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Moyamoya disease (MMD) is a rare, idiopathic, progressive, obstructive, vasculopathy affecting primarily the terminal portions of the intracerebral internal carotid arteries, typically at the base of the brain. It is more commonly seen in people of East Asian descent. The moyamoya phenomenon refers to the characteristic appearance of the tangle of fine blood vessels, also described as a puff of smoke. Moyamoya syndrome (MMS) refers to the constriction-induced chronic brain ischemia that is believed to cause overexpression of proangiogenic factors, creating a fragile network of collateral capillaries. MMS refers to the moyamoya phenomenon in the presence of other congenital or acquired disorders. Intracerebral hemorrhage is the leading cause of death for MMS patients. Overall, the prognosis is variable. Cardiac myxoma can cause embolization of tumor cells, plaques, and thrombus, and recurrent thromboembolism can lead to chronic brain ischemia, which can lead to the development of collaterals. There have been cases reported where the moyamoya phenomenon resolved following myxoma resection. Here, we present the case of a female who had intraventricular bleeding and was diagnosed with MMD. Eighteen months later, she presented with shortness of breath and was diagnosed with cardiac myxoma with multiple valvular regurgitations. The myxoma was surgically removed., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Konduru et al.)

Moyamoya syndrome, known as secondary moyamoya disease, is associated with various primary illnesses, such as brain tumor, meningitis, autoimmune disease, and thyrotoxicosis, and their relations are not clear. We report a rare case of moyamoya syndrome in a patient with Graves' disease. An 18-year-old woman was admitted to our hospital due to convulsions. She had symptoms of palpitations and fatiguability for half a year and transient numbness in her left upper extremity and dysarthria for a month. In physical findings, tachycardia and diffuse thyroid swelling were noted. A blood test revealed thyrotoxicosis and antithyroid antibody, and a diagnosis of Graves' disease was obtained. Brain magnetic resonance imaging (MRI) showed bilateral internal carotid artery occlusion. We finally diagnosed the patient with moyamoya syndrome caused by Graves' disease. Moyamoya disease or syndrome can cause symptoms like a stroke, sometimes requiring neurosurgical treatment. In our case, the therapy for Graves' disease resolved the symptoms. When diagnosing moyamoya disease, it is necessary to confirm whether there are any background diseases, such as Graves' disease., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Maezawa et al.)

Moyamoya disease is a cerebrovascular disease characterized by stenosis of large intracranial arteries and the development of smaller collateral vessels. Moyamoya may cause strokes and stroke-like symptoms in young patients. It has also been linked to autoimmune diseases and neuropsychiatric conditions. We present a case of moyamoya disease in a young patient with concomitant hyperaldosteronism, uncontrolled hypertension, and cocaine use disorder, along with features of antisocial personality disorder. This is a unique presentation of an underlying neurological disease causing psychiatric features exacerbated by cocaine use, and it describes a rare clinical presentation that physicians should consider in patients with moyamoya disease., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, DeRon et al.)

Moyamoya represents a rare, progressive cerebrovascular disease, characterized by a gradual stenosis of the intracranial internal carotid arteries, thereby increasing the risk of stroke. Down syndrome is known to be a predisposing factor for Moyamoya syndrome. This review discusses a distinctive case of a seven-year-old female with Down syndrome who manifested with Moyamoya syndrome, evident from acute stroke-like symptoms., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Brenner et al.)

Moyamoya disease (MMD) is a rare yet progressive cerebrovascular disorder caused by the constriction of arteries, which leads to the twisting and tangling of small arteries in the brain, ultimately causing blockages. Although moyamoya angiopathy (MMA) has been known for almost six decades, its pathophysiology remains unknown, posing challenges to timely diagnosis. Moyamoya syndrome (MMS) refers to the association of MMA with various diseases, including infections, tumors, arteriovenous malformations, radiation treatment, and hereditary disorders. On the other hand, MMD, an idiopathic form, is now more frequently linked to genetic abnormalities. MMS is more common in people of Asian descent, but we encountered and aim to discuss a rare case of a 32-year-old Caucasian from Colombia who was diagnosed with it. The patient initially presented with unexplained symptoms of stroke, prompting doctors to conduct additional imaging. Fortunately, this led to her timely diagnosis. The report discusses the challenges that healthcare professionals face in diagnosis when presented with such uncommon cases. Through this case report, we try to review the presentation, diagnosis, and treatment used for this patient with MMS. The limited information available about the disease, especially the demographic data in countries outside Asia, often leads to delayed diagnoses, emphasizing the need for further exploration. Timelier diagnosis and heightened research into the disease's presentation and risk factors could lead to improved outcomes. Our report also briefly discusses the effectiveness of the current treatment protocol for patients. Currently, the patient is undergoing rehabilitation and showing promising progress., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Singh et al.)

Moyamoya syndrome (MMS) is a progressive disease that can result in debilitating strokes. Surgical revascularization is the mainstay of treatment. Selection of the proper bypass technique depends on the vascular anatomy and location of the hypoperfused cerebral territory. We describe here a case of successful indirect bypass utilizing a pericranial flap as well as dural inversion. A seven-month-old female was transferred from an outside facility to our institution for further evaluation and surgical treatment of MMS. She presented with bilateral brain infarcts worse on the left, with right-sided body weakness. After medical stabilization and hyperhydration, she was taken to the operating room for a left-sided indirect bypass. The superficial temporal artery (STA) was traced utilizing doppler but was determined to be too diminutive for transposition, so the decision was made to proceed with encephalo-duro-pericranio-synangiosis (EDPS). A pericranial graft was successfully affixed to the cortical surface in the hypoperfused middle cerebral artery (MCA) territory, and the dura was inverted. Postoperatively, the patient developed a pseudomeningocele, so a revision surgery was performed. She was discharged shortly after this and returned for encephalo-duro-arterio-synangiosis (EDAS) of the contralateral side. She followed up three months after her initial bypass surgery at age 10 months and was crawling without any focal deficits. She was lost to follow-up thereafter. EDPS is a safe technique for infants with MMS whose STA is too diminutive to be used for bypass surgery. This may be an effective method for indirect bypass in these patients., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Ajala et al.)

Moyamoya disease (MMD) is a rare idiopathic progressive vaso-occlusive disease characterized by irreversible vascular occlusion and collateral development of distal internal carotid arteries. Initially perceived as an exclusive entity to the East Asian population, the disease is now being reported globally, affecting individuals of diverse ethnicities. We present a case of a 55-year-old African American male patient with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) and a prior history of cryptococcal meningitis presenting to the emergency department with recurrent episodic headaches, which was refractory to routine medical therapy. Neuroimaging with computed tomography angiogram of the head and neck and magnetic resonance imaging of the brain led to the subsequent diagnosis of moyamoya syndrome (MMS). To our knowledge, MMS is uncommon in adult HIV/AIDS patients. It is crucial that clinicians are aware of the disease progression. For effective recognition and prevention of the condition, it is of utmost importance that clinicians possess a comprehensive understanding of the disease and its clinical manifestations., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Arficho et al.)

This case report provides a detailed examination of a rare co-occurrence of Costello syndrome, euryblepharon, and Moyamoya syndrome in a 14-year-old female. Costello syndrome, a rare genetic disorder characterized by developmental delays, distinctive facial characteristics, and a predisposition to certain malignancies, presents an array of ocular manifestations, including downward-slanting palpebral fissures. A significant similarity is noted with euryblepharon, a rare periocular anomaly marked by the downward slanting of the eyelids. Despite these striking resemblances, the association between euryblepharon and Costello syndrome is yet to be documented in the literature. Furthermore, the coexistence of Costello syndrome and Moyamoya syndrome, a cerebrovascular disorder, is exceedingly rare. This report provides an in-depth analysis of the patient's ocular and periocular manifestations, establishing a potential association of euryblepharon within the phenotypic spectrum of Costello syndrome and documenting the unusual co-occurrence with Moyamoya syndrome. These findings aim to augment our understanding of Costello syndrome's phenotypic variability and potential associations., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Alhazmi et al.)

Moyamoya disease is a unique cerebrovascular disease characterized by narrowing of the terminal portion of internal carotid arteries and circle of Willis, with consequent development of a network of collateral vessels in response to brain ischemia. Moyamoya vascular pattern can be idiopathic (Moyamoya disease), is more likely to occur in individuals of Asian ascent and in the pediatric age, or is associated with other diseases (Moyamoya syndrome). We present two cases of stroke in young adults, where workup revealed Moyamoya-type vascular changes. The first case report is of a 42-year-old woman presenting with hemorrhagic stroke, with classic angiographic findings of Moyamoya disease, otherwise asymptomatic. The second case concerns a 36-year-old woman admitted with ischemic stroke; besides the typical angiographic pattern of Moyamoya, the patient was diagnosed with antiphospholipid antibody syndrome and Graves' disease, two conditions known to be associated with this vasculopathy. These case reports illustrate the need to consider this entity in the etiological evaluation of ischemic and hemorrhagic cerebrovascular events, even in Western countries, since management and secondary prevention require specific approaches., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Neves et al.)

Multiple myeloma (MM) is a common hematologic malignancy. Multi-agent chemotherapy and anti-myeloma immunomodulatory drugs increase the incidence of arterial and venous thrombosis. We present a moyamoya patient with MM who had a stroke shortly after induction chemotherapy. We present the case of an adult female patient who arrived at the ER due to automatism seizures, dysarthria, and left hemiparesis. The patient had a medical history of MM and underwent six cycles of induction chemotherapy (cyclophosphamide, dexamethasone, thalidomide, and bortezomib). MRI of the brain showed bilateral watershed ischemic strokes. Angiogram showed occlusion at the supraclinoid segment of both internal carotid arteries consistent with moyamoya. The patient was discharged with full-dose anticoagulation, levetiracetam, and physical therapy. At three years of follow-up, the patient has no recurrent cerebrovascular disease. MM patients treated with thalidomide/lenalidomide in combination with high-dose dexamethasone, doxorubicin, or multiagent chemotherapy should be on anticoagulation for venous thromboembolism (VTE) prophylaxis. There are no clear recommendations for arterial thrombosis prevention. Moyamoya is a vasculopathy characterized by progressive intracranial artery stenosis with a high risk of ischemic stroke, ischemia recurrence, and intracerebral hemorrhage. Despite the risk of intracerebral hemorrhage, we decided on anticoagulation due to the high risk of thrombosis due to MM, multi-agent chemotherapy, and moyamoya., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Castañeda-Hurtado et al.)

Moyamoya disease is a rare idiopathic disease characterized by progressive stenosis and collateral development of the distal internal carotid arteries. It is mainly seen in East Asia and is the most common cause of stroke in Asian children. However, it is rare in the Indian subcontinent. We present three exciting cases of moyamoya disease with varied clinical presentations in one pediatric, one young adult, and one older patient., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Rajamani et al.)

Moyamoya syndrome in a sickle cell disease patient may be a difficult task to manage in the setting of a vaso-occlusive pain crisis. Maintaining stable blood pressure is necessary to prevent stroke as both hypertension and hypotension can be detrimental to the patient, leading to hemorrhagic and ischemic stroke, respectively. Opioid management for pain control in such patients must be taken into consideration. Because every patient is unique, opioid regimens should be optimized to relieve patients' specific pain while also practicing non-maleficence in preventing hypotension and strokes., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Mills et al.)

Moyamoya disease (MMD) is a rare, chronic cerebrovascular disease affecting the cerebral arteries, leading to the development of unique collateral vessels. Few cases were reported from Saudi Arabia; however, the incidence rate is not well-defined. Hence, we present a case of a 13-month-old child who presented to the emergency room with first onset focal seizures with relatively unremarkable past medical and family history. Investigations were ordered accordingly including head computed tomography (CT) scan, brain magnetic resonance imaging (MRI), and cerebral angiogram, and he was diagnosed with MMD and considering a broader variety of differential diagnoses for seizures in children is highlighted in our case. Furthermore, considering their predominance in east Asian countries, it highlights a rare presentation in the middle eastern race., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, M. Ali et al.)

Moyamoya syndrome is a chronic and progressive narrowing of the arteries in the brain caused by different mechanisms than the genetic mutation that leads to moyamoya disease. It is characterized by the narrowing and/or closing of the carotid artery with a collateral circulation development around the blocked vessels to compensate for the ischemia. In this report, we present a unique case of moyamoya syndrome that developed over the course of a few months in a patient with new-onset strokes and seizures in the setting of late diagnosis of neurosyphilis and acquired immunodeficiency syndrome (AIDS). To our knowledge, moyamoya syndrome secondary to coinfection with AIDS and meningovascular neurosyphilis has only been reported once in the literature., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Wilson et al.)

Moyamoya syndrome consists of internal carotid artery stenosis with development of collateral vasculature responsible for ischemic events and cerebral hemorrhage. Moyamoya vasculopathy is commonly treated with external carotid artery to internal carotid artery bypass, either through direct or indirect anastomosis. Klippel-Trenaunay Syndrome (KTS) is a tissue hyper-proliferation disorder known to have a significant angio-dysplastic component to the pathology. No other instances of a patient with both KTS and Moyamoya syndrome are presently reported in the literature. We present a patient who had been diagnosed with KTS as a child who was found to have Moyamoya vasculopathy after experiencing frequent cerebral ischemic events. He underwent a left direct superficial temporal artery to middle cerebral artery bypass with subsequent significant improvement of his stroke symptoms. This case report demonstrates an association between KTS and Moyamoya syndrome with a possible shared pathophysiology. Patients with KTS may benefit from screening for cerebral ischemic events and monitoring for development of Moyamoya syndrome., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Brimley et al.)

Moyamoya disease (MMD) is an unusual vasculopathy in which the blood vessels of the brain are occluded, resulting in thrombosis. When MMD occurs in association with an underlying pathology, it is known as moyamoya syndrome. The association of moyamoya syndrome with sickle cell disease is uncommon. Herein, we report a case of moyamoya syndrome in a 32-year-old male adult patient., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Yamani et al.)

Moyamoya disease is a rare cerebrovascular disease of unknown etiology, once known to be rare in the United States as compared to East-Asian countries, it is now an increasingly recognized cause of strokes in the United States, as the prevalence of the disease appears to be increasing. We describe a case of a 41-year-old Hispanic female patient presenting to our hospital with a stroke. She had two episodes of right arm weakness and clumsiness prior to presentation to the hospital that had resolved upon arrival. Despite a CT head negative for stroke, further imaging work-up was performed including MRI of the brain with magnetic resonance angiography (MRA) and conventional angiogram, which showed characteristic imaging findings leading to the diagnosis of Moyamoya disease. The patient subsequently underwent elective surgical intervention with Encephaloduroarteriosynangiosis (EDAS) procedure to prevent further complications., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Bani Hani et al.)

Moyamoya vasculopathy is a condition of chronic, progressive occlusion of the distal internal carotid arteries and the Circle of Willis. The resultant ischemia produces compensatory angiogenesis and the growth of a network of collateral blood vessels, which on angiography resemble a "puff of smoke" or "moyamoya" in Japanese. The objective of this case report is to describe the clinical course of a patient with Down and moyamoya syndromes and to enlighten clinicians about strategies that can be taken to enhance the care of similar patients. A 55-year-old African American female presented to the hospital with complaints of headache, vision loss, dysarthria, and ataxia. She had a past medical history of Down syndrome and a stroke with residual lower extremity weakness. At her baseline, the patient was able to perform her activities of daily living but required assistance with independent activities of daily living. Computed tomography of the brain showed hypodense areas at the right occipital, temporal, and parietal lobes. Computed tomography angiography of the head and neck identified occlusion bilaterally at the supraclinoid internal carotid arteries and right posterior cerebral artery; there was collateral arterial flow within the right middle cerebral and anterior cerebral arteries that was consistent with moyamoya vasculopathy. Patients with Down syndrome experience premature accelerated aging and suffer from comorbidities seen in geriatric patients by the time they reach their 40s. Patients with moyamoya vasculopathy experience neurocognitive and neuropsychiatric deficits that correspond to the regions of the brain that are affected. This patient with Down and moyamoya syndromes had impaired neurocognitive and functional status, and we believe that she would have benefited from receiving a comprehensive geriatric assessment and neuropsychiatric testing., Competing Interests: The authors have declared that no competing interests exist.

Moyamoya syndrome is a rare cerebrovasculopathy of unknown etiology which is associated with multiple risk factors. Moyamoya was first discovered in Japan and is reported to have an increased prevalence in the Japanese population. The term "Moyamoya" translates into "puff of smoke" and is named after the finding of the collateral cerebral vasculature that develops secondary to occlusion of an internal carotid artery at the entrance into the circle of Willis. This collateral vasculature characterizes the disease. Moyamoya should be included in the differential diagnosis in the pediatric population when a patient presents with stroke or stroke-like symptoms. Diagnosis can be made with catheter angiogram or magnetic resonance angiogram. Recent use of magnetic resonance perfusion imaging has been shown to be useful in clinical decision making while assessing the need for revascularization surgery. We present the case of a 15-year-old with comorbid psychiatric illness, neurofibromatosis type I with brainstem glioma, and Moyamoya syndrome. Considering our patient`s complex medical history of psychiatric illness and previously diagnosed neurofibromatosis, magnetic resonance imaging (MRI) with magnetic resonance angiogram (MRA) and magnetic resonance perfusion proved instrumental in helping rule out the progression of arteriopathy as the cause of his worsening seizures and behavior. In our patient, it was determined that the relative perfusion for each hemisphere of the patient's brain quantitatively lacked significant differences and he was therefore not a candidate for surgical revascularization. These modalities proved instrumental in surgical decision-making and clinical management of the patient., Competing Interests: The authors have declared that no competing interests exist.