Moyamoya disease and syndrome

Moyamoya syndrome (MMS) is a rare, chronic, progressive cerebrovascular disorder characterized by stenosis at the apices of the intracranial internal carotid arteries, including the proximal anterior cerebral arteries and middle cerebral arteries. Cerebral angiography images are used for detection through measurement. Systemic lupus erythematosus (SLE) is an autoimmune disease that can cause multisystemic involvement. The coexistence of SLE and MMS has been rarely reported in the literature. A 46-year-old male patient with malar rash, Raynaud phenomenon presented to the hospital with a complaint of weakness in the left lower extremity, which began 3 days before the date of the visit...

INTRODUCTION: Moyamoya disease (MMD) is a life-threatening condition characterized by stenosis of intracranial arteries. Despite the frequency and the impact of psychiatric symptoms on the long-term prognosis and quality of life of MMD patients, no systematic review on this topic exists. METHODS: This systematic review and meta-analysis included 41 studies (29 being case reports), from PubMed, Scopus, Embase until 27/3/2023, on MMD patients exhibiting psychiatric symptoms...

BACKGROUND: Postoperative hyperperfusion syndrome (PHS) is a well-known complication following superficial temporal artery (STA)-middle cerebral artery (MCA) bypass for moyamoya disease (MMD). The early detection of postoperative radiological hyperperfusion (PRH), characterized by a transient increase in local cerebral blood flow (CBF), is crucial for the early diagnosis of PHS. This study aimed to investigate the effectiveness of waveform analysis for early PRH detection. METHODS: We reviewed 52 consecutive patients who underwent STA-MCA bypass for MMD...

Craniopharyngioma (CP) treatment, including surgery and radiotherapy, can have short- and long-term vascular side effects. Hypothalamic damage is related to morbid obesity and may increase the lifelong risk of experiencing vascular events in CP patients. This review summarized the available evidence regarding vascular complications in adamantinomatous or papillary CP patients, whatever their age at diagnosis. Three databases (Medline, CINAHL, Web of Science) were searched (06/2023) to retrieve eligible articles...

PURPOSE: Sickle-cell disease-associated moyamoya syndrome (SCD-MMS) carries a high risk for recurrent strokes and cerebrovascular morbidity in children. However, few data are available about complications that occur in children hospitalized with SCD-MMS. The purpose of this analysis was to determine the risk factors for in-hospital complications in pediatric SCD-MMS admissions, and thus aid physicians in optimizing future treatment plans. METHODS: A national database of pediatric hospital admissions was examined across the years 2003-2019...

Fanconi anemia (FA) is an inherited bone marrow failure syndrome associated with congenital anomalies and a predisposition to cancer. We report the case of a 9-year-old boy with FA who developed an abrupt onset of hemiplegia and dysarthria. The diagnosis of moyamoya disease (MMD) was suggested by magnetic resonance angiography (MRA) which demonstrated severe stenosis in the right internal carotid artery along with collateral vessel formation in the right basal ganglia. It is questioned whether the moyamoya pattern in this case is part of congenital malformations associated with FA or is the result of recurrent bleedings around the carotid siphon...

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...

Moyamoya disease is a progressive cerebrovascular disorder for which there is no cure. It is characterized by narrowing of and occlusions in the blood vessels that supply the brain, which causes a fine vascular network to develop to serve as collateral pathways. Moyamoya disease can lead to a reduction of blood flow to the brain and increase the risk of stroke. Patients with moyamoya disease may present with ischemic or hemorrhagic complications. Treatment options may involve medical management or surgical revascularization (indirect, direct, or a combined approach)...

The coexistence of Moyamoya Syndrome with Arteriovenous Malformation is exceedingly rare. Here, we present the case of a 37-year-old female patient diagnosed with AVM in the right parietal lobe, accompanied by severe stenosis of the right middle cerebral artery and right anterior cerebral artery, along with moyamoya collateral induction. Our objective was to investigate the frequency and mutual influence of these conditions, and to determine a preferable treatment strategy by conducting a comprehensive review of previous case reports...

Background and objectives Several Asian populations abroad are reported to have a higher prevalence of spontaneous intracranial hemorrhages (sICH) and a greater proportion of all stroke types attributed to ICH compared to non-Asians. However, the causes are unknown, and few studies have examined the issue among Asian Americans. This report aims to highlight some less common, but not rare, clinical features that could bear on several pathophysiological factors, by presenting a selected case series of 13 Asian American patients admitted to a Boston-based healthcare system and hospital...

BACKGROUND AND AIMS: Moyamoya is a chronic brain vasculopathy involving the distal intracranial internal carotid artery (ICA) or proximal middle cerebral artery (MCA). Moyamoya patients can be divided into those with primary moyamoya disease (MMD) and those with moyamoya secondary to other known causes such as intracranial atherosclerosis (moymoya syndrome [MMS]). Our aim was to compare the characteristics of MMD patients to those of MMS patients in a sample of Israeli patients seen over the course of 20 years at a tertiary referral center...

Moyamoya disease (MMD) is a rare idiopathic cerebrovascular disorder that causes transient ischemic attack (TIA) and ischemic stroke in the pediatric population. Herein, we report an extremely rare case of Moyamoya syndrome (MMS) and late-onset idiopathic aqueduct stenosis, a unique form of non-communicating hydrocephalus. A 17-year-old female presented with an intractable headache and occasional faintness. Pertinent medical history included a fourth ventricle epidermoid cyst without any evidence of aqueduct stenosis, which was surgically removed when she was two years of age...

Moyamoya disease (MMD), characterized by progressive internal carotid artery stenosis and collateral vessel formation, prompts cerebral perfusion complications and is stratified into idiopathic and Moyamoya syndrome subtypes. A multifaceted approach toward MMD management addresses cerebral infarctions through revascularization surgery and adjunctive medical therapy, while also navigating risks such as intracranial hemorrhage and cerebral infarction resulting from arterial stenosis and fragile collateral vessels...

BACKGROUND: Cerebral hyperperfusion syndrome (CHS) is known as a complication after bypass surgery for Moyamoya disease (MMD). However, the incidence of CHS has not been accurately reported, and there is no consensus on the risk factors associated with it. AIM: The aim of this study was to determine the risk factors associated with postoperative CHS after surgical combined revascularization used to treat adult patients with MMD. OBJECTIVE: To assess the frequency and characteristics of CHS in patients with MMD after revascularization operations...

BACKGROUND: The optimal timing and surgical approach for surgical revascularization in patients with moyamoya syndrome (MMS) associated with neurofibromatosis type I (NF1) remain so far elusive. We aimed to compare the long-term clinical, radiological, and cognitive effects of different revascularization procedures in a pediatric cohort of NF1-associated MMS. METHODS: We reviewed the clinical, radiological, and surgical data of 26 patients with NF1-associated MMS diagnosed at our institution between 2012 and 2022, at the clinical onset and last follow-up...

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...

BACKGROUND: Moyamoya disease (MMD) is a chronic ischemic cerebrovascular disease. Collateral circulation in MMD has emerged as a research focus. Our aims were to assess the impact of anastomoses between the anterior and posterior circulations on the prognosis of MMD patients. METHODS: We reviewed the preoperative digital subtraction angiography images of patients with MMD who underwent revascularization surgery at our hospital between March 2014 and May 2020 and divided the patients into two groups: those with anastomoses (PtoA group) and those without anastomoses (non-PtoA group)...

In this article, we describe a rare and complex case of moyamoya syndrome in a 7-year-old boy with Down syndrome and atlantoaxial subluxation. The patient presented with an ischemic stroke in the left hemisphere and cervical cord compression with increased cord edema. Diagnostic digital subtraction angiography revealed unique patterns of vascular involvement, with retrograde flow through the anterior spinal artery, ascending cervical artery, occipital artery, and multiple leptomeningeal arteries compensating for bilateral vertebral artery occlusion...

BACKGROUND AND OBJECTIVES: Many reversible brain MRI abnormalities have been described, among these the most frequently reported are cortical hyperintensities on FLAIR/T2 occurring during seizures. Much less attention has been given to those situations where White Matter goes Dark: subcortical white matter hypointensity on T2/FLAIR. Our aim is to identify the medical condition "Dark White Matter" (DWM) is more frequently associated with. This is the first systematic review on DWM. METHODS: PubMed was searched in August 2023...

The term Moyamoya , or "puff of smoke" in Japanese , was first used in 1969 by Suzuki and Takaku to describe the classical appearance of collateral blood vessels in response to progressive vascular stenosis of distal internal carotid artery (ICA). Such condition may result in various clinical presentations ranging from strokes to developmental delays. In order to cease the progression of such stenotic vasculopathy, several means of revascularization have been developed over the years. In this paper we present a case of a two-year-old girl with history of myelomeningocele repair and ventriculoperitoneal shunt insertion followed by manifestation of Moyamoya syndrome later in childhood as an evidence of revascularization through a burr hole...