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.     

Moyamoya syndrome precipitated by cranial irradiation for craniopharyngioma in children

Recently, combination of surgery and radiation therapy (RT) has been recommended in the treatment of craniopharyngioma. RT could be associated with late complications, including vasculopathy. We report two cases of the moyamoya syndrome seen in children with craniopharyngioma who received RT after surgical resection. Thirty-five patients in pediatric age with craniopharyngioma were surgically treated. Fifteen out of 35 patients underwent surgical resection followed by RT or gamma knife surgery. Two of the 15 were found to have symptoms of transient ischemic attack and were diagnosed as moyamoya syndrome through the cerebral angiography. Age at RT was 4 and 13 years, respectively. The latent period for development of the moyamoya syndrome was 27 months and 3 years, respectively, after RT. The RT dose of both patients was 54 Gy. These two patients received bilateral encephaloduroarteriosynangiosis procedures. We report here these two cases of radiation-induced moyamoya syndrome in pediatric craniopharyngioma. Pediatric patients with craniopharyngioma who received RT should be reminded, during follow-up, about the risk of development of the moyamoya syndrome.     

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.     

Sickle cell disease: ischemia and seizures

Although the prevalence of seizures in children with sickle cell disease (SCD) is 10 times that of the general population, there are few prospectively collected data on mechanism. With transcranial Doppler and magnetic resonance imaging (MRI) and angiography, we evaluated 76 patients with sickle cell disease, 29 asymptomatic and 47 with neurological complications (seizures, stroke, transient ischemic attack, learning difficulty, headaches, or abnormal transcranial Doppler), who also underwent bolus-tracking perfusion MRI. The six patients with recent seizures also had electroencephalography. Group comparisons (seizure, nonseizure, and asymptomatic) indicated that abnormal transcranial Doppler was more common in the seizure (4/6; 67%) and nonseizure (26/41; 63%) groups than in the asymptomatic (10/29; 34%) group (chi2; p = 0.045), but abnormal structural MRI (chi2; p = 0.7) or magnetic resonance angiography (chi2; p = 0.2) were not. Relative decreased cerebral perfusion was found in all seizure patients and in 16 of 32 of the remaining patients with successful perfusion MRI (p = 0.03). In the seizure patients, the perfusion abnormalities in five were ipsilateral to electroencephalographic abnormalities; one had normal electroencephalogram results. These findings suggest that vasculopathy and focal hypoperfusion may be factors in the development of sickle cell disease-associated seizures.     

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.     

“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.     

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.     

Multicenter prospective study of children with sickle cell disease: radiographic and psychometric correlation

After obtaining familial informed consent, between January 1996 and July 1997, 173 children (5 to 15 years old) with sickle cell disease were enrolled in a prospective multicenter study using blood screening, transcranial Doppler ultrasonography (n = 143), cerebral magnetic resonance imaging (n = 144), and neuropsychologic performance evaluation (n = 156) (Wechsler Intelligence tests WISC-III, WIPPSI-R), which were also performed in 76 sibling controls (5 to 15 years old). Among the 173 patients with sickle cell disease (155 homozygous for hemoglobin SS, 8 sickle cell beta0 thalassemia, 3 sickle cell beta+ thalassemia, 7 sickle cell hemoglobin C disease SC), 12 (6.9%) had a history of overt stroke, and the incidence of abnormal transcranial Doppler ultrasonography (defined as mean middle cerebral artery velocity > 200 cm/sec or absent) was 8.4% in the overall study population and 9.6% in patients with homozygous sickle cell anemia The silent stroke rate was 15%. Significantly impaired cognitive functioning was observed in sickle cell disease patients with a history of stroke (Performance IQ and Full Scale IQ), but also in patients with silent strokes (Similarities, Vocabulary, and Verbal Comprehension). However, infarcts on magnetic resonance imaging were not the only factors of cognitive deficit: Verbal IQ, Performance IQ, and Full Scale IQ were strongly impaired in patients with severe chronic anemia (hematocrit < or = 20%) and in those with thrombocytosis (platelets > 500 x 10(9)/L). Multivariate logistic regression analysis showed that abnormal magnetic resonance imaging (odds ratio [OR] = 2.76) (P = .047), hematocrit < or =20% (OR = 5.85) (P = .005), and platelets > 500 x 10(9)/L (OR = 3.99) (P = .004) were independent factors of cognitive deficiency (Full Scale IQ < 75) in sickle cell disease patients. The unfavorable effect of low hematocrit has already been suggested, but this is the first report concerning an effect of thrombocytosis and showing that silent stroke alone is not a factor of cognitive deficit when not associated with low hematocrit or thrombocytosis. The effect of hydroxyurea, which is known to increase hematocrit and decrease platelet count, on cognitive functioning of sickle cell patients should be evaluated prospectively.     

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.     

Radiographic predictors of neurocognitive functioning in pediatric Sickle Cell disease

We compared magnetic resonance imaging (MRI), magnetic resonance angiography, and transcranial Doppler ultrasonography as predictors of specific neurocognitive functions in children with sickle cell disease. Participants were 27 children with sickle cell anemia (hemoglobin SS) who were participants in the Stroke Prevention Trial in Sickle Cell Anemia (STOP) and had no documented history of stroke. Children's MRIs were classified as normal or silent infarct, and their magnetic resonance angiograms were classified as normal or abnormal. The highest time-averaged mean flow velocity on transcranial Doppler ultrasonographic examination of the major cerebral arteries was analyzed. Age and hematocrit also were analyzed as predictor variables. The battery of neurocognitive tests included measures of intellectual functioning, academic achievement, attention, memory, visual-motor integration, and executive functions. MRI, magnetic resonance angiography, transcranial Doppler ultrasonography, age, and hematocrit were analyzed as predictors of participants' performance on the various measures of neurocognitive functioning. Age and hematocrit were robust predictors of a number of global and specific neurocognitive functions. When age and hematocrit were controlled, transcranial Doppler ultrasonography was a significantly unique predictor of verbal memory. We found an association between low hemoglobin and neurocognitive impairment. We also found that abnormalities on transcranial Doppler ultrasonography can herald subtle neurocognitive deficits. (J Child Neurol 2006;21:37-44).     

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.     

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。     

Antiphospholipid antibody syndrome in childhood strokes

The significance of antiphospholipid antibodies was examined in patients with childhood strokes. Eight patients, aged 2–13 years, who presented with acute hemiplegia were studied. On the basis of magnetic resonance imaging, magnetic resonance angiography, single photon emission computed tomography, and cerebral angiographic findings, 3 children were diagnosed as having infarctions due to moyamoya disease, and the others as having idiopathic infarctions. Serologic studies revealed elevated anticardiolipin antibody (ACA) IgG in 3 of the 5 patients with idiopathic infarction; no such elevation was revealed in patients with moyamoya disease. Values for all other tests, including ACA IgM and lupus anticoagulant, were within normal limits or negative in all patients. ACA IgG, therefore, may be a more important causative agent of childhood strokes than was previously considered.     

Additional Revascularization Using Multiple Burr Holes for PCA Involvement in Moyamoya Disease

In specific cases of moyamoya disease (MMD), posterior cerebral artery (PCA) stenosis can develop after treatment of the anterior circulation and require additional revascularization. Here, we report two cases that underwent additional posterior indirect revascularization with multiple burr holes for PCA involvement after bilateral revascularization treatment of the anterior circulation. They presented with transient ischemic attack even after bilateral superficial temporal artery–middle cerebral artery bypass, and magnetic resonance angiography (MRA) showed that PCA stenosis had worsened. Indirect revascularization with multiple burr holes using Benz-marked skin incisions was performed. After surgery, the symptoms improved without perioperative complications, and cerebral angiography showed collateral circulation via the burr hole. Indirect revascularization for MMD is often combined with direct revascularization, and there are only a few reports on the use of multiple burr hole surgery alone. In addition, there are few reports of posterior circulation, despite the emphasis on the importance of PCA involvement in MMD. Indirect revascularization with multiple burr holes alone can be performed in multiple areas and applied to patients who cannot undergo direct revascularization using the occipital artery. The procedure is simple and less invasive than traditional direct revascularization procedures. Therefore, it can be effective, especially in pediatric cases of MMD with PCA involvement.     

Moyamoya Syndrome in a Child with Noonan Syndrome

Previous studies have rarely described the association between Noonan syndrome and moyamoya syndrome. Although most affected children with moyamoya exhibit ischemic symptoms, headache is also a frequent symptom. We report the case of a 9-year-old girl with Noonan syndrome and moyamoya syndrome that manifested as recurrent headaches without history of transient ischemic attack. Brain magnetic resonance imaging and magnetic resonance angiography revealed bilateral moyamoya syndrome and mild ventriculomegaly with focal encephalomalacia at right peritrigonal region. Disruption of vascular development during the prenatal stage has been proposed as the cause of cerebrovascular disease in Noonan syndrome. However, genetic factors may also contribute.     

成人烟雾病的临床特点及影像分析

目的　研究成人型烟雾病的临床特点、影像表现及其两者的关系。方法　 6 1例经常规血管造影 (包括 DSA)和 MRA确诊的成人型烟雾病患者 (>16岁 )。分析其临床表现及血管造影结果。结果　主要临床表现为突发剧烈头痛、意识障碍 ,伴呕吐、失语及一侧肢体瘫痪。在 CT和 MRI上 ,脑出血 4 1例 (6 7.2 % ) ,脑梗死 15例(2 4 .6 % ) ,脑萎缩 2例 ,无异常表现 3例。在 DSA和 MRA上 ,双侧病变者 32例 ,单侧病变 2 9例 ,5 1例可见到烟雾状异常血管网 ,尤其在出血型患者中。后交通动脉增粗 38支、眼动脉增粗 31支及软脑膜吻合支增多 4 4侧。 5例大面积脑梗死侧支循环很差或缺乏。结论　成人型烟雾病以脑出血多见 ,在发病率和血管造影上和儿童无明显差异。治疗应首选血管重建手术。 DSA为诊断金标准 ,为术前必要检查 ;MRA无创伤性 ,为本病筛选、诊断、随访的首选方法。     

Abnormalities of cerebral arteries are frequent in patients with late-onset Pompe disease

Cerebral aneurysms and arteropathies causing severe cerebrovascular events have been reported as rare complications in patients with late-onset Pompe disease. We investigated the frequency of cerebrovascular anomalies in six patients with late-onset Pompe disease followed at our institution. Clinical data collection and magnetic resonance angiography were performed as part of routine annual examinations. Four out of six patients had brain vascular anomalies including dolichoectasia of the basilar artery and ectasia of internal carotids. These patients also complained of gastrointestinal symptoms (chronic constipation and gastrointestinal reflux). Two patients had clinical signs related to the arteriopathy, including partial paralysis of the third cranial nerve and transient ischemic attacks. At 1 year follow-up, enzyme replacement therapy did not modify the size of cerebral vessels, but patients reported a marked improvement of intestinal symptoms. In conclusion, neurologists should be aware that intracranial artery abnormalities are not infrequent in patients with late-onset Pompe disease, and they should be specifically investigated in the presence of unexplained CNS symptoms.     

Angiographic examination of spontaneous putaminal hemorrhage

BACKGROUND: This study was carried out to examine indications for angiographic evaluation in the case of spontaneous putaminal hemorrhage. METHODS: Angiographic examinations were performed on 62 consecutive adults with spontaneous putaminal hemorrhage without remarkable subarachnoid hemorrhage. Most patients were evaluated using conventional catheter angiography except for hypertensive patients over the age of 60 who underwent a magnetic resonance angiogram or a computed tomography angiogram. Computed tomography and clinical factors such as age and hypertension were correlated with the angiographic findings. RESULTS: Nine of the 62 patients (15%) exhibited angiographic abnormalities, including middle cerebral artery aneurysm (n = 1), arteriovenous malformations (n = 3), moyamoya disease (n = 2), and the moyamoya phenomenon associated with a proximal middle cerebral artery occlusion (n = 3). The angiographic yield was significantly higher (1) among patients at or below the median age of 55 compared with those above (9/33, 27%, versus 0/29, 0%; p < 0.01), and (2) among patients without hypertension compared with those with hypertension (5/9, 56%, versus 4/53, 8%; p < 0.01). CONCLUSIONS: Although our data could be biased due to the patient selection procedure, they suggest that angiographic evaluation is highly useful for spontaneous putaminal hemorrhage regardless of volume and shape of the hematomas, excepting hypertensive patients over 55 years of age.     

Efficacy of the revascularization surgery for adult-onset moyamoya disease with the progression of cerebrovascular lesions

Object

In moyamoya disease, despite its progressive nature of the occlusive lesions in pediatric patients, the prevalence of the progression in adult patients is undetermined. Furthermore, the optimal timing of the revascularization surgery for progressive cases is controversial. To address these issues, we retrospectively investigate four cases with the adult-onset moyamoya disease manifesting as progression before revascularization surgery.

Methods

From March 2004 to May 2007, 49 patients with adult-onset moyamoya disease aged from 19 to 62 years old (mean 40.5) underwent superficial temporal artery–middle cerebral artery (STA–MCA) anastomosis on 63 hemispheres. All patients were strictly followed up by magnetic resonance (MR) imaging/angiography postoperatively. Twenty-seven hemispheres of 15 adult patients without surgery were also followed up at outpatient service during the same period. If the patients manifest as the progression of the steno-occlusive lesion on the hemisphere without surgery, they undergo revascularization surgery after the confirmation of hemodynamic compromise.

Results

During this period, 47 hemispheres including those of outpatient cases were conservatively followed up after initial diagnosis. Among them, six hemispheres (12.8%) of four patients had been proven to show apparent progression of steno-occlusive lesion and were subjected to revascularization surgery. Postoperative courses were uneventful in all four cases, and no patient suffered cerebrovascular event on the operated hemisphere after surgery.

Conclusion

Adult-onset moyamoya disease, either bilateral or unilateral, has a substantial risk for progression, and careful follow-up is necessary for asymptomatic hemisphere. Once the patient manifests as the progression of cerebrovascular occlusive lesions or ischemic symptoms, we recommend revascularization surgery after the confirmation of the hemodynamic compromise.     

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.