Experiencia y resultados de las técnicas de revascularización en patología cerebral isquémica: enfermedad de moyamoya y oclusión carotídea

Introduction

Cerebral revascularization techniques are an indispensable tool in the current armamentarium of vascular neurosurgeons. We present revascularization surgery experience and results in both moyamoya disease and occlusive cerebral ischaemia.

Ischemic complications occurring in the contralateral hemisphere after surgical treatment of adults with moyamoya disease

Objective

Direct revascularization surgery is regarded as the most effective method of treatment of adults with moyamoya disease. These patients, however, have a higher risk of perioperative ischemic complications than do patients with atherosclerotic stroke, and are at risk for ischemic complications in the hemisphere contralateral to the one operated on. We investigated the incidence and risk factors for ischemic stroke in the contralateral hemisphere after surgical treatment of adults with moyamoya disease.

Methods

We retrospectively reviewed the medical records and results of neuroimaging studies on 79 hemispheres of 73 consecutive patients with adult moyamoya disease (mean±SD age, 37.96±11.27 years; range, 18-62 years) who underwent direct bypass surgery over 6 years.

Results

Ischemic complications occurred in 4 of 79 (5.1%) contralateral hemispheres, one with Suzuki stage 3 and three with Suzuki stage 4. Three patients showed posterior cerebral artery (PCA) involvement by moyamoya vessels. Advanced stage of moyamoya disease (Suzuki stages 4/5/6; p=0.001), PCA involvement (p=0.001) and postoperative hypotension (mean arterial blood pressure <80% of preoperative mean arterial blood pressure) on the first (p<0.0001) and second (p=0.003) days after surgery were significantly correlated with postoperative contralateral ischemic complications.

Conclusion

In patients with advanced moyamoya disease and involvement of the PCA, intentional hypotension can result in ischemic stroke in the hemisphere contralateral to the one operated on. Careful control of perioperative blood pressure is crucial for good surgical results.     

A genome-wide study of moyamoya-type cerebrovascular disease in the korean population

Objective

Structural genetic variation, including copy-number variation (CNV), constitutes a substantial fraction of total genetic variability, and the importance of structural variants in modulating susceptibility is increasingly being recognized. CNV can change biological function and contribute to pathophysiological conditions of human disease. Its relationship with common, complex human disease in particular is not fully understood. Here, we searched the human genome to identify copy number variants that predispose to moya-moya type cerebrovascular disease.

Methods

We retrospectively analyzed patients who had unilateral or bilateral steno-occlusive lesions at the cerebral artery from March, 2007, to September, 2009. For the 20 subjects, including patients with moyamoya type pathologies and three normal healthy controls, we divided the subjects into 4 groups : typical moyamoya (n=6), unilateral moyamoya (n=9), progression unilateral to typical moyamoya (n=2) and non-moyamoya (n=3). Fragmented DNA was hybridized on Human610Quad v1.0 DNA analysis BeadChips (Illumina). Data analysis was performed with GenomeStudio v2009.1, Genotyping 1.1.9, cnvPartition_v2.3.4 software. Overall call rates were more than 99.8%.

Results

In total, 1258 CNVs were identified across the whole genome. The average number of CNV was 45.55 per subject (CNV region was 45.4). The gain/loss of CNV was 52/249, having 4.7 fold higher frequencies in loss calls. The total CNV size was 904,657,868, and average size was 993,038. The largest portion of CNVs (613 calls) were 1M-10M in length. Interestingly, significant association between unilateral moyamoya disease (MMD) and progression of unilateral to typical moyamoya was observed.

Conclusion

Significant association between unilateral MMD and progression of unilateral to typical moyamoya was observed. The finding was confirmed again with clustering analysis. These data demonstrate that certain CNV associate with moyamoya-type cerebrovascular disease.     

Analysis of ischemic cerebral lesions using 3.0-T diffusion-weighted imaging and magnetic resonance angiography after revascularization surgery for ischemic disease

Background

Cerebral revascularization surgery (CRS) is increasingly recognized as an important component in the treatment of complex cerebral vascular disease and tumors. CRS requires that the incidence of perioperative neurological complications should be minimized, because CRS for ischemic disease is often not the goal of treatment, but rather a prophylactic surgery. CRS carries the risk of focal postoperative neurological deficits. Little has been established concerning mechanisms of post-CRS ischemia. We used 3.0-T diffusion-weighted magnetic resonance imaging (DWI) and magnetic resonance angiography (MRA) to analyze the incidence and mechanism of ischemic lesions.

Methods

We studied the anterior circulation territory after 20 CRS procedures involving 33 vascular anastomosis procedures (13 double anastomoses and 7 single anastomoses) in 12 men and 8 women between June 2007 and October 2011. The operations included single or double superficial temporal artery–middle cerebral artery (STA–MCA) anastomosis to treat internal carotid artery/MCA occlusions or severe MCA stenosis. A combined STA–MCA anastomosis and indirect bypass were performed for moyamoya disease. Postoperative DWI and MRA were obtained in all patients between 24 and 96 h after surgery to detect thromboembolism, hypoperfusion, or procedural ischemic complications and vasospasms of the donor STA.

Results

Follow-up DWI and MRA were carried out 1.8 ± 0.6 days after CRS (range, 1–4 days). Temporary occlusion time for anastomoses averaged 18.9 min (range, 16–32 min). Asymptomatic new hyperintensities occurred in the ipsilateral hemisphere of 2 patients on postoperative DWI (10% patients/6.0% anastomoses), and 1 moyamoya patient (5.0% patients/3.0% anastomoses) developed a symptomatic hyperintensity in the ipsilateral occipital lobe in response to the operation. Two abnormal small (<5 mm) cortical DWI lesions were caused by sacrifices of a small branch of the recipient MCA.

Conclusion

This study is the first postoperative 3.0-T DWI study of CRS and related clinical events. The incidence of symptomatic postoperative DWI abnormalities was restricted to 1 moyamoya patient representing 5.0% of total patients and 3.0% anastomoses. Although some postoperative DWI abnormalities occurred, CRS was found to be safe with a low risk of symptomatic ischemia.     

Coexistence of moyamoya and Graves’ diseases: The clinical characteristics and treatment effects of 21 Chinese patients

Objective

To investigate the clinical features, pathogenesis and treatment effect of patients who had moyamoya disease (MMD) concurrent with Graves’ disease (GD).

Patients and methods

Retrospective analysis of the examination and treatment results of the MMD patients with GD from January 2003 to April 2012 treated by our teamwork.

Results

There were 1493 MMD patients included in this retrospective study. Among them, there were 16 female and 5 male MMD patients coexisted with Graves’ diseases, and 15 of them were adult patients. Bilateral and unilateral lesions were observed in 18 and 3 cases, respectively. 19 patients show cerebral infarction. 18 patients manifested MMD symptoms when they were in a hyperthyroid state. 17 patients received encephaloduroarteriosynangiosis (EDAS) surgery after antithyroid treatment. There were 20 patients completed 2 and 77 months follow-up. 17 patients had good recovery and the other 3 patients had mild or moderate disability. Post-operative DSA achieved in 8 patients examinations showed good collateral circulation to the brain from superficial temporal artery (STA).

Conclusion

Moyamoya disease complicating GD is mostly occurred in adult female patients. Their clinical symptoms are mainly ischemic lesions and usually occur during the state of hyperthyroidism. The pathogenesis of moyamoya disease complicating GD may be associated with a variety of genetic and immune factors. Surgical treatment can establish effective collateral circulation and reduce the risk of recurrence of stroke.     

The Usefulness of the Ivy Sign on Fluid-Attenuated Intensity Recovery Images in Improved Brain Hemodynamic Changes after Superficial Temporal Artery-Middle Cerebral Artery Anastomosis in Adult Patients with Moyamoya Disease

Objective

MR perfusion and single photon emission computerized tomography (SPECT) are well known imaging studies to evaluate hemodynamic change between prior to and following superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis in moyamoya disease. But their side effects and invasiveness make discomfort to patients. We evaluated the ivy sign on MR fluid attenuated inversion recovery (FLAIR) images in adult patients with moyamoya disease and compared it with result of SPECT and MR perfusion images.

Methods

We enrolled twelve patients (thirteen cases) who were diagnosed with moyamoya disease and underwent STA-MCA anastomosis at our medical institution during a period ranging from September of 2010 to December of 2012. The presence of the ivy sign on MR FLAIR images was classified as Negative (0), Minimal (1), and Positive (2). Regions were classified into four territories: the anterior cerebral artery (ACA), the anterior MCA, the posterior MCA and the posterior cerebral artery.

Results

Ivy signs on preoperative and postoperative MR FLAIR were improved (8 and 4 in the ACA regions, 13 and 4 in the anterior MCA regions and 19 and 9 in the posterior MCA regions). Like this result, the cerebrovascular reserve (CVR) on SPECT was significantly increased in the sum of CVR in same regions after STA-MCA anastomosis.

Conclusion

After STA-MCA anastomosis, ivy signs were decreased in the cerebral hemisphere. As compared with conventional diagnostic modalities such as SPECT and MR perfusion images, the ivy sign on MR FLAIR is considered as a useful indicator in detecting brain hemodynamic changes between preoperatively and postoperatively in adult moyamoya patients.     

Regional cerebral perfusion and ischemic status after standard superficial temporal artery–middle cerebral artery (STA-MCA) bypass surgery in ischemic cerebrovascular disease

Background and purpose  

Standard superficial temporal artery–middle cerebral artery (STA-MCA) bypass surgery is an effective treatment for ischemic cerebrovascular diseases, including moyamoya disease and occlusive cerebrovascular disease. Our purpose in this study was to evaluate the ischemic status based on the imaging modality of computed tomographic perfusion (CTP) before and after STA-MCA bypass in patients with moyamoya disease and occlusive cerebrovascular disease.     

Comparison of Effects between Clopidogrel and Cilostazol on Cerebral Perfusion in Nonsurgical Adult Patients with Symptomatically Ischemic Moyamoya Disease: Subanalysis of a Prospective Cohort

Background and purpose

Adult patients with symptomatically ischemic moyamoya disease (MMD) initially undergo medical treatment alone including antiplatelet drugs when symptomatic cerebral hemispheres do not exhibit hemodynamic compromise. The purpose of the present study subanalyzing the same patient cohort used in a previous study was to determine which antiplatelet drug, clopidogrel or cilostazol, provides better improvement of cerebral perfusion in such patients.

Systemic arterial inflammation, measured with 18FDG-PET, is common amongst subjects with both recent and prior cerebrovascular disease

Objective

To compare systemic arterial inflammation in subjects with recent ischaemic stroke or TIA and controls with prior cerebrovascular disease.

Methods

Systemic arterial inflammation was prospectively measured by ¹⁸F-fluorodeoxygluose positron emission tomography in 11 cases with recent ischaemic stroke or TIA, and 11 sex matched controls with prior cerebrovascular disease.

Results

Hot spots (both carotid and non-carotid) of localised ¹⁸FDG uptake were found in more than half of all patients with either recent (n = 6) or prior (n = 8) cerebrovascular disease. There was no significant difference in the total number of hotspots, or hotspots at specific sites, in cases compared with controls. Mean standard uptake values (SUV) were similar in the carotid arteries and aorta of cases and controls, and showed a trend toward higher values in the femoral arteries of the controls (median 1.8; IQR 1.6–2.2) compared to cases (median 1.5; IQR 1.4–1.7).

Conclusion

Arterial inflammation was common, and appeared similar, in patients with recent stroke/TIA, and controls with stroke/TIA more than two years previously.     

Aspirin resistance determined with PFA-100 does not predict new thrombotic events in patients with stable ischemic cerebrovascular disease

Objective

Evidence is growing that some patients are not responsive to the antithrombotic action of aspirin. We prospectively evaluated the ability of aspirin resistance status, determined by PFA-100, to predict new thrombotic events in patients with stable ischemic cerebrovascular disease.

Methods

We studied 129 consecutive patients with stroke, transient ischemic attack (TIA) or vascular cognitive impairment. We assessed relationships between aspirin resistance, risk factors for ischemic cerebrovascular disease, and occurrence of new thrombotic events (composite of stroke, TIA, myocardial infarction, and cardiovascular death).

Results

Aspirin resistance, found in 26 (20.1%) cases, was unrelated to any of the examined vascular risk factors. During mean follow-up of 56 months, new thrombotic events occurred in 19 patients (14.7%), four with aspirin resistance (15.4%) and 15 (14.6%) without aspirin resistance (p = 1.00).

Conclusion

Aspirin resistance determined by PFA-100 does not predict new thrombotic events in patients with stable ischemic cerebrovascular disease.     

Varied presentations of moyamoya disease in a tertiary care hospital of north-east India

Introduction:

Moyamoya disease is a chronic progressive cerebrovascular disorder, characterized by stenosis or occlusion of bilateral internal carotid arteries (ICAs), anterior cerebral arteries (ACAs) and middle cerebral arteries (MCAs), accompanied by a collateral network of vessels formed at the base of the brain. Ischemia and intracranial hemorrhage are the common typical manifestations. However moyamoya disease has been associated with atypical presentations like headache, seizures and involuntary movements. Although frequently reported from Asian countries like Japan, China and Korea, only few studies reported on clinical manifestations of moyamoya disease from India.

Objectives:

To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India.

Material and Methods:

Relevant investigations were done to rule out other causes of moyamoya syndrome.

Results:

We report 6 cases of moyamoya disease with varied presentations from a tertiary care referral government hospital. Case 1, 2 and 6 presented with alternating hemiparesis. Case 3 had amaurosis fugax. Case 4 had history suggestive of ischemic stroke and presented with hemichorea. Case 4 had focal seizure as the only manifestation. Cases 4 and 5 notably had stenosis of posterior cerebral artery (PCA) in addition to stenosis of bilateral ICAs, ACAs and MCAs.

Conclusion:

Owing to its low incidence in India, moyamoya disease is easily overlooked as a possible diagnosis. However, because of its progressive nature, it is imperative to diagnose this disease early and offer surgical treatment to the patients.     

Prevalence of cerebrovascular lesions in patients with Lewy body dementia: A neuropathological study

Background

The co-existence of vascular pathology in patients with Lewy body dementia (LBD) is still a matter of debate. This study analyses the prevalence and the severity of cerebrovascular lesions in post-mortem brains of patients with LBD.

Patients and methods

Twenty brains of demented patients with autopsy-proven Lewy body disease were compared to 14 brains of age-matched controls.

Results

Associated Alzheimer disease (AD) features, stages I–IV, were present in 70% of the LBD brains and in 7% of the controls (P < 0.001). Cerebral amyloid angiopathy (CAA) was only present in 30% and lipohyalinosis in 10%. A semi-quantitative analysis, performed on a coronal section of a whole cerebral hemisphere and on a horizontal section through the pons and the cerebellum, revealed significantly more mini-bleeds in the LBD brains (P = 0.007), predominantly in the cerebral cortex (P = 0.03). Other cerebrovascular lesions were only rarely observed. Comparison of the LDB brains, with and without moderate AD features and CAA, showed no difference in the severity of the cerebrovascular lesions including mini-bleeds.

Conclusions

The prevalence of mini-bleeds in LBD brains appears to be independent from the co-existence of moderate AD pathology and CAA. It is more probably due to disturbances of the blood–brain barrier, related to the neurodegenerative process itself.     

Early superficial temporal artery to middle cerebral artery bypass in acute ischemic stroke

Objective

To evaluate the effects and safety of superficial temporal artery–middle cerebral artery (STA–MCA) anastomosis in the early stage after an acute ischemic event and the improvement of present symptoms in patients with intracranial atherosclerotic occlusive disease with stroke/stroke in progress.

Methods

From 2006 to 2010, 20 patients (15 males and five females) with atherosclerotic cerebrovascular disease were treated with an STA–MCA bypass. All of the patients presented with an acute ischemic stroke or stroke in progress despite maximal medical treatment. The patients underwent an STA–MCA bypass within 7 days from symptom onset. The clinical outcome and hemodynamic study of the 20 patients were preoperatively and postoperatively investigated. A pooled analysis was performed, and the results were compared with those obtained from other delayed STA–MCA bypass studies.

Results

Among the 20 patients who underwent an early STA–MCA bypass, fourteen (70%) patients achieved a good functional outcome (mRS 0, n = 3; mRS 1, n = 9; mRS 2, n = 2). Prior to surgery, the mean basal regional cerebral blood flow (rCBF) and cerebrovascular reserve capacity (CVR) in the symptomatic hemisphere were 37.3 ± 4.3 ml/100 g/min and −1.68 ± 2.9%. The mean basal rCBF and CVR had significantly increased postoperatively, and no reperfusion-induced hemorrhage had occurred. In the pooled analysis, no significant differences were observed in the clinical outcome (P = 0.328) or in the incidence of postoperative complications (P = 0.516) between patients who underwent an early STA–MCA bypass and in patients who underwent a delayed STA–MCA bypass in previous studies.

Conclusions

In this study, which consisted of 20 carefully selected patients with acute ischemic stroke, an early STA–MCA bypass was safely and effectively performed, and in some cases, an early STA–MCA bypass resulted in rapid neurological improvement. An early STA–MCA bypass was beneficial in select patients who had acute ischemic stroke with imaging evidence of a small infarction.     

Perioperative complications of superficial temporal artery to middle cerebral artery bypass for the treatment of complex middle cerebral artery aneurysms

Object

Only a few studies have reported the risk of ischemic complications occurring when superficial temporal artery (STA) to middle cerebral artery (MCA) anastomosis is performed during surgery for complex MCA aneurysms.

Subjects and methods

This is a retrospective study of 10 patients (age 52–73) with MCA aneurysms treated with revascularization surgery. The aneurysms were 10–50 mm in size (mean: 21 mm). We studied the causes and frequency of ischemic complications by analyzing postoperative magnetic resonance imaging.

Results

Postoperative diffusion-imaging confirmed ischemic complications in six of the 10 patients (in two of the five ruptured aneurysms and in four of the five unruptured). The ischemic complications that observed were infarction of the lenticulostriate artery territory in three cases, cortical infarction in two cases, and cerebral infarction that was likely to be due to cerebral vasospasm in one case. In one case, both cortical infarction and infarction of the lenticulostriate artery territory were observed. The Glasgow Outcome Scale (GOS) scores at the time of discharge indicated good recovery (GR) and moderate disability (MD) in seven cases, severe disability (SD) in two cases, and death (D) in one case.

Conclusions

The present study suggests the possibility that STA–MCA anastamosis in surgeries for MCA aneurysms can be performed with comparatively better safety. However, the temporary occlusion time with this surgery is longer than that with a temporary clipping for aneurysmal surgery; thus, we believe that adequate countermeasures are required to prevent ischemic complications.     

Preliminary Study of Neurocognitive Dysfunction in Adult Moyamoya Disease and Improvement after Superficial Temporal Artery-Middle Cerebral Artery Bypass

Objective

Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease of unknown etiology. In addition, the neurocognitive impairment of adults with MMD is infrequently reported and, to date, has not been well described. We attempted to determine both the neurocognitive profile of adult moyamoya disease and whether a superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis can improve the neurocognitive impairment in exhibiting hemodynamic disturbance without stroke.

Methods

From September 2010 through November 2012, 12 patients with angiographically diagnosed MMD underwent STA-MCA anastomosis for hemodynamic impairment. Patients with hypoperfusion and impaired cerebrovascular reserve (CVR) capacity but without evidence of ischemic stroke underwent a cognitive function test, the Seoul Neuropsychological Screening Battery (SNSB). Five patients agreed to undergo a follow-up SNSB test. Data from preoperative and postoperative neurocognitive function tests were compared and analyzed.

Results

Five of 12 patients were enrolled. The median age was 45 years (range, 24-55 years). A comparison of preoperative to postoperative status of SNSB, memory domain, especially delayed recall showed significant improvement. Although most of the domains showed improvement after surgery, the results were not statistically significant.

Conclusion

In our preliminary study, large proportions of adult patients with MMD demonstrate disruption of cognitive function. This suggests the possibility of chronic hypoperfusion as a primary cause of the neurocognitive impairment. When preoperative and postoperative status of cognitive function was compared, memory domain showed remarkable improvement. Although further study is needed, neurocognitive impairment may be an indication for earlier intervention with reperfusion procedures that can improve cognitive function.     

Reduced percentage of neurocysticercosis cases among patients with late-onset epilepsy in the new millennium

Objective

To determine if the number of neurocysticercosis cases among patients with late-onset epilepsy has decreased over the past two decades.

Design

Retrospective cohort study of 431 consecutive patients with recurrent seizures starting after the age of 20 years evaluated at our Institution from 1990 to 2009.

Methods

Patients were classified according to the year in which they were first seen. Group I included 129 patients evaluated between 1990 and 1994, Group II included 108 patients evaluated between 1995 and 1999, Group III included 106 patients evaluated between 2000 and 2004, and Group IV included 88 patients evaluated between 2005 and 2009. We correlated the percentage of persons with cryptogenic and symptomatic epilepsy between the groups to determine if there was any change in the causes of late-onset epilepsy.

Results

One hundred seventy-one patients had cryptogenic and 260 had symptomatic epilepsy. Common causes of symptomatic epilepsy were neurocysticercosis in 120 cases, cerebrovascular disease in 68, and brain tumors in 40. We found a reduction in the number of patients with symptomatic epilepsy (p = 0.0007) as well as a reduction in the number of neurocysticercosis cases (p = 0.0004) over the study years. There was a reduction in the weight of neurocysticercosis as an etiological factor for symptomatic late-onset epilepsy related to a drop in the number of patients with this condition evaluated between 2005 and 2009 (p = 0.0045).

Conclusion

The number of neurocysticercosis cases among patients with late-onset epilepsy has changed over the years. This parasitic disease is no longer the most common cause of symptomatic late-onset epilepsy in our population.     

Análisis del movimiento de la extremidad superior hemiparética en pacientes con accidente cerebrovascular: estudio piloto

Introduction

As a result of neurophysiological injury, stroke patients have mobility limitations, mainly on the side of the body contralateral to the lesioned hemisphere. The purpose of this study is to quantify motor compensation strategies in stroke patients during the activity of drinking water from a glass.

Material and methods

Four male patient with cerebrovascular disease and four right-handed, healthy male control subjects. The motion analysis was conducted using the Vicon Motion System^® and surface electromyography equipment ZeroWire Aurion^®. We analysed elbow, shoulder and trunk joint movements and performed a qualitative analysis of the sequence of muscle activation.

Results

Trunk, shoulder and elbow movements measured in the stroke patient along the sagittal plane decreased during the drinking from a glass activity, while the movements in the shoulder in the coronal plane and trunk increased. As for the sequence of muscle activation, anterior, middle and posterior deltoid all contracted in the patient group during the task, while the upper trapezius activation remained throughout the activity.

Conclusions

Quantitative analysis of movement provides quantitative information on compensation strategies used by stroke patients, and is therefore, clinically relevant.     

“STA-MCA bypass with encephalo-duro-myo-synangiosis combined with bifrontal encephalo-duro-periosteal-synangiosis” as a one-staged revascularization strategy for pediatric moyamoya vasculopathy

Purpose

Moyamoya vasculopathy progressively compromises cerebral blood flow resulting in chronic hypoperfusion. The middle cerebral artery (MCA) territory and the bifrontal areas are the regions most frequently affected. Although most techniques aim to only revascularize the MCA territory, augmentation of blood flow of the bifrontal areas is of importance in the pediatric moyamoya population since these regions play an important role in cognition, intellectual development, and in lower extremity and sphincter function. We recently described a one-staged surgical procedure combining revascularization of three regions, the MCA territory unilaterally and the frontal areas bilaterally. The purpose of this article is to report our surgical experience in eight children and to emphasize the rational for bifrontal revascularization.

Methods

We report a case series consisting of eight children where the following surgical strategy was applied: (1) a direct superficial temporal artery-to-middle cerebral artery (STA-MCA) bypass with encephalo-duro-myo-synangiosis (EDMS) for unilateral MCA revascularization; in combination with (2) a bifrontal encephalo-duro-periosteal-synangiosis (EDPS) for bifrontal revascularization. Patients’ characteristics and 30-day follow-up data are reported.

Results

The patient group consisted of six girls and two boys (mean age 10.0, range 4.2–17.5 years): six children presented with moyamoya disease, two with moyamoya syndrome. We performed a one-staged revascularization of one MCA territory and both frontal areas in all patients. No significant complications occurred. Two patients experienced postoperative focal seizures, successfully treated with anti-epileptic medication.

Conclusions

The single-staged STA-MCA bypass with EDMS combined with bifrontal EDPS allowed revascularization of three regions (the MCA territory unilaterally and the frontal areas bilaterally) and may serve as an alternative and safe treatment option for pediatric moyamoya patients.

     

Trends of Antiplatelet Therapy for the Management of Moyamoya Disease in Japan: Results of a Nationwide Survey

Background

The efficacy and safety of antiplatelet drugs in the treatment of moyamoya disease remain unclear. This study reports results of a nationwide survey conducted in 2016 on the trends of antiplatelet therapy for moyamoya disease in Japan.

Multiple encephalogaleoperiosteal synangiosis for bilateral carotid artery stenosis in a 13-year-old girl: a case report

Introduction

Encephalogaleoperiosteal synangiosis (EGS) has been widely used to treat children with moyamoya disease (MMD). We present the first case of successful multiple EGS in a patient with brain ischemic disease who presented with different cerebrovascular findings from MMD.

Methods

A 13-year-old girl had an increased frequency of transient ischemic attacks that affected her right extremities. Digital subtraction angiography showed tapering of the internal carotid artery (ICA). The anterior cerebral artery (ACA) and middle cerebral artery (MCA) were visible on vertebral angiogram, but not on carotid angiogram. The intact circle of Willis and lack of hypervascularity of the lenticulostriate arteries were observed. Decreased regional cerebral blood flow (CBF) in the bilateral ACA and MCA territories quantified by ¹²³I-N-isopropyl-p-iodoamphetamine-single photon emission computed tomography indicated the need for extracranial-intracranial bypass surgery. Multiple EGS procedures were performed instead of direct anastomosis, which is the standard procedure for intracranial ICA stenosis, because the space for the craniotomy was limited by transdural anastomosis.

Results

Despite the fact that the diagnosis of MMD was questionable, the hemispheres were well vascularized, and the neurology and CBF improved postoperatively.

Conclusion

The preserved circle of Willis and lack of moyamoya vessels were inconsistent with the features of MMD. However, childhood onset, bilateralness, chronic intracranial ICA stenosis, and transdural anastomosis indicated the same underling pathogenicity as MMD. It is hypothesized that ICA stenosis occurred immediately proximal to the posterior communicating artery in this case. This would have produced the atypical finding of the remaining circle of Willis without growth of the basal moyamoya vessels.