I saw the 'puff of smoke' sign before it vanished into thin air.

Moyamoya is characterized as a non-atherosclerotic and non-inflammatory vasculopathy that leads to progressive stenosis of the intracranial internal carotid arteries as well as the Circle of Willis. While it can be idiopathic (Moyamoya disease) or associated with another condition (Moyamoya syndrome), there is a characteristic 'puff of smoke' sign that can be appreciated on cerebral angiography.

Moyamoya Disease Causing Stroke in the Setting of Cocaine Use and Uncontrolled Hypertension Due to Primary Hyperaldosteronism.

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.

Fascinating Case Presentation of Moyamoya Disease in Children and Adults.

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.

Beta-Thalassemia Presenting as Moyamoya Syndrome With a Review of Skeletal Manifestations.

Moyamoya angiopathy (MMA) is a progressive vasculopathy characterized by slowly progressive stenosis involving the proximal portions of the major intracranial arteries, resulting in strokes and intracranial hemorrhages. If it occurs secondary to a known cause, it is called Moyamoya syndrome (MMS). Here, we describe the case of a five-year-old male child who presented to us with symptoms of stroke and, upon evaluation, revealed Moyamoya angiopathy. He was further evaluated, and it was found that MMA occurred as a complication of undetected beta-thalassemia. Thalassemia is an autosomal recessive blood disorder where there is a defect in hemoglobin production. It affects 100 to 150 thousand children in the Indian subcontinent. It is classified into two main types: alpha thalassemia and beta thalassemia, depending on which globin chain is affected. It primarily presents with symptoms of anemia such as easy fatiguability, dizziness, jaundice, or breathlessness. The occurrence of Moyamoya syndrome in beta-thalassemia is extremely rare, and it is extremely important to identify MMS at the earliest as it can cause long-term disabilities. We describe the imaging findings in MMS and the various classical skeletal radiographic findings in thalassemia that were seen in our patient.

A single - center retrospective observational study on patients undergoing Encephalo-Duro-Arterio -Myo-Synangiosis in patients with moyamoya disease.

BACKGROUND: Many cases of moyamoya disease are refractory to conventional medical therapy, hence surgical revascularization techniques have emerged as one of the primary choices of treatment. In this study, we present the functional and angiographic outcomes of patients undergoing encephalo-duro-arterio-myo-synangiosis (EDAMS). METHODS: This is a retrospective observational cohort study, done over 8 years (2012-2020) in a neurological center in Eastern India. Data were retrieved from the hospital's electronic system, recorded in a standard data abstract sheet, and analyzed. RESULTS: This study included 75 patients, with a male (n = 42; 56.0%) preponderance. Majority belonged to the pediatric age group (≤18 years) (n = 70; 93.3%); remaining adult population included 5 (6.6%) patients. The most common presenting complaint was that of an ischemic cerebrovascular accident (CVA) (n = 57; 76.0%). Symptomatic hemispheres (n = 69; 92.0%) were treated and later followed if they had progressed to bilateral disease formation. Preoperative DSA showed 50 (71.4%) to have Suzuki grade 3 type of angiographic findings. Postoperative complications included worsening unilateral hemiparesis 4 (40%), slurring of speech (n = 2; 20.0%), hematoma (n = 2; 20.0%), and surgical site infection (n = 2; 20.0%). One patient succumbed to his illness on the second postoperative day. A postoperative angiogram showed regression of moyamoya vessels in the majority (n = 69; 93.3%) of patients. All (n = 74; 100%) had an intensification of transdural vessels; none had a regression. None of the study participants showed an intensification of moyamoya vessels. Many of our patients (83.8%) had a good grade of revascularization (modified Matsushima and Inaba A and B), while 16.2% had low-grade revascularization (grade C). On assessing outcomes using a modified Rankin Score, a large number of our patients had an excellent (n = 45, 60%) neurological outcomes. CONCLUSION: There was a bimodal age distribution with most of them presenting with ischemic CVA. This procedure (EDAMS) had good angiographic (Matsushima and Inaba) and functional (modified Rankin Score) outcomes.

Moyamoya Disease in a 13-Month-Old Middle Eastern Boy.

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.

Complications following Encephalo-Duro-Arterio-Myo-Synangiosis in a case of Moyamoya disease.

We report a case of an 18-year-old girl diagnosed to have Moyamoya disease (MMD), who underwent bilateral encephalo-duro-arterio-myo-synangiosis. Literature search has clearly inferred that in comparison to an adult patient, children with MMD can have a good prognosis if early diagnosis and active surgical intervention are achieved. Evidence has demonstrated that active surgical management, including indirect bypass surgery, may improve the cerebral circulation on a relatively larger scale than direct bypass surgeries, which can only improve the cerebral circulation in the area of the vascular reconstruction.

Moyamoya Syndrome in a Child with Neurofibromatosis Type 1: Magnetic Resonance Imaging as a Tool for Clinical Decision Making.

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.