for physicians to improve practice and patient outcome through more refined use of diagnostic tests prior to angiography or endarterectomy.     

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.     

甲状腺功能亢进症合并烟雾综合征

目的 分析和探讨经颅多普勒超声（TCD）筛查的甲状腺功能亢进症（甲亢）合并烟雾综合征的临床和 血管病变特征。 方法 总结北京协和医院和解放军307医院2000～2007年经TCD筛查诊断烟雾综合征患者13例，8例经 磁共振血管成像（MRA）、5例经数字减影血管造影（DSA）明确诊断；所有患者经协和医院内分泌科确 诊为甲亢，且甲状腺球蛋白抗体（TGAb）和髓过氧化物酶抗体（TPOAb）高，除外血管狭窄的其他常见 原因。 结果 13例患者中，男1例，女12例；11例表现为缺血性卒中（2例合并不自主运动），1例仅表现为不自 主运动，1例以头晕为临床主诉，后2例经TCD筛查发现血管狭窄。血管病变分布：11例为典型的颈内动脉 终末端（TICA）、大脑中动脉（MCA）、大脑前动脉（ACA）狭窄或闭塞，1例为颈内动脉虹吸段狭窄，1例 为双侧颈内动脉起始严重狭窄或闭塞。2例患者同时合并大脑后动脉（PCA）狭窄。随访发现，1例患者因 甲亢控制不佳复查TCD血管病变加重；而1例甲状腺功能稳定，缺血临床症状明显改善。4例患者行颞浅 动脉－大脑中动脉（MCA）血管融通术，术后缺血发作减少。 结论 TCD的临床应用使越来越多的甲亢合并烟雾综合征得以诊断；烟雾综合征患者常规行甲状腺 功能筛查十分必要；血管成形术可能是预防缺血性卒中的有效方法之一。     

Moyamoya disease in children

Moyamoya disease is an uncommon cerebrovascular disease characterized by progressive steno-occlusive changes in the terminal internal carotid arteries (ICA) and their main branches, associated with the development of moyamoya vessels. The incidence of the disease is high in east Asia, especially in Japan and Korea. The familial form accounts for 10-15%. Moyamoya disease has two age distribution peaks at around 5 and 40 years. Most pediatric patients exhibit transient ischemic attacks or infarction. Headache and involuntary movements are serious symptoms associated with pediatric moyamoya disease. MRI and MR angiography (MRA) are useful and non-invasive methods for diagnosing or monitoring moyamoya disease. Cerebral angiography is still the gold standard for a diagnosis, however, it is not mandatory when MRI and MRA show typical findings of moyamoya disease; steno-occlusive changes at the ends of ICA and an abnormal vascular network in the basal ganglia. Other MRI findings have been reported, including T2 shortening in the white matter, the ivy sign on fluid-attenuated inversion recovery (FLAIR) images, and medullary streaks on FLAIR or enhanced T1-weighted images.     

Usefulness of carotid ultrasonography for the early detection of moyamoya disease]

We report carotid ultrasonographic findings in moyamoya disease. A 44-year-old man was admitted to our hospital because of fever, headache and nausea. Neurological examination showed neck stiffness and Kernig's sign but he was otherwise normal. Brain computed tomography showed hemorrhage in the right thalamus and the lateral ventricle. Conventional carotid ultrasonography (CCU) detected marked narrowing of the right internal carotid artery (ICA) at the proximal portion without arteriosclerosis, which fulfilled the criteria of bottle neck sign, namely, the ratio of diameter of proximal portion of ICA to that of the distal portion of common carotid artery (CCA) was less than 0.5. Additionally, CCU as well as transoral carotid ultrasonography (TOCU) showed the diameter of the ICA to be smaller than that of the external carotid artery (ECA) (diameter reversal sign). These signs strongly suggested moyamoya disease. Cerebral angiography confirmed the occlusions of intracranial ICA and moyamoya vessels. Bottle neck sign and diameter reversal sign of the carotid artery on carotid ultrasonography are useful for the early detection of moyamoya disease.     

25例烟雾病临床影像特点观察

目的探讨烟雾病的临床特征、影像学特点。方法对25例烟雾病患者首发症状、临床表现及影像学结果进行分析。结果烟雾病发病平均年龄较轻,女性多于男性;少儿烟雾病患者以缺血改变为主(87.5%),成年烟雾病患者缺血改变(58.8%)与出血改变(41.2%)相当;临床以头痛、头晕、肢体无力或偏瘫或交替性瘫痪为主要表现,同时可伴失语、智能下降、视力下降、癫痫发作、小舞蹈发作等表现。14例行头CT检查仅示颅内病灶,均未提示烟雾病;25例均行MRI和MRA检查发现颅内病灶,显示颈内动脉虹吸末段和大脑前或中动脉近段狭窄或闭塞,并有脑基底部异常血管网及侧支循环形成,均被诊断为烟雾病。其中9例又行数字减影血管造影术(DSA),结果与其MRA相符。结论头颅MRI和MRA是诊断烟雾病理想的非介入性检查方法。     

Hemodynamic Effects of Innominate Artery Occlusive Disease on Anterior Cerebral Artery

Stenoses of the innominate artery (IA) may affect flow conditions in the carotid arteries. However, alternating flow in ipsilateral anterior cerebral artery (ACA) due to IA stenosis is extremely rare. A 49-year-old woman who was evaluated for symptomatic cerebrovascular disease presented with right latent subclavian and right carotid system steal. Transcranial Doppler examination displayed systolic deceleration wave-forms in the right terminal internal carotid artery and alternating flow in the right ACA. Magnetic resonance angiography demonstrated tight stenosis of the right IA. For a thorough study of the hemodynamic effects of IA stenosis, a combination of duplex and transcranial Doppler examination is required.     

Diagnosis of Moyamoya Disease with Transcranial Doppler Sonography: Correlation Study with Magnetic Resonance Angiography

BACKGROUND AND PURPOSE: Although the diagnosis of moyamoya disease may be confirmed by digital subtraction angiography, recent studies have shown the accuracy of magnetic resonance angiography. Characteristics of transcranial Doppler, a noninvasive and cost-effective method, and specific transcranial Doppler parameters reflecting distinct vascular status in moyamoya disease are explored. METHOD AND PATIENTS: Consecutive patients (> 15 years of age) diagnosed with moyamoya disease by a typical clinical history and digital sub traction angiography or magnetic resonance angiography were included. The statuses of the anterior, middle, and posterior cerebral arteries were graded as stage 1, stage 2, and stage 3 by magnetic resonance angiography. Mean flow velocity and pulsatility index were compared between these groups, and the receiver operating characteristic analysis was used to define transcranial Doppler criteria for distinct vascular status. RESULTS: Forty-five patients were included (37 women; mean age, 34.9 +/- 11.4 years). Mean flow velocity was higher and pulsatility index was lower in stage 2 (P < .01), while mean flow velocity was lower and pulsatility index was higher in stage 3 than in stage 1 (P < .01). Cutoff values reflecting stenosis or occlusion with substantial sensitivity and specificity were as follows: mean flow velocity > 85 cm/s or pulsatility index < 0.60 for stage 2, and mean flow velocity < 50 cm/s for stage 3 of middle cerebral artery; mean flow velocity > 80 cm/s or pulsatility index < 0.60 for stage 2 of anterior cerebral artery; and mean flow velocity > 60 cm/s or pulsatility index < 0.60 for stage 2 of posterior cerebral artery. CONCLUSION: Transcranial Doppler may help to refine magnetic resonance angiography findings and thus help clinicians differentiate severity or stages of moyamoya disease.