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.     

Usefulness of carotid duplex ultrasonography in a patient with moyamoya disease]

The patient is a 35-year-old man who had a medical history of epilepsy in childhood. He came to our hospital because of transient disturbance of consciousness and left hemiplegia just after evacuation. At first, we thought that he had epilepsy with Todd's palsy. But we had to do a differential diagnosis for a transient ischemic attack such as paradoxical embolism, because his symptoms occurred just after evacuation. An electroencephalogram and brain computerized tomography were immediately performed, but no abnormality was detected. Hematologic studies were normal, and no deep vein thrombosis was detected in the veins of the lower extremities by duplex ultrasonography Doppler. But carotid duplex ultrasonography showed an increase in end-diastolic flow velocity and a decrease in vascular resistance in both external carotid arteries. These findings indicated that there was arteriovenous malformation such as moyamoya disease. Brain magnetic resonance imaging showed spotty high signal lesions in the subcortical areas on a fluid-attenuated inversion-recovery(FLAIR) image, and the middle cerebral artery was not visualized on magnetic resonance angiography (MRA). Cerebral angiography demonstrated moyamoya vessels in the brain and collateral circulation from the external carotid artery. Therefore, we diagnosed him as having moyamoya disease. Duplex ultrasonography of the common and, internal carotid, and vertebral arteries is a widely-used technique. Recently, cerebral angiography, MRA and transcranial Doppler have been applied to detect intracranial vascular malformation. But these results suggested that moyamoya disease could be detected by means of carotid duplex ultrasonography. Finally, we considered that carotid duplex ultrasonography was not only a noninvasive screening method but also a useful for the diagnosis of moyamoya disease.     

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.     

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.     

Moyamoya disease associated with hereditary spherocytosis

A 5-year-old girl with hereditary spherocytosis presented with two episodes of transient ischemic attacks within a month. Cranial magnetic resonance imaging angiography revealed a left internal carotid artery and middle cerebral artery stenosis, with an extensive vascular mesh in the thalamic area indicative of moyamoya disease. Treatment consisted of supporting cerebral perfusion with blood transfusions, and splenectomy to prevent recurrence. Moyamoya disease is a very unusual cerebrovascular disorder in childhood and its association with hereditary spherocytosis is rarely reported.     

Moyamoya disease associated with Behcet's disease.

A 32-year-old woman with Behcet's disease suffered repeated transient ischemic attacks (TIA) consisting of left hemiparesis. Cerebral angiography revealed the typical findings of moyamoya disease, with occlusion of the supraclinoid portion of both internal carotid arteries, coupled with abnormal collateral vessels. She underwent right superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis and encephalomyosynangiosis, due to decreased reserve capacity demonstrated on acetazolamide single positron emission computed tomography (SPECT). Postoperatively, the TIA symptoms subsided. This is the first report of moyamoya disease associated with Behcet's disease, and moyamoya disease should be considered in the differential diagnosis of cerebrovascular events in patients with Behcet's disease. Revascularization surgery is recommended for the prevention of ischemic insults resulting in permanent deficits.     

Moyamoya disease presenting with paroxysmal exercise-induced dyskinesia

We report a patient with moyamoya disease presenting with paroxysmal exercise-induced dyskinesia (PED). A 31-year-old lathe man developed recurrent attacks of paroxysmal hemichorea. The attacks always affected his left limbs and occurred either after several hours of working or while playing football. The duration of attacks ranged from 30 min to 4h. Attacks were not provoked by sudden movements, consumption of coffee or alcohol, hyperventilation, emotional stress, exposure to cold or passive movement. An MRI of the brain showed no parenchymal lesions. However, (99m)Tc-ethylcysteine dimer SPECT study showed hypoperfusion in the right striatum. Digital subtraction angiography showed stenosis of the right internal carotid and middle cerebral artery with prominent basal collaterals, which was compatible with moyamoya disease. Imaging studies of the cerebral arteries should be done in patients with clinical features of PED in order to detect possible cases of moyamoya disease.     

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.     

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.     

Comparison of magnetic resonance angiography, conventional angiography, and duplex scanning.

BACKGROUND AND PURPOSE: To determine the accuracy of magnetic resonance angiography in assessing patients with cerebrovascular disease, we performed a study comparing the results of conventional cerebral angiography, duplex scanning, and magnetic resonance angiography. METHODS: From 42 patients, a total of 25 carotid arteries were evaluated by all three techniques. The studies were independently read and sorted into five categories according to the degree of stenosis: 0-15%, normal; 16-49%, mild; 50-79%, moderate; 80-99%, severe; and totally occluded. RESULTS: Magnetic resonance angiography correlated exactly with conventional angiography in 39 arteries (52%); duplex scanning correlated with conventional angiography in 49 cases (65%). Compared with conventional angiography, both magnetic resonance angiography and duplex scanning tended to overread the degree of stenosis. The most critical errors associated with magnetic resonance angiography were three readings of total occlusion in vessels found to be patent on conventional angiograms. CONCLUSIONS: Although magnetic resonance angiography offers great hope of providing high-quality imaging of the carotid artery with no risk and at less cost, data from this study suggest that misreading the degree of stenosis, or misinterpreting a stenosis for an occlusion, could lead to errors in clinical decisions. Guidelines for use of magnetic resonance angiography in a clinical setting are offered.     

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。     

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。     

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。     

Cardio-facio-cutaneous syndrome and moyamoya syndrome

We reported a patient with cardio-facio-cutaneous (CFC) syndrome associated with moyamoya syndrome. The patient was referred at 6 years 5 months with left hemiplegia and right-sided eye deviation. He had an apparently short stature, macrocephaly, left ptosis and atopic skin, and was odd looking. He exhibited an incomplete right bundle branch block on electrocardiogram and an atrial septal defect on ultrasound cardiography. He was diagnosed as having CFC syndrome. Head magnetic resonance imaging showed a flow void in the bilateral basal ganglia, but did not show any ischemic changes. Magnetic resonance angiography showed bilateral stenosis with an internal carotid artery at the Willis artery ring level and bilateral moyamoya. Contrast angiography demonstrated occlusion of both middle cerebral arteries. Cerebrovascular anomalies have not previously been reported in CFC syndrome. This is the first case of CFC syndrome associated with moyamoya syndrome.     

320排动态容积CT诊断脑血管病的价值

目的探讨320排动态容积cT诊断脑血管病的价值。方法对80例怀疑脑血管病患者行320排动态容积cT检查,经两位有经验的神经影像医师读片,评估其诊断价值。结果80例患者发现颅内血管性病变75例,其中60例为颅内动脉瘤,10例脑动脉硬化,3例脑动静脉畸形,2例烟雾病,5例未发现明确原因。结论320排动态容积CT-次扫描16cm容积数据采集,实现脑血管病cT平扫、脑血管造影及脑灌注一站式检查,具有快速、无创、准确性高等优点,对诊断脑血管疾病具有很高临床价值。     

A 10-year-old boy with Marfan syndrome exhibiting cerebrovascular abnormalities

A young male with Marfan syndrome, diagnosed at the age of 10 years, presented with conspicuous elongation and tortuosity of the internal carotid, middle cerebral, vertebral and basilar arteries on cranial magnetic resonance and computed tomography angiography. There is a little mention of cerebral blood vessel examinations in the guidelines of the American Academy of Pediatrics for Marfan syndrome. Guidelines may be provided for the evaluation of cerebrovascular system for the patients with Marfan syndrome who have family history of Marfan syndrome as well as a family history of death from subarachnoid hemorrhage.     

Hemidystonia and hemichoreoathetosis as an initial manifestation of moyamoya disease

OBJECTIVE: To describe hemidystonia and hemichoreoathetosis in an adult patient with moyamoya disease without a previous history of cerebrovascular accident. DESIGN: Case report. SETTING: Tertiary care center. PATIENT: A 22-year-old woman suddenly developed dystonic spasms in her left hand and left foot after a severe emotional stress. The dyskinesia gradually subsided over the next 4 months. Five months after the onset, she suddenly developed choreoathetoid movement in her right hand and right foot. MAIN OUTCOME AND RESULTS: The patient had both somatic and cortical sensory deficits in the right hand and right foot. Magnetic resonance imaging of the brain showed an infarction at the right putamen and lesions involving the right frontal lobe and the left frontotemporoparietal lobe. Magnetic resonance cerebral angiography showed severe stenoses of both internal carotid arteries at the supraclinoid portion and numerous collateral vessels, compatible with moyamoya disease. Single photon emission tomography of the brain showed hypoperfused areas at the right frontal and left frontotemporoparietal lobes. The choreoathetosis of the right limbs improved markedly, along with improvement of sensory deficits. CONCLUSIONS: To our knowledge, this is the first report of an adult patient presenting with hemidystonia and hemichoreoathetosis as the initial manifestations of moyamoya disease. Arch Neurol. 2000;57:1510-1512     

Cerebral hemodynamics in adult ischemic-type patients with moyamoya disease compared with those of atherothrombotic middle cerebral artery occlusion]

We measured regional cerebral blood flow (rCBF) in adult ischemic-type patients with moyamoya disease and in patients with atherothrombotic middle cerebral artery occlusion (MCAO) to investigate cerebral hemodynamics in adult ischemic-type of moyamoya disease. In this study we measured rCBF and regional cerebrovascular response (rCVR) using acetazolamide by Xenon-enhanced CT. Our subjects consisted of 15 adult ischemic-type patients with moyamoya disease and 27 atherothrombotic stroke patients with proximal occlusion of the middle cerebral artery. The region of interest was conducted in the anterior cerebral artery, middle cerebral artery and posterior cerebral artery territories as well as basal ganglia regions. rCBF was preserved in all regions of patients with moyamoya disease. However, rCVR severely decreased in the anterior circulation territory in patients with moyamoya disease compared with those of MCAO. These results suggest that rCBF in the anterior circulation territory of adult ischemic-type patients with moyamoya disease is preserved by vasodilation of the cerebral arteries, while cerebral hemodynamic reserve capacity is severely reduced. The results indicated that basal moyamoya vessels are dilated. These findings may be one of the reasons why stroke occurs more frequently in adult than child patients with moyamoya disease.     

Contrast-enhanced transcranial color-coded duplex sonography: efficiency and validity

OBJECTIVE: To evaluate the diagnostic efficiency and accuracy of contrast-enhanced transcranial color-coded sonography (CE-TCCS). BACKGROUND: TCCS is hampered by insufficient ultrasonic penetration in 20% of cerebrovascular patients. METHODS: In 47 patients whose basal arteries could not be assessed adequately, 59 TCCS examinations were performed before and after administration of the ultrasonic contrast agent (CA) Levovist. The assessability of different basal cerebral arteries after CA administration was evaluated off-line. Angiographic records were available from 11 patients. RESULTS: Satisfactory investigation of the middle cerebral artery, the anterior cerebral artery, the P1 and P2 segments of the posterior cerebral artery, and the supraclinoid portion of the internal carotid artery siphon was possible in 5.1%, 28.8%, 35.6%, 55.9%, and 47.5% of patients before, and in 84.7%, 91.5%, 93.2%, 94.5%, and 93.2% of patients after contrast enhancement. Stenoses or occlusions of basal cerebral arteries were registered in 28 patients (60%). CE-TCCS diagnosis was confirmed by digital subtraction angiography or magnetic resonance angiography in 10 of the 11 patients, leading to positive and negative predictive values of 0.86 and 1.00. CONCLUSION: Contrast enhancement improves the diagnostic potential of TCCS significantly in patients with temporal bone window failure, and proved to be a reliable method for detecting middle cerebral artery and siphon occlusion.     

The large striatocapsular infarct. A clinical and pathophysiological entity

We examined 29 patients with strictly subcortical large striatocapsular infarctions. Eight of them had aphasia or neglect. All patients underwent transcranial Doppler ultrasonography or selective carotid angiography, magnetic resonance imaging, and single photon emission tomography for assessment of cerebral blood flow, blood volume, and cerebral perfusion reserve. The signs were compatible with cortical territorial infarctions rather than lacunes. On both magnetic resonance imaging and computed tomographic scans, the lesions corresponded to the territories of the medial and lateral group of the lenticulostriate arteries, Heubner's artery, or the anterior choroidal artery. The infarctions were either due to cerebral embolization into the M1 segment of the middle cerebral artery or due to stenosis at the same site, ie, lesions that acutely and simultaneously occluded the orifices of the lenticulostriate or neighboring arteries. Persistent occlusion of the middle cerebral arteries and a decrease of cortical regional cerebral blood flow were only found in patients with aphasia or neglect. All patients without aphasia or neglect showed a rapid recanalization of the middle cerebral artery occlusion or a stenosis of the M1 segment and no cortical regional cerebral blood flow decrease. Large striatocapsular infarctions occur due to occlusive disease of the middle cerebral artery (large-vessel disease) and not due to a disseminated in situ occlusion of the long penetrating arteries (small-vessel disease), as in lacunes. Neuropsychological deficits can be explained by decreased cortical blood flow due to a persistent occlusive lesion of the middle cerebral artery.