Are the strokes in moyamoya syndrome associated with Down syndrome due to protein C deficiency?

Moyamoya syndrome has occasionally been seen in association with Down syndrome. We report a child with moyamoya syndrome and Down syndrome who was admitted with repeated episodes of strokes; his magnetic resonance imaging and magnetic resonance angiography findings confirmed the presence of occlusive cerebrovascular disease with basal collateral vessels. His protein C levels were significantly decreased during the stroke. Complete clinical recovery was seen during follow-up. This raises the possibility of a link between protein C deficiency and Down syndrome with moyamoya syndrome.     

Intraoperative infrared brain surface blood flow monitoring during superficial temporal artery–middle cerebral artery anastomosis in patients with childhood moyamoya disease

Objective  To monitor patency of the bypass and to accomplish comprehensive visualized evaluation of brain surface hemodynamics in childhood moyamoya patients, we performed intraoperative monitoring using novel infrared imaging system. Materials and methods  Intraoperative monitoring of brain surface blood flow by IRIS V infrared imaging system has been conducted during superficial temporal artery–middle cerebral artery anastomosis on seven sides of five pediatric patients with moyamoya disease (man/woman = 2:3, 7–8 years old). The range of recipient artery was 0.7–0.8 mm (average 0.75 mm). Magnetic resonance imaging and magnetic resonance angiography were performed routinely before and after surgery. Conclusions  In all cases, patency of bypass, as well as detailed local hemodynamics and changes of brain surface temperature distribution could be evaluated. Intraoperative infrared system will be a feasible monitoring not only for noninvasive intraoperative evaluation of bypass patency but also for local hemodynamics even in patients with childhood moyamoya disease.     

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。     

Rational approach to treatment of moyamoya disease in childhood

Early diagnosis and treatment of moyamoya disease in children is essential to minimize residual mental and physiologic deficits. Current treatment of childhood moyamoya disease in Japan, preoperative evaluation of perfusion reserve as a surgical indication, and the role of noninvasive follow-up by magnetic resonance angiography are reported. Approximately 20% of children with definite moyamoya disease were observed or treated medically. Among surgical procedures, single indirect bypass surgery was used in approximately 30% of all patients; combinations of direct and indirect bypass surgery, 20%; and multiple-indirect bypass surgery, 18%. Both adequate understanding of the primary condition and determination of optimal treatment, including specific operative procedures, required evaluation of cerebral circulation and metabolism. Surgical indications included reduced perfusion reserve in affected brain by positron emission tomography or single photon emission tomography with administration of acetazolamide or a CO2 load. Postoperative improvements of cerebral perfusion reserve show better correlation with disappearance of ischemic attacks than does angiographically demonstrated collateral formation. Follow-up evaluation with magnetic resonance angiography has advantages over conventional angiography because it is noninvasive and avoids general anesthesia.     

Acute childhood hemiplegia associated with chickenpox and elevated anticardiolipin antibody

Chickenpox is a common infection of childhood. Central nervous system complications, such as cerebellar ataxia and meningoencephalitis, are estimated to occur in less than 1% of cases of chickenpox. Although transverse myelitis and hemiplegia have also, although rarely, been reported, hemiplegia associated with chickenpox and elevated anticardiolipin antibody has not been reported in the literature. We report the case of a 2.5-year-old boy who developed a right hemiplegia 3 weeks after the onset of a primary varicella infection. The serum level of anticardiolipin antibody IgG was markedly elevated. Brain magnetic resonance imaging (MRI) revealed an infarction involving the left globus pallidus, caudate nucleus, and posterior leg of the internal capsule. Laboratory studies ruled out all known causes of stroke. Neurologic signs and symptoms slowly improved with supportive treatment. Varicella infection should be considered one of the possible causes of acute ischemic strokes in children. Usually, two or more risk factors are detected in children with ischemic strokes, and we suggest that all possible causes of strokes be investigated in children with ischemic strokes.     

Idiopathic recurrent transverse myelitis

OBJECTIVE: To determine whether idiopathic recurrent transverse myelitis (RTM) can be distinguished from multiple sclerosis-associated RTM (MSRTM) on the basis of clinical manifestations of myelopathy, or findings from magentic resonance imaging or cerebrospinal fluid examination. DESIGN: A retrospective analysis of 37 cases was conducted. Patients were classified as having idiopathic RTM on the basis of recurrent myelitis confirmed by clinical manifestations of myelopathy and magnetic resonance imaging findings. On review patients with idiopathic RTM had normal cranial magnetic resonance imagings and did not demonstrate paraclinical evidence of spatial dissemination beyond the spinal cord of the disease process. Patients were classified as having MSRTM on the basis of criteria of Poser et al for clinically definite multiple sclerosis involving the central nervous system. Fifteen patients met study criteria for idiopathic RTM. Twenty-two patients had MSRTM. SETTING: Asan Medical Center, Seoul, South Korea, from January 1, 1992, through December 31, 2001. MAIN OUTCOME MEASURES: Presenting symptoms and clinical manifestations, relapsing times, magnetic resonance imaging features (involved spinal cord segments in T2-weighted images and gadolinium 64-enhanced lesions on T1-weighted images), IgG index, and oligoclonal bands in cerebrospinal fluid were compared. RESULT: Idiopathic RTM occurred preponderantly in male patients and presented more often with acute transverse myelitis than did MSRTM. More than 2 relapses occurred in 6 cases (40%) of idiopathic RTM. The involved segments of spinal cord on T2-weighted images were not significantly different in idiopathic RTM and MSRTM, with enhancing lesions mostly in the posterior columns, and the spinothalamic and spinocerebellar tracts of white matter. Additionally, almost all patients with idiopathic RTM had normal cerebrospinal fluid indexes. CONCLUSION: Idiopathic RTM might be a disease entity distinct from MSRTM, differing in its male preponderance, absence of oligoclonal bands, frequent multiple relapses, and frequent presentation as acute transverse myelitis.     

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.     

Moyamoya Phenomenon Secondary to Intracranial Atherosclerotic Disease: Diagnosis by 3T Magnetic Resonance Imaging

Moyamoya phenomenon occurs in response to an occlusive vasculopathy affecting the distal internal carotid artery and its proximal branches. The nature of the occlusive vasculopathy is unknown in most patients. We present a patient in whom 3T magnetic resonance imaging was used to examine the arterial wall at the site of occlusion in a patient with unilateral moyamoya phenomenon. Signal characteristics were consistent with atherosclerotic disease. 3T magnetic resonance imaging may be useful for distinguishing the underlying etiology of moyamoya phenomenon in some patients.     

Pediatric moyamoya disease presenting with intracerebral hemorrhage--report of three cases and review of the literature

Intracerebral hemorrhage in patients with moyamoya disease is rare in children. We report three unique cases of pediatric moyamoya disease with hemorrhagic onset. Two 7-year-old girls and a 9-year-old girl were admitted to our hospital because of intracerebral hemorrhage associated with angiographically verified moyamoya disease. Two of them did not demonstrate either an ischemic episode or cerebral infarct on the magnetic resonance images. A decreased regional cerebral blood flow was revealed on single photon emission computed tomography in two patients, who developed cerebral infarction in the acute stage following hemorrhage. They underwent superficial temporal artery-middle cerebral artery anastomoses combined with encephalo-myo-synangiosis, and have not experienced any further ischemic episodes thereafter. Hemodynamic insufficiency associated with moyamoya disease could cause intracerebral hemorrhage even in children. Adequate management in the acute stage of hemorrhage and revascularization surgery are recommended to prevent cerebral infarction, which may easily occur in pediatric patients with 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.     

“Recycling” A Failed Superficial Temporal Artery Indirect Bypass into a Double Barrel Direct Bypass in Moyamoya Disease

ObjectivesMoyamoya disease is an idiopathic cerebrovascular disorder in which patients experience recurrent transient ischemic attacks, ischemic or hemorrhagic strokes, headaches, and seizures from progressive stenosis of the vessels of the anterior circulation. The mainstay of treatment in symptomatic patients is surgical revascularization. Here, we present the case of a moyamoya patient in which a failed encephaloduroarteriosynangiosis, after new strokes, is recycled and converted into a combined “double barrel” direct superficial temporal artery to middle cerebral artery bypass with included video.Case report/resultsWe describe a 37-year-old woman with a history of hypertension, obstructive sleep apnea, celiac disease, and moyamoya disease complicated by multiple ischemic strokes who presented with progressive dysarthria, dysphagia, and new left-sided ischemic infarcts. The patient had previously undergone right-sided direct bypass and left-sided encephaloduroarteriosynangiosis in the setting of multiple ischemic strokes. After more strokes, the patient underwent a left-sided frontotemporoparietal craniotomy for conversion of the failed indirect bypass into a “double barrel” direct bypass.ConclusionsThe literature is divided over which revascularization procedure should be preferred. Irrespectively, most failed bypass grafts are repaired via direct bypasses, to good effect. We highlight a case in which a failed indirect bypass is directly incorporated into a combined direct bypass with resulting restoration of blood flow. In the case of a failed indirect bypass in an adult patient with moyamoya disease, this method provides the operator with an additional option for restoration of perfusion, especially in patients without other viable vessels available for anastomosis.     

Moyamoya disease associated with stenosis of extracranial arteries: a case report and review of the literature

We report on a 6-year-old boy with a 2.5-year history of recurrent cerebral ischemic strokes and moderate systemic hypertension. Brain angio-MR examination revealed stenosis of cerebral arteries with a network of collateral vessels that are characteristic features for moyamoya disease. However, further examination, including angio-CT of abdominal vessels, revealed a significant stenosis of the aorta, celiac trunk and bilateral renal arteries. As the literature shows worse prognosis for moyamoya hypertensive patients having their blood pressure reduced, our patient, though hypertensive, remains disqualified for blood pressure reduction.     

Clinical and MRI patterns of pericallosal artery infarctions: the significance of supplementary motor area lesions

Cerebral infarctions in the anterior cerebral artery (ACA) territory account for only up to 3-5% of strokes. Subject to the affected ACA branches, different clinical patterns can be defined. We report a case series of patients with isolated infarctions of the pericallosal (PC) artery territory. We analyzed 36 consecutive patients presenting over a 10-year period with isolated PC artery territory infarctions (15 left-sided, 19 right-sided, 2 bilateral) regarding clinical symptoms as well as MRI findings. Analysis of DWI lesion pattern showed complete PC artery infarctions in three patients. The majority of patients had partial infarctions predominantly involving either the superior frontal gyrus (n = 12), the corpus callosum (n = 1) or both (n = 20). Hemodynamic lesion patterns were found in 13 patients, while multiple cortical emboli occurred in six. Distal pathology of the ACA (n = 13) was the most frequent MRA finding. Core symptom was contralateral hemiparesis with lower limb predominance (n = 29), partly associated with early-onset spasticity. Interestingly, motor evoked potential recording was abnormal in only five patients. Further characteristic symptoms were psychomotor slowing (n = 9), often with speech disturbances such as decreased verbal fluency, and confusional state (n = 4). Visual or motor hemineglect (n = 5) as well as apraxia (n = 5) was confined to a few patients only. Pericallosal artery infarctions are a rare localization of stroke, mostly occurring as partial infarctions due to distal ACA pathology. Clinically, they are mainly characterized by hemiparesis predominately in the lower limb caused by involvement of supplementary motor cortex areas without affection of the corticospinal tract.     

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.     

烟雾病的MRI和MRA诊断

目的探讨磁共振成像(MRI)和磁共振动脉成像(MRA)对烟雾病的诊断价值。方法回顾性分析22例烟雾病患者的临床和影像资料,全部行MRI和MRA(3D-TOF法)检查,其中10例进行了DSA检查。结果MRI上脑实质内均出现异常信号,其中脑梗死8例,脑出血7例,脑萎缩3例,脑软化4例,8例患者在基底节区有血管流空现象。MRA均良好显示病变血管狭窄或闭塞,呈多支受累,与DSA所见相似。结论MRI结合MRA是诊断烟雾病的无创和有效手段。     

Moyamoya disease in childhood: a familial case report

Moyamoya is an obstructive cerebrovascular disease characterized by a cerebral angiographic picture of stenosis or occlusion of main cerebral arteries with an abnormal vascular network at the base of the brain. No definitive cause has been found for this disease and opinion is still divided between a congenital and an acquired etiology. Hemiplegia of sudden onset and epileptic seizures are the prevailing presentation in childhood, while subarachnoid bleeding occurs more frequently in adults. We report a new case of childhood moyamoya with clinical onset of the neurological symptoms within the 3rd year of life; during the child's illness the maternal grandmother presented with moyamoya disease too. Antiaggregating and calcium-antagonist drugs seem effective in preventing further vascular accidents, while a surgical approach is not possible. Computed tomography, single positron emission computed tomography, and magnetic resonance imaging are very useful in the diagnosis of this rare disease.     

Moyamoya disease in children A review of the clinical and radiological features and current treatment

Two cases of moyamoya disease observed in two children are reported. The two cases recall the frequency of ischemic strokes, transient ischemic attacks, and seizures revealing the disease. Diagnosis is made by cerebral arteriography showing carotid stenosis and the dense deep arterial collateral as seen in these two cases. Magnetic resonance imaging makes it possible to identify this vascular disease as it reveals vascular varicosities in the basal ganglia, as in case 1. Strokes can sometimes be prevented by constructing an anastomosis between the superficial temporal artery and the middle cerebral artery, as demonstrated in case 1. Thus, the diagnosis of moyamoya disease has been improved by magnetic resonance imaging and there is now a surgical treatment to prevent ischemic stroke.     

Spectrum of anterior cerebral artery territory infarction: clinical and MRI findings

To evaluate and review the clinical spectrum of anterior cerebral artery (ACA) territory infarction, we studied 48 consecutive patients who admitted to our stroke unit over a 6-year period. We performed magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) in all patients, and diffusion magnetic resonance imaging (DWI) in 21. In our stroke registry, patients with ACA infarction represented 1.3% of 3705 patients with ischemic stroke. The main risk factors of ACA infarcts was hypertension in 58% of patients, diabetes mellitus in 29%, hypercholesterolemia in 25%, cigarette smoking in 19%, atrial fibrillation in 19%, and myocardial infarct in 6%. Presumed causes of ACA infarct were large-artery disease and cardioembolism in 13 patients each, small-artery disease (SAD) in the territory of Heubner's artery in two and atherosclerosis of large-arteries (<50% stenosis) in 16. On clinico-radiologic analysis there were three main clinical patterns depending on lesion side; left-side infarction (30 patients) consisting of mutism, transcortical motor aphasia, and hemiparesis with lower limb predominance; right side infarction (16 patients) accompanied by acute confusional state, motor hemineglect and hemiparesis; bilateral infarction (two patients) presented with akinetic mutism, severe sphincter dysfunction, and dependent functional outcome. Our findings suggest that clinical and etiologic spectrum of ACA infarction may present similar features as that of middle cerebral artery infarction, but frontal dysfunctions and callosal syndromes can help to make a clinical differential diagnosis. Moreover, at the early phase of stroke, DWI is useful imaging method to locate and delineate the boundary of lesion in the territory of ACA.     

30例烟雾病的临床及影像学特征分析

目的研究烟雾病的临床特点、影像表现及其两者之间关系。方法对30例经磁共振血管成像(MRA)和数字减影全脑血管造影(DSA)确诊的烟雾病患者,分析其临床和影像学特征。结果(1)烟雾病发病年龄在35～45岁左右,发病以脑缺血为主,临床表现以单侧肢体无力或偏瘫最为常见。(2)CT和MRI提示,脑出血11例(36.7%),脑梗死10例(33.3%),脑出血合并梗死灶5例。MRA和DSA均发现大脑前、中、后动脉狭窄或闭塞性病变,双侧病变均多于单侧病变,烟雾状异常血管网分别为5例(45.5%)和29例(100.0%);DSA发现动脉瘤3例(10.3%);后交通动脉增粗13例、前交通动脉增粗7例、眼动脉增粗6例及软脑膜吻合支增多4例。4例大面积脑梗死侧枝循环差。结论烟雾病发病高峰为35～45岁;本病临床表现多样,但以脑缺血常见;MRI和MRA为无创伤性,为本病筛选、诊断、随访的重要方法。对临床疑似病例应及早行DSA检查。     

Cerebral blood flow velocities in an infant with moyamoya disease

Moyamoya disease is a progressive cerebrovascular disorder with bilateral occlusion of the basal circulation and development of collateral blood supply. In a 6-month-old female with multifocal ischemic infarctions, transcranial pulsed Doppler sonography revealed extremely high and low cerebral blood flow velocities, dampened waveforms, reversed flow, and musical murmurs. Magnetic resonance angiography revealed different degrees of vascular stenosis and an abnormal collateral network. Moyamoya disease was confirmed by conventional angiography at the age of 10.5 months. Pulsed-wave transcranial Doppler sonography is a noninvasive screening method in infants at risk of moyamoya disease.