Peek through the smoke: a report of moyamoya disease in a 32-year-old female patient presenting with ischaemic stroke. Omer Siddiq,Zbyszynska Renata,Kirthivasan Ramanathan BMJ case reports Moyamoya disease is a vasculopathy causing chronic progressive stenosis and occlusion of the large arteries of the circle of Willis that could lead to brain ischaemia. The condition may also present with haemorrhagic strokes. This is a case report of moyamoya disease in a 32-year-old woman presenting with ischaemic stroke. The report describes her inpatient stay and investigations and findings.The report reviews the main aspects of moyamoya disease definition, epidemiology, clinical features, diagnosis, classification and treatment. This case is interesting because her first presentation occurred after 3 months of her second delivery. Whether the different physiological stresses of pregnancy, child birth and puerperium have had some effect in accelerating the pathogenesis of her moyamoya disease remains unknown. . 10.1136/bcr-2017-221685
    Moyamoya disease. Kondo Takeshi CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne 10.1503/cmaj.180681
    Multiple encephalogaleoperiosteal synangiosis for bilateral carotid artery stenosis in a 13-year-old girl: a case report. Inamura Akinori,Nomura Sadahiro,Sadahiro Hirokazu,Oku Takayuki,Ishihara Hideyuki,Suzuki Michiyasu Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 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 (123)I-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. 10.1007/s00381-015-2918-9
    A Case of Delayed Diagnosis of Moyamoya Disease After Recurrent Strokes. Mohammadi Oranus,Krieger David,Butt Ifrah,Danckers Mauricio Cureus We present a case of a 58-year-old man with delayed diagnosed moyamoya disease who underwent encephaloduroarteriosynangiosis (EDAS) procedure. This patient with a history of three strokes presented to our facility with new left facial droop. Neurological examination revealed left facial droop and hemiparesis. Brain magnetic resonance imaging (MRI) described right frontal lobe acute ischemia. Head computed tomography (CT) angiography revealed bilateral supraclinoid internal carotid artery (ICA) occlusions. Cerebral angiography demonstrated diffuse intracranial vascular irregularity with stenosis, more above the bilateral supraclinoid ICAs and the right middle cerebral artery (MCA) suggestive of moyamoya disease. Due to the lack of MCA patency, he underwent EDAS. Superficial temporal artery (STA) was dissected inferiorly and the posterior branch was bipolared, then STA was movable. A bur hole made at the superior and inferior portion along the STA. Dura was opened, and STA was brought on top of the pia. His facial droop gradually improved after that. Nine months later, no new strokes reported. Moyamoya disease is a rare neurovascular disorder characterized by narrowing and occlusion of the ICA branches. Its symptoms include recurrent ischemic/hemorrhagic strokes. Incidence in Hispanics has not been studied. The gap between the first manifestations and disease progression is one to eight years. Its diagnosis is often delayed. Our patient had recurrent strokes for five years. Despite therapy with antiplatelets, new ischemic stroke brought him to our institution. Rate of recurrent strokes despite antiplatelets was reported 10.3% per year. Brain CTs and MRIs had failed to detect strokes' etiology. Catheter-directed angiography is the gold standard test for diagnosis of moyamoya disease. Antiplatelet alone is ineffective and surgery is the effective method to prevent further strokes, although there are no studies in adults regarding the efficacy of indirect revascularization. In direct revascularization, usually STA anastomoses to MCA. Indirect method works through the development of leptomeningeal collaterals. Postoperative complications are infarction and hyperperfusion syndrome. Seong-eun et al. proposed that modified EDAS is simpler with less complications in comparison with direct revascularization. Some other studies showed higher chance of stroke in indirect method versus direct technique. In conclusion, it is important to consider moyamoya disease as a differential diagnosis in patients with recurrent strokes. 10.7759/cureus.6446
    Postpartum Seizure and Subarachnoid Haemorrhage Secondary to Moyamoya Disease. Varanasi L Chitra,Brown James,Athayde Neil Case reports in obstetrics and gynecology Postpartum seizures secondary to subarachnoid haemorrhage (SAH) are rare. The incidence of pregnancy-related SAH is increasing and is highest during the delivery and postpartum periods. While there have been cases in the literature of SAH occurring postpartum, very few are associated with Moyamoya disease. We present a rare case of a young woman diagnosed with Moyamoya disease following immediate postpartum seizures secondary to a SAH. She was medically managed and discharged without any neurological deficits. This case highlights how seizures and SAH may develop in the immediate postpartum period in an otherwise healthy young woman. 10.1155/2019/6132835
    Subacute Stroke in a Young Female: A Case of Moyamoya Syndrome Initially Anchoring with Anxiety. Chaughtai Saira,Chaughtai Zeeshan,Haider Muhammad Shaheryar,Ijaz Hasnan M,Elmedani Sarah,Bakr Mohamed,Hossain Mohammad A,Asif Arif Case reports in medicine Moyamoya disease is an arterial disorder causing stroke in a young patient. This is a chronic condition causing progressive cerebrovascular disease due to bilateral stenosis and occlusion of the arteries around the circle of Willis, with prominent arterial collateral circulation. It was first described in Japan and subsequently reported in other Asian countries, but infrequently found in the Western world. Interestingly, there may be racial differences in the presentation and subsequent prognostication of treatment of moyamoya. It is diagnosed with classic angiographic findings of stenosis or occlusion of the circle of Willis vessels. Here, we describe a 28-year-old Caucasian female who was initially diagnosed with anxiety when she presented with symptoms of impaired concentration and fatigue. After the development of remitting slurred speech and facial droop, magnetic resonance imaging and cerebral angiogram yielded the discovery of high-grade stenosis of the origin of the left middle cerebral artery with associated thrombosis in that area. She did well after getting surgery and rehabilitation. This demonstrates a unique presentation of prominent psychiatric symptoms initially thought to be anxiety and culminated in the finding of ischemic stroke in an adult patient with moyamoya. 10.1155/2019/7919568
    Individualized Perioperative Blood Pressure Management for Adult Moyamoya Disease: Experience from 186 Consecutive Procedures. Li Changwen,Zhang Nan,Yu Shaojie,Xu Yong,Yao Yang,Zeng Minghui,Li Dongxue,Xia Chengyu Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association BACKGROUND:In adult patients with moyamoya disease (MMD) underwent combined revascularization, cerebral infarction during the acute postoperative phase is common and can lead to neurological dysfunction after revascularization in MMD patients. The aim of this study was to share the experience of individualized perioperative blood pressure (BP) management for adult MMD patients in one single center. METHODS:We retrospectively reviewed 144 adult patients with MMD who underwent 186 procedures of combined revascularization at our institution from March 2013 to July 2019. Clinical features and outcomes were analyzed, in particular regarding cerebral infarction and hyperperfusion syndrome (HPS). All of the patients received individualized management perioperatively, especially about the blood pressure management according to the characteristics of moyamoya disease. RESULTS:Postoperative cerebral infarction and HPS within 14 days after revascularization were recorded. Cerebral infarction occurred in four (2.1%) procedures among four patients. No patients suffered from a malignant cerebral infarction and only one patient had permanent neurological deficits. The incidence of HPS was 10.8% and no one presented with intracranial hemorrhage. All of the symptoms were reversible without any brain parenchymal injury. CONCLUSIONS:Our findings suggest that we can decrease the incidence and extent of cerebral infarction in adult MMD patients following combined revascularization by individualized perioperative BP management. 10.1016/j.jstrokecerebrovasdis.2020.105413
    High variance of intraoperative blood pressure predicts early cerebral infarction after revascularization surgery in patients with Moyamoya disease. Li Jiaxi,Zhao Yahui,Zhao Meng,Cao Penghui,Liu Xingju,Ren Hao,Zhang Dong,Zhang Yan,Wang Rong,Zhao Jizong Neurosurgical review Few studies focused on the intraoperative blood pressure in Moyamoya disease (MMD) patients. We aimed to clarify whether or not it relates to early cerebral infarction after revascularization. We reviewed a retrospective cohort of Moyamoya disease from 2011 to 2018 in Beijing Tiantan Hospital, and patients with radiologically confirmed early postoperative infarction were included in the analysis. Controls were matched based on age, sex, and revascularization modality at a ratio of 1:5. Perioperative clinical factors and intraoperative blood pressure data were collected and analyzed. A total of 52 patients out of 1497 revascularization surgeries (3.5%) who experienced CT or MRI confirmed early postoperatively cerebral infarction, aged 38.46 ± 11.70; 26 were male (50.0%). Average real variability (ARV)-systolic blood pressure (SBP) (OR 3.29, p = 0.003), ARV-diastolic blood pressure (DBP) (OR 4.10, p = 0.005), ARV-mean arterial pressure (MAP) (OR 4.08, p = 0.004), and the maximum drops of DBP (OR 1.08, p = 0.003) and MAP (OR 1.06, p = 0.004) were associated with early postoperative infarction. In patients who experienced massive cerebral infarction, the maximum drops of DBP (OR 1.11, p = 0.004) and MAP (OR 1.11, p = 0.003) are independent risk factors, whereas ARVs of SBP (OR 3.90, p < 0.001), DBP (OR 4.69, p = 0.008), and MAP (OR 4.72, p = 0.003) are significantly associated with regional infarction. High variance of intraoperative blood pressure and drastic blood pressure decline are independent risk factors for postoperative infarction in MMD patients who underwent revascularization surgery. Maintaining stable intraoperative blood pressure is suggested to prevent early postoperative cerebral infarction in MMD patients. 10.1007/s10143-019-01118-z