Materiały źródłowe

• #1 Moyamoya disease: Diagnostic imaging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3389911/

  Moyamoya disease is a progressive vasculopathy leading to stenosis of the main intracranial arteries. […] Currently, magnetic resonance angiography and CT angiography with multi-row systems are the main imaging methods of diagnostics of the entire range of vascular changes in moyamoya disease. […] An appropriate qualification to surgery should be based on a comprehensive angiographic and imaging evaluation of brain structures. […] Angiographic criteria of the diagnosis of moyamoya disease were established in 1998. They include stenosis or occlusion of the distal parts of intracranial internal carotid arteries and proximal parts of anterior and middle arteries, as well as the presence of collateral vasculature in the regions of the brain base, without causal disease. […] In ambiguous cases, it is indicated to carry out MRI, which not only helps in establishing the diagnosis, but also allows for a better evaluation of the range and time phase of ischaemic lesions.

• #2 Moyamoya disease | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/moyamoya-disease-1?lang=us

  Moyamoya disease is an idiopathic, non-inflammatory, non-atherosclerotic progressive vaso-occlusive disease involving the terminal supraclinoid internal carotid arteries and circle of Willis. […] The term moyamoya disease should be reserved for an idiopathic, sometimes familial, condition, which leads to characteristic intracranial vascular changes. […] This article concerns itself with moyamoya disease. For a list of conditions that can mimic moyamoya disease please refer to the article on moyamoya syndrome or the differential diagnosis list at the end of the article. […] In addition to demonstrating the aforementioned arterial stenoses (best seen on MRA) or enlarged perforators (best seen on T2 weighted images), vessel wall imaging can also be helpful, particularly in distinguishing moyamoya disease from moyamoya syndrome from atherosclerotic or vasculitic/inflammatory causes. […] Vessel wall imaging in moyamoya disease typically demonstrates concentric luminal narrowing, none or only minimal enhancement, homogeneous T2 signal, and no outward remodeling (outer wall area of the stenotic segment greater than proximal normal segment; seen in atherosclerosis).

• #3 Epidemiology, diagnosis and treatment of moyamoya disease (Review)

  https://www.spandidos-publications.com/10.3892/etm.2019.7198

  Moyamoya disease (MMD) is a type of chronic cerebrovascular occlusion disease, which frequently occurs in East Asian populations, including pediatric and adult patients, and may lead to ischemic or hemorrhagic stroke, headache, epilepsy or transient ischemic attack. […] Cerebral angiography is the best approach for diagnosing MMD. However, with technological advances, noninvasive techniques are increasingly used to accurately evaluate MMD. […] In 1996, Japan issued a guide for the diagnosis and treatment of the spontaneous occlusion of the circle of Willis (Moyamoya disease, MMD), which suggests the following manifestations on cerebral angiography i) Stenosis or occlusion at the end of the carotid artery, the proximal ACA and/or MCA; ii) an abnormal vascular network in the vicinity of stenotic occlusion lesions in the arterial phase; and iii) the above manifestations are bilateral.

• #4 Moyamoya Disease Workup: Approach Considerations, Laboratory Studies, Angiography

  https://emedicine.medscape.com/article/1180952-workup

  Misdiagnosis and delayed diagnosis of moyamoya disease are particular pitfalls in the treatment of patients with this disorder. Misdiagnosis can occur easily if the physician does not incorporate moyamoya disease into the differential diagnosis of any patient presenting with stroke. […] If moyamoya disease is not considered seriously, then appropriate diagnostic tests may not be performed and a delay in diagnosis could result. […] If an ischemic stroke progresses despite preventative treatment with antiplatelet agents or anticoagulants, then moyamoya disease should be considered as a possible etiology. […] Cerebral angiography is the criterion standard for the diagnosis of moyamoya disease. The following findings support the diagnosis: Stenosis or occlusion at the terminal portion of the internal carotid artery or the proximal portion of the anterior or middle cerebral arteries. […] Magnetic resonance angiography (MRA) or computed tomography angiography (CTA) can be performed. Any of the above findings on MRA or CTA may preclude the need for conventional angiography.

• #5 Moyamoya Disease | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/m/moyamoya-disease.html

  Moyamoya disease is most commonly diagnosed in children, but it can affect adults as well. […] Because moyamoya disease can cause severe brain damage or even death, it is important to diagnose and treat the condition as quickly as possible. Diagnosis of the condition generally begins with a physical exam and a review of the patient’s medical history. The patient’s physician may recommend one or more of the following tests or image diagnostic tools in order to more clearly understand the patient’s condition. […] These imaging tools can assist the physician in finding the blockage and the tangle of blood vessels, as well as see how much damage may have been done by the condition.

• #6 Moyamoya Disease – Child Neurology Foundation

  https://www.childneurologyfoundation.org/disorder/moyamoya-disease/

  Moyamoya disease is a common cause of stroke in children. […] It is important that children with moyamoya disease are diagnosed early. […] Moyamoya is diagnosed using pictures of a child’s brain and blood vessels. The most common way to get these pictures is with MRI, or magnetic resonance imaging. However, additional pictures may be needed. […] A procedure called a cerebral angiogram can confirm a moyamoya diagnosis. A cerebral angiogram can also show how severe the condition is. It can help plan for surgery. […] Most children with moyamoya do not need blood tests. But blood testing may be needed for other reasons, including medication monitoring, genetic testing, surgical planning, and in rare cases when other causes are suspected.

• #7 Diagnostic Criteria for Moyamoya Disease – 2021 Revised Version

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9357455/

  In this report, we, the Research Committee on Moyamoya Disease (Spontaneous Occlusion of the circle of Willis), describe in detail the changes in the new Diagnostic Criteria 2021 for moyamoya disease and its scientific basis to make it widely known to the world. The revised criteria cover all aspects of the disease, including a definition of the disease concept, diagnostic imaging, and the concept of quasi-moyamoya disease (moyamoya syndrome). […] The diagnostic criteria for this worldwide known moyamoya disease should be shared widely across the world as a common language. […] In 2021, we refined the diagnostic criteria for moyamoya disease again to further improve the accuracy of the diagnosis. […] The 2009 English version of the diagnostic criteria is shown in Table 1. […] Diagnostic Assessment Moyamoya disease should be classified as definite or probable based on the abovementioned items (1) to (4).

• #8 Diagnostic Criteria for Moyamoya Disease – 2021 Revised Version

  https://www.jstage.jst.go.jp/article/nmc/62/7/62_2022-0072/_article

  In this report, we, the Research Committee on Moyamoya Disease (Spontaneous Occlusion of the circle of Willis), describe in detail the changes in the new „Diagnostic Criteria 2021” for moyamoya disease and its scientific basis to make it widely known to the world. The revised criteria cover all aspects of the disease, including a definition of the disease concept, diagnostic imaging, and the concept of quasi-moyamoya disease (moyamoya syndrome).

• #9 Diagnostic Criteria for Moyamoya Disease – 2021 Revised Version

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9357455/

  Moyamoya disease can be diagnosed when all of the following findings are found on MRI and MRA (time-of-flight; TOF) using a scanner with a static magnetic field strength of 1.5 Tesla (T) or higher (3.0 T is even more useful). […] Moyamoya disease is diagnosed when (1) and (2) of A-1 or (1) to (3) of A-2 are met and B is excluded. […] In the 2021 version of the diagnostic criteria, therefore, we decided to clearly state as follows: Especially in the case of unilateral lesions or lesions complicated by atherosclerosis, it is essential to perform cerebral angiography to exclude other diseases. Thus, the 2021 version of the diagnostic criteria emphasizes the importance of cerebral angiography more than ever. […] In fact, it is well known that the diagnosis of moyamoya disease in the early stage of the disease is not easy by MRA alone. In early-stage cases where the above three findings cannot be obtained by MRI and MRA, we recommend definitive diagnosis by cerebral angiography. […] Quasi-moyamoya disease is also often referred to as moyamoya syndrome. […] The revised criteria cover all aspects of the disease, including a definition of the disease concept, diagnostic imaging, and the concept of quasi-moyamoya disease (moyamoya syndrome).

• #10 Moyamoya disease | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/moyamoya-disease-1?lang=us

  Moyamoya disease is an idiopathic, non-inflammatory, non-atherosclerotic progressive vaso-occlusive disease involving the terminal supraclinoid internal carotid arteries and circle of Willis. […] The term moyamoya disease should be reserved for an idiopathic, sometimes familial, condition, which leads to characteristic intracranial vascular changes. […] This article concerns itself with moyamoya disease. For a list of conditions that can mimic moyamoya disease please refer to the article on moyamoya syndrome or the differential diagnosis list at the end of the article. […] In addition to demonstrating the aforementioned arterial stenoses (best seen on MRA) or enlarged perforators (best seen on T2 weighted images), vessel wall imaging can also be helpful, particularly in distinguishing moyamoya disease from moyamoya syndrome from atherosclerotic or vasculitic/inflammatory causes. […] Vessel wall imaging in moyamoya disease typically demonstrates concentric luminal narrowing, none or only minimal enhancement, homogeneous T2 signal, and no outward remodeling (outer wall area of the stenotic segment greater than proximal normal segment; seen in atherosclerosis).

• #11 Moyamoya disease: Diagnostic imaging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3389911/

  Moyamoya disease is a progressive vasculopathy leading to stenosis of the main intracranial arteries. […] Currently, magnetic resonance angiography and CT angiography with multi-row systems are the main imaging methods of diagnostics of the entire range of vascular changes in moyamoya disease. […] An appropriate qualification to surgery should be based on a comprehensive angiographic and imaging evaluation of brain structures. […] Angiographic criteria of the diagnosis of moyamoya disease were established in 1998. They include stenosis or occlusion of the distal parts of intracranial internal carotid arteries and proximal parts of anterior and middle arteries, as well as the presence of collateral vasculature in the regions of the brain base, without causal disease. […] In ambiguous cases, it is indicated to carry out MRI, which not only helps in establishing the diagnosis, but also allows for a better evaluation of the range and time phase of ischaemic lesions.

• #12 Moyamoya Disease Workup: Approach Considerations, Laboratory Studies, Angiography

  https://emedicine.medscape.com/article/1180952-workup

  Misdiagnosis and delayed diagnosis of moyamoya disease are particular pitfalls in the treatment of patients with this disorder. Misdiagnosis can occur easily if the physician does not incorporate moyamoya disease into the differential diagnosis of any patient presenting with stroke. […] If moyamoya disease is not considered seriously, then appropriate diagnostic tests may not be performed and a delay in diagnosis could result. […] If an ischemic stroke progresses despite preventative treatment with antiplatelet agents or anticoagulants, then moyamoya disease should be considered as a possible etiology. […] Cerebral angiography is the criterion standard for the diagnosis of moyamoya disease. The following findings support the diagnosis: Stenosis or occlusion at the terminal portion of the internal carotid artery or the proximal portion of the anterior or middle cerebral arteries. […] Magnetic resonance angiography (MRA) or computed tomography angiography (CTA) can be performed. Any of the above findings on MRA or CTA may preclude the need for conventional angiography.

• #13 Moyamoya Disease Diagnosis [Neurosurgery Education Wiki]

  https://neurosurgery.education/wiki/doku.php?id=moyamoya_disease_diagnosis

  Diagnosis of Moyamoya disease requires bilateral symmetrical stenosis or occlusion of the terminal portion of the internal carotid arterys (ICA)s as well as the presence of dilated collateral vessels at the base of the brain 1). (If unilateral, the diagnosis is considered questionable, 2) and these cases may progress to bilateral involvement). […] Work-up in suspected cases typically begins with a non-enhanced head CT. Up to 40% of ischemic cases have normal CT. […] In addition to helping to establish the diagnosis, angiography also identifies suitable vessels for revascularization procedures and unearths associated aneurysms. […] Six angiographic stages of MMD are described by Suzuki and Takaku 3) that tend to progress up until adolescence and stabilize by age 20. […] Ultrasound provides a new way to identify stroke in MMD patients. Future prospective cohort studies are needed to verify the clinical value of ultrasound in identifying patients with MMD at high risk of stroke 7). […] This study demonstrated a characteristic metabolic pattern that can distinguish patients with MMD without VCI from those with VCI, namely, hypometabolic lesions in the left hemisphere played a more important role in cognitive decline in patients with MMD 8).

• #14 Moyamoya disease – Wikipedia

  https://en.wikipedia.org/wiki/Moyamoya_disease

  Cerebral angiography is the gold standard of diagnosing moyamoya disease and its progression. According to Suzuki’s system, it can be classified into six stages: […] Magnetic resonance angiography (MRA) is also useful in diagnosing the disease with good correlation with Suzuki’s grading system. […] Often nuclear medicine studies such as SPECT (single photon emission computerized tomography) are used to demonstrate the decreased blood and oxygen supply to areas of the brain involved with moyamoya disease. Conventional cerebral angiography provides the conclusive diagnosis of moyamoya disease in most cases and should be performed before any surgical considerations. […] There is also research that has shown that certain radiographic biomarkers that lead to the diagnosis of moyamoya disease have been identified. The specific radiographic markers are now considered an acceptable key component to moyamoya disease and have been added to the International Classification of Diseases (ICD). […] If this is not the case and the findings are unilateral, it is diagnosed as moyamoya syndrome. […] The occlusion of the ICA results in concomitant diminution of the „puff-of-smoke” collaterals, as they are supplied by the ICA.

• #15 Moyamoya disease: Diagnostic imaging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3389911/

  Moyamoya disease is a progressive vasculopathy leading to stenosis of the main intracranial arteries. […] Currently, magnetic resonance angiography and CT angiography with multi-row systems are the main imaging methods of diagnostics of the entire range of vascular changes in moyamoya disease. […] An appropriate qualification to surgery should be based on a comprehensive angiographic and imaging evaluation of brain structures. […] Angiographic criteria of the diagnosis of moyamoya disease were established in 1998. They include stenosis or occlusion of the distal parts of intracranial internal carotid arteries and proximal parts of anterior and middle arteries, as well as the presence of collateral vasculature in the regions of the brain base, without causal disease. […] In ambiguous cases, it is indicated to carry out MRI, which not only helps in establishing the diagnosis, but also allows for a better evaluation of the range and time phase of ischaemic lesions.

• #16 Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis – UpToDate

  https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print

  Moyamoya disease (MMD) is a condition characterized by the occurrence of moyamoya angiographic findings along with associated genetic susceptibilities but no underlying contributing medical conditions. MMD may also be called primary or idiopathic moyamoya as well and may also be referred to descriptively as the „spontaneous occlusion of the circle of Willis.” […] Moyamoya syndrome (MMS) is a condition characterized by the occurrence of moyamoya angiographic findings along with an associated medical condition implicated in the development of vascular changes.

• #17 Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis – UpToDate

  https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print

  Moyamoya disease (MMD) is a condition characterized by the occurrence of moyamoya angiographic findings along with associated genetic susceptibilities but no underlying contributing medical conditions. MMD may also be called primary or idiopathic moyamoya as well and may also be referred to descriptively as the „spontaneous occlusion of the circle of Willis.” […] Moyamoya syndrome (MMS) is a condition characterized by the occurrence of moyamoya angiographic findings along with an associated medical condition implicated in the development of vascular changes.

• #18 Epidemiology, diagnosis and treatment of moyamoya disease (Review)

  https://www.spandidos-publications.com/10.3892/etm.2019.7198

  In the 2012 guidelines, cerebral angiography remains the gold standard for the diagnosis of MMD with the staging performed according to angiographic findings. […] According to the new guidelines, differential diagnoses of MMD, which should be excluded, are the following underlying cerebrovascular diseases: Atherosclerosis, autoimmune diseases, meningitis, brain tumors, Down syndrome, Recklinghausen’s disease, head injury and cerebrovascular damage after head irradiation. […] The clinical symptoms of MMD include transient ischemic attacks, ischemic stroke, hemorrhagic stroke, epilepsy, headache and cognitive dysfunction, with the incidence of each symptom varying depending on the age of the patient. […] MMD has two major symptoms: Cerebral ischemia and cerebral hemorrhage. […] Cerebral angiography is the gold standard for diagnosing MMD and assessing its progression.

• #19 The contribution of imaging in diagnosis, preoperative assessment, and follow-up of moyamoya disease in: Neurosurgical Focus Volume 26 Issue 4 (2009) Journals

  https://thejns.org/focus/view/journals/neurosurg-focus/26/4/article-pE3.xml

  The aim of this review was to evaluate the imaging tools used in diagnosis and perioperative assessment of moyamoya disease, with particular attention to the last decade. […] Catheter angiography still represents the gold standard for moyamoya diagnosis. […] Angiography might also reveal the presence of aneurysms that may be related to the increased flow load in relation to the normal vessel diameter in these patients. […] According to the guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis, angiography is not mandatory for the diagnosis of moyamoya disease, because MR imaging and MR angiography can show all the findings required for diagnosis. […] The MR imaging should be performed on 1.5-T (or higher) systems, as suggested by the Research Committee for Diagnosis of moyamoya.

• #20 Epidemiology, diagnosis and treatment of moyamoya disease (Review)

  https://www.spandidos-publications.com/10.3892/etm.2019.7198

  In the 2012 guidelines, cerebral angiography remains the gold standard for the diagnosis of MMD with the staging performed according to angiographic findings. […] According to the new guidelines, differential diagnoses of MMD, which should be excluded, are the following underlying cerebrovascular diseases: Atherosclerosis, autoimmune diseases, meningitis, brain tumors, Down syndrome, Recklinghausen’s disease, head injury and cerebrovascular damage after head irradiation. […] The clinical symptoms of MMD include transient ischemic attacks, ischemic stroke, hemorrhagic stroke, epilepsy, headache and cognitive dysfunction, with the incidence of each symptom varying depending on the age of the patient. […] MMD has two major symptoms: Cerebral ischemia and cerebral hemorrhage. […] Cerebral angiography is the gold standard for diagnosing MMD and assessing its progression.

• #21 Moyamoya Disease Workup: Approach Considerations, Laboratory Studies, Angiography

  https://emedicine.medscape.com/article/1180952-workup

  Misdiagnosis and delayed diagnosis of moyamoya disease are particular pitfalls in the treatment of patients with this disorder. Misdiagnosis can occur easily if the physician does not incorporate moyamoya disease into the differential diagnosis of any patient presenting with stroke. […] If moyamoya disease is not considered seriously, then appropriate diagnostic tests may not be performed and a delay in diagnosis could result. […] If an ischemic stroke progresses despite preventative treatment with antiplatelet agents or anticoagulants, then moyamoya disease should be considered as a possible etiology. […] Cerebral angiography is the criterion standard for the diagnosis of moyamoya disease. The following findings support the diagnosis: Stenosis or occlusion at the terminal portion of the internal carotid artery or the proximal portion of the anterior or middle cerebral arteries. […] Magnetic resonance angiography (MRA) or computed tomography angiography (CTA) can be performed. Any of the above findings on MRA or CTA may preclude the need for conventional angiography.

• #22 Moyamoya Disease Worldwide-Global Burden East and West | IntechOpen

  https://www.intechopen.com/chapters/75556

  The Suzuki staging system is most widely used for evaluation and staging of Moyamoya disease. The gold standard diagnostic modality is cerebral angiography but magnetic resonance imaging [MRA] has also been employed for diagnosis. […] The disease diagnosis often rests upon angiography, and revascularization procedures are performed to avoid recurrence of symptoms. […] Angiography is the gold standard for diagnosis and assessing disease progression. The hallmark findings of cerebral angiography are occlusion of intracranial internal carotid arteries and abnormal smog-like arteriolar network [moyamoya vessels] at the base of the brain. […] Both the staging systems highlight that with the progression of the disease, the contribution of blood supply from ICA decreases and an intricate collateral network is formed which derives its blood flow from vessels outside the cerebral circulation.

• #23 Moyamoya Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17244-moyamoya-disease

  A healthcare provider will perform a physical examination and ask you about your symptoms. If they suspect moyamoya disease, you may need the following tests: […] Cerebral arteriography: Your provider inserts a small tube called a catheter into an artery in your arm or leg. They then use it to inject iodine contrast dye into your bloodstream. Next, they take X-ray scans of the dye in your blood vessels. This technique can reveal how much your blood vessels have narrowed and map blood flow patterns. […] Magnetic resonance angiography (MRA): In this pain-free test, your provider uses a magnetic field, radio waves and a computer to look at your blood vessels. […] Magnetic resonance imaging (MRI): MRI uses similar technology to take pictures inside your body and can help assess the effect of moyamoya disease on your brain.

• #24 Get Moyamoya Disease Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/moyamoya-disease-treatment

  An MRA is a type of MRI that focuses on your blood vessels. Your providers may use an MRA to check how well blood is flowing to your brain and to see if anything looks unusual. Our advanced MRA imaging techniques allow us to visualize the underlying process happening in your brain blood vessels. […] During cerebral angiography, a radiologist will insert a small tube (catheter) into an artery in your arm or leg so they can inject a special contrast dye (iodine) into your bloodstream. They then use X-rays to create a map of how your blood flows through your blood vessels and show any narrowing. This minimally invasive test can also show us moyamoya diseases characteristic backup blood vessels, which look like a puff of smoke. In fact, moyamoya means puff of smoke in Japanese.

• #25 Cerebral Angiogram to Diagnose Moyamoya | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/moyamoya/diagnosis/cerebral-angiogram.html

  This is the definitive test that confirms the diagnosis of moyamoya. „Contrast” (or dye) is injected into arteries to reveal the anatomy of the arteries of the brain and scalp. This is a minimally invasive test that requires patients to stay at the hospital for several hours. This test assesses the severity of moyamoya and its results guide treatment options, which are determined by how severe the disease is and what the external (scalp) blood supply is. […] Angiogram of a patient with moyamoya disease. You can see the MCA and ACA vessels are impaired and moyamoya vessels have appeared along with other collateral vessels.

• #26 Epidemiology, diagnosis and treatment of moyamoya disease (Review)

  https://www.spandidos-publications.com/10.3892/etm.2019.7198

  In the 2012 guidelines, cerebral angiography remains the gold standard for the diagnosis of MMD with the staging performed according to angiographic findings. […] According to the new guidelines, differential diagnoses of MMD, which should be excluded, are the following underlying cerebrovascular diseases: Atherosclerosis, autoimmune diseases, meningitis, brain tumors, Down syndrome, Recklinghausen’s disease, head injury and cerebrovascular damage after head irradiation. […] The clinical symptoms of MMD include transient ischemic attacks, ischemic stroke, hemorrhagic stroke, epilepsy, headache and cognitive dysfunction, with the incidence of each symptom varying depending on the age of the patient. […] MMD has two major symptoms: Cerebral ischemia and cerebral hemorrhage. […] Cerebral angiography is the gold standard for diagnosing MMD and assessing its progression.

• #27 Diagnostic Criteria for Moyamoya Disease – 2021 Revised Version

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9357455/

  Moyamoya disease can be diagnosed when all of the following findings are found on MRI and MRA (time-of-flight; TOF) using a scanner with a static magnetic field strength of 1.5 Tesla (T) or higher (3.0 T is even more useful). […] Moyamoya disease is diagnosed when (1) and (2) of A-1 or (1) to (3) of A-2 are met and B is excluded. […] In the 2021 version of the diagnostic criteria, therefore, we decided to clearly state as follows: Especially in the case of unilateral lesions or lesions complicated by atherosclerosis, it is essential to perform cerebral angiography to exclude other diseases. Thus, the 2021 version of the diagnostic criteria emphasizes the importance of cerebral angiography more than ever. […] In fact, it is well known that the diagnosis of moyamoya disease in the early stage of the disease is not easy by MRA alone. In early-stage cases where the above three findings cannot be obtained by MRI and MRA, we recommend definitive diagnosis by cerebral angiography. […] Quasi-moyamoya disease is also often referred to as moyamoya syndrome. […] The revised criteria cover all aspects of the disease, including a definition of the disease concept, diagnostic imaging, and the concept of quasi-moyamoya disease (moyamoya syndrome).

• #28 Moyamoya disease: Diagnostic imaging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3389911/

  Moyamoya disease is a progressive vasculopathy leading to stenosis of the main intracranial arteries. […] Currently, magnetic resonance angiography and CT angiography with multi-row systems are the main imaging methods of diagnostics of the entire range of vascular changes in moyamoya disease. […] An appropriate qualification to surgery should be based on a comprehensive angiographic and imaging evaluation of brain structures. […] Angiographic criteria of the diagnosis of moyamoya disease were established in 1998. They include stenosis or occlusion of the distal parts of intracranial internal carotid arteries and proximal parts of anterior and middle arteries, as well as the presence of collateral vasculature in the regions of the brain base, without causal disease. […] In ambiguous cases, it is indicated to carry out MRI, which not only helps in establishing the diagnosis, but also allows for a better evaluation of the range and time phase of ischaemic lesions.

• #29 Moyamoya disease: Diagnostic imaging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3389911/

  Before modern angiographic examinations (such as CTA or MRA) were introduced to a wide clinical practice, the final diagnosis of the vascular changes was based on conventional angiography or digital subtraction angiography (DSA). […] At present, the diagnosis of the whole range of vascular changes in the course of the disease is based mainly on MRA and CTA, using multi-row systems. […] A proper qualification for surgery should base on a comprehensive angiographic and imaging evaluation of brain structures.

• #30 Epidemiology, diagnosis and treatment of moyamoya disease (Review)

  https://www.spandidos-publications.com/10.3892/etm.2019.7198

  Brain MR imaging (MRI) is able to display the brain parenchymal lesions associated with MMD, and MRA is able to reveal abnormalities consistent with cerebral angiography. […] This assessment provides a more objective and realistic indicator for the selection and efficacy assessment of surgery regimens for MMD.

• #31

  https://link.springer.com/article/10.1007/s00234-012-1020-1

  The purpose of this study was to propose new magnetic resonance (MR) criteria of diagnosing moyamoya disease (MMD) from cisternal moyamoya vessels (MMVs) on 3-T magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and compare the diagnostic accuracy of the existing MR criteria and the proposed MR criteria. […] Diagnostic accuracy was 62.5% with the existing MR criteria and 97.5% with the proposed MR criteria. The proposed MR criteria was more sensitive (1.00) than the existing MR criteria (0.45), but less specific (0.95) than the existing MR criteria (1.00). […] The proposed MR criteria using cisternal MMVs showed significantly higher diagnostic accuracy than the existing MR criteria. We believe that our proposed MR criteria will be beneficial for diagnosing MMD.

• #32 Moyamoya Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17244-moyamoya-disease

  A healthcare provider will perform a physical examination and ask you about your symptoms. If they suspect moyamoya disease, you may need the following tests: […] Cerebral arteriography: Your provider inserts a small tube called a catheter into an artery in your arm or leg. They then use it to inject iodine contrast dye into your bloodstream. Next, they take X-ray scans of the dye in your blood vessels. This technique can reveal how much your blood vessels have narrowed and map blood flow patterns. […] Magnetic resonance angiography (MRA): In this pain-free test, your provider uses a magnetic field, radio waves and a computer to look at your blood vessels. […] Magnetic resonance imaging (MRI): MRI uses similar technology to take pictures inside your body and can help assess the effect of moyamoya disease on your brain.

• #33 Moyamoya disease | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/moyamoya-disease-1?lang=us

  Moyamoya disease is an idiopathic, non-inflammatory, non-atherosclerotic progressive vaso-occlusive disease involving the terminal supraclinoid internal carotid arteries and circle of Willis. […] The term moyamoya disease should be reserved for an idiopathic, sometimes familial, condition, which leads to characteristic intracranial vascular changes. […] This article concerns itself with moyamoya disease. For a list of conditions that can mimic moyamoya disease please refer to the article on moyamoya syndrome or the differential diagnosis list at the end of the article. […] In addition to demonstrating the aforementioned arterial stenoses (best seen on MRA) or enlarged perforators (best seen on T2 weighted images), vessel wall imaging can also be helpful, particularly in distinguishing moyamoya disease from moyamoya syndrome from atherosclerotic or vasculitic/inflammatory causes. […] Vessel wall imaging in moyamoya disease typically demonstrates concentric luminal narrowing, none or only minimal enhancement, homogeneous T2 signal, and no outward remodeling (outer wall area of the stenotic segment greater than proximal normal segment; seen in atherosclerosis).

• #34 The contribution of imaging in diagnosis, preoperative assessment, and follow-up of moyamoya disease in: Neurosurgical Focus Volume 26 Issue 4 (2009) Journals

  https://thejns.org/focus/view/journals/neurosurg-focus/26/4/article-pE3.xml

  The aim of this review was to evaluate the imaging tools used in diagnosis and perioperative assessment of moyamoya disease, with particular attention to the last decade. […] Catheter angiography still represents the gold standard for moyamoya diagnosis. […] Angiography might also reveal the presence of aneurysms that may be related to the increased flow load in relation to the normal vessel diameter in these patients. […] According to the guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis, angiography is not mandatory for the diagnosis of moyamoya disease, because MR imaging and MR angiography can show all the findings required for diagnosis. […] The MR imaging should be performed on 1.5-T (or higher) systems, as suggested by the Research Committee for Diagnosis of moyamoya.

• #35 Diagnostic Criteria for Moyamoya Disease – 2021 Revised Version

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9357455/

  Moyamoya disease can be diagnosed when all of the following findings are found on MRI and MRA (time-of-flight; TOF) using a scanner with a static magnetic field strength of 1.5 Tesla (T) or higher (3.0 T is even more useful). […] Moyamoya disease is diagnosed when (1) and (2) of A-1 or (1) to (3) of A-2 are met and B is excluded. […] In the 2021 version of the diagnostic criteria, therefore, we decided to clearly state as follows: Especially in the case of unilateral lesions or lesions complicated by atherosclerosis, it is essential to perform cerebral angiography to exclude other diseases. Thus, the 2021 version of the diagnostic criteria emphasizes the importance of cerebral angiography more than ever. […] In fact, it is well known that the diagnosis of moyamoya disease in the early stage of the disease is not easy by MRA alone. In early-stage cases where the above three findings cannot be obtained by MRI and MRA, we recommend definitive diagnosis by cerebral angiography. […] Quasi-moyamoya disease is also often referred to as moyamoya syndrome. […] The revised criteria cover all aspects of the disease, including a definition of the disease concept, diagnostic imaging, and the concept of quasi-moyamoya disease (moyamoya syndrome).

• #36 The contribution of imaging in diagnosis, preoperative assessment, and follow-up of moyamoya disease in: Neurosurgical Focus Volume 26 Issue 4 (2009) Journals

  https://thejns.org/focus/view/journals/neurosurg-focus/26/4/article-pE3.xml

  Different acquisition techniques are available for MR angiography, including 3D time-of-flight MR angiography, phase-contrast MR angiography, and contrast-enhanced MR angiography. […] This MR angiographic technique presents acceptable sensitivity (98%), specificity (100%), and accuracy (98%). […] Postoperatively, MR angiography has been used for the assessment of bypass filling (with cine-phase MR angiography) and for the assessment of changes in the ECAs in cases of indirect revascularization (with time-of-flight). […] The ultrasonography modality combines both static and dynamic information, and provides supplementary data regarding quantitative flow characteristics (flow velocity), including the ratio between systolic and end-diastolic velocity, flow resistance, and vessel pulsatility.

• #37 Moyamoya disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591

  Computerized tomography scan. Also known as a CT scan, this imaging test uses a series of X-rays to create a detailed image of your brain. A healthcare professional may inject a dye into a blood vessel to highlight blood flow in your arteries and veins. This is called a CT angiogram. This test can’t diagnose early stages of moyamoya disease. But it may help find problems with blood vessels. […] Cerebral angiogram. In a cerebral angiogram, a healthcare professional inserts a long, thin tube called a catheter into a blood vessel in your groin. The healthcare professional then uses X-ray imaging to guide the catheter to your brain and injects dye through the catheter into the blood vessels of your brain. The dye conforms to the shape of the blood vessels to make them more visible under X-ray imaging. […] If necessary, your neurologist may order other tests to rule out other conditions.

• #38 Moyamoya disease | Causes, Symptoms & Treatment | UW Medicine

  https://www.uwmedicine.org/conditions-symptoms/brain-nervous-system/moyamoya

  CT and CTA. A CT scan uses X-rays to take detailed pictures of your brain. Another type of CT scan, called a CT angiogram or CTA, uses a contrast agent to show how well blood flows through the arteries in your brain. […] MRI and MRA. An MRI scan uses magnetic energy instead of radiation to take pictures of your brain. If your provider needs to see inside the blood vessels, you may have a type of MRI called a magnetic resonance angiogram, or MRA. During an MRA, you may need an IV with liquid contrast agent. […] Single-photon emission computerized tomography (SPECT). A SPECT scan is a type of nuclear imaging test. We give you a small, safe amount of radioactive material we track using special cameras. With SPECT, we can see which areas of your brain aren’t receiving normal amounts of oxygen.

• #39 Moyamoya Disease Diagnosis [Neurosurgery Education Wiki]

  https://neurosurgery.education/wiki/doku.php?id=moyamoya_disease_diagnosis

  Diagnosis of Moyamoya disease requires bilateral symmetrical stenosis or occlusion of the terminal portion of the internal carotid arterys (ICA)s as well as the presence of dilated collateral vessels at the base of the brain 1). (If unilateral, the diagnosis is considered questionable, 2) and these cases may progress to bilateral involvement). […] Work-up in suspected cases typically begins with a non-enhanced head CT. Up to 40% of ischemic cases have normal CT. […] In addition to helping to establish the diagnosis, angiography also identifies suitable vessels for revascularization procedures and unearths associated aneurysms. […] Six angiographic stages of MMD are described by Suzuki and Takaku 3) that tend to progress up until adolescence and stabilize by age 20. […] Ultrasound provides a new way to identify stroke in MMD patients. Future prospective cohort studies are needed to verify the clinical value of ultrasound in identifying patients with MMD at high risk of stroke 7). […] This study demonstrated a characteristic metabolic pattern that can distinguish patients with MMD without VCI from those with VCI, namely, hypometabolic lesions in the left hemisphere played a more important role in cognitive decline in patients with MMD 8).

• #40 Moyamoya disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591

  Computerized tomography scan. Also known as a CT scan, this imaging test uses a series of X-rays to create a detailed image of your brain. A healthcare professional may inject a dye into a blood vessel to highlight blood flow in your arteries and veins. This is called a CT angiogram. This test can’t diagnose early stages of moyamoya disease. But it may help find problems with blood vessels. […] Cerebral angiogram. In a cerebral angiogram, a healthcare professional inserts a long, thin tube called a catheter into a blood vessel in your groin. The healthcare professional then uses X-ray imaging to guide the catheter to your brain and injects dye through the catheter into the blood vessels of your brain. The dye conforms to the shape of the blood vessels to make them more visible under X-ray imaging. […] If necessary, your neurologist may order other tests to rule out other conditions.

• #41 Imaging of the Diagnosis, Work-Up, and Treatment of Moyamoya Vasc…: Ingenta Connect

  https://www.ingentaconnect.com/content/10.3174/ng.1900046

  Moyamoya disease is a complex phenomenon with many potential etiologies and can present with a variety of clinical manifestations. Imaging plays a crucial role in the diagnosis of Moyamoya disease, and the radiologist may be the first to suggest the disease. Either invasive or noninvasive angiography can be used to establish the diagnosis, based on defined radiologic criteria. […] Once the diagnosis is established, cerebral perfusion imaging before and after a vasodilatory challenge can be used to assess cerebrovascular reserve. Noninvasive imaging plays a key role in the evaluation of patients following surgical revascularization to assess the effectiveness of surgery as well as for complications. Noncontrast CT or MR imaging can be used to evaluate for early postoperative complications. CTA can be used to assess the patency of bypass grafts. Perfusion imaging can be performed to assess the degree of improvement in cerebral perfusion. […] Learning Objectives: Describe the imaging appearance of Moyamoya disease, identify typical postoperative findings after surgical revascularization, and recognize the complications of Moyamoya disease and its treatments.

• #42 Moyamoya disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591

  Moyamoya disease is usually diagnosed by a doctor who specializes in conditions of the brain, called a neurologist. The neurologist reviews your symptoms and your family and medical history. The neurologist also may perform a physical exam. Several tests are generally needed to diagnose moyamoya disease and any underlying conditions. […] Tests may include: […] Magnetic resonance imaging. This imaging test also is known as an MRI. An MRI uses powerful magnets and radio waves to create detailed images of the brain. A healthcare professional may inject a dye into a blood vessel to view your arteries and veins and highlight blood circulation. This type of test is called a magnetic resonance angiogram. […] Your neurologist may recommend a perfusion MRI. This type of imaging can measure the amount of blood passing through the vessels. It can show if there is a decrease in blood supply to the brain.

• #43 Moyamoya Syndrome Diagnosis & Treatment – NYC | Columbia Neurosurgery in New York City

  https://www.neurosurgery.columbia.edu/patient-care/conditions/moyamoya-syndrome

  Moyamoya is a rare condition, and physicians who do not specialize in cerebrovascular disorders sometimes do not consider it as a diagnosis. […] Computed tomography (CT) and magnetic resonance imaging (MRI) scans may provide the first indications of the disease. […] If the disease is suspected, cerebral angiography is conducted to establish the diagnosis. For this test, a catheter is inserted in a large blood vessel in the groin and threaded up to the blood supply of the brain. A special dye visible on X-ray is injected into the blood vessels, and a series of X-rays are taken. In this way, a specialist can study the blood flow in the brain. Magnetic resonance angiography (MRA), another imaging study that shows the blood vessels in the brain, also may be used. […] In addition, single photon emission computerized tomography (SPECT) scans may be used to identify the regions of the brain that are not receiving sufficient oxygen.

• #44 Moyamoya disease | Causes, Symptoms & Treatment | UW Medicine

  https://www.uwmedicine.org/conditions-symptoms/brain-nervous-system/moyamoya

  CT and CTA. A CT scan uses X-rays to take detailed pictures of your brain. Another type of CT scan, called a CT angiogram or CTA, uses a contrast agent to show how well blood flows through the arteries in your brain. […] MRI and MRA. An MRI scan uses magnetic energy instead of radiation to take pictures of your brain. If your provider needs to see inside the blood vessels, you may have a type of MRI called a magnetic resonance angiogram, or MRA. During an MRA, you may need an IV with liquid contrast agent. […] Single-photon emission computerized tomography (SPECT). A SPECT scan is a type of nuclear imaging test. We give you a small, safe amount of radioactive material we track using special cameras. With SPECT, we can see which areas of your brain aren’t receiving normal amounts of oxygen.

• #45 Moyamoya Disease Diagnosis [Neurosurgery Education Wiki]

  https://neurosurgery.education/wiki/doku.php?id=moyamoya_disease_diagnosis

  Diagnosis of Moyamoya disease requires bilateral symmetrical stenosis or occlusion of the terminal portion of the internal carotid arterys (ICA)s as well as the presence of dilated collateral vessels at the base of the brain 1). (If unilateral, the diagnosis is considered questionable, 2) and these cases may progress to bilateral involvement). […] Work-up in suspected cases typically begins with a non-enhanced head CT. Up to 40% of ischemic cases have normal CT. […] In addition to helping to establish the diagnosis, angiography also identifies suitable vessels for revascularization procedures and unearths associated aneurysms. […] Six angiographic stages of MMD are described by Suzuki and Takaku 3) that tend to progress up until adolescence and stabilize by age 20. […] Ultrasound provides a new way to identify stroke in MMD patients. Future prospective cohort studies are needed to verify the clinical value of ultrasound in identifying patients with MMD at high risk of stroke 7). […] This study demonstrated a characteristic metabolic pattern that can distinguish patients with MMD without VCI from those with VCI, namely, hypometabolic lesions in the left hemisphere played a more important role in cognitive decline in patients with MMD 8).

• #46 Moyamoya disease: Causes, symptoms, diagnosis and treatments | Health – Hindustan Times

  https://www.hindustantimes.com/lifestyle/health/moyamoya-disease-causes-symptoms-diagnosis-and-treatments-101675228288905.html

  Dr BK Misra said, MRI Brain with MR Angio of brain vessels is usually diagnostic. Further evaluation can be done by perfusion studies by CT, MRI or PET scan. […] Dr VR Roopesh Kumar elaborated, The diagnosis of MMD is based on anatomical and functional aspects which include qualitative and non-qualitative imaging studies. Non-qualitative imaging techniques include different angiographies such as DSA, CT angiography (CTA), and magnetic resonance angiography (MRA). Digital subtraction angiography (DSA) continues to be the gold standard to confirm the diagnosis and in showing the dynamic vascular changes and excellent for disease staging as the Suzuki classification. […] He added, Qualitative imaging techniques include Single-photon emission CT, PET, xenon-enhanced computed tomography (Xe-CT), perfusion CT, dynamic susceptibility contrast (DSC), and arterial spin labelling (ASL) MRI, Acetazolamide challenged ASL Perfusion studies and quantitative DSA (QDSA). CT and MR perfusion studies are easier to perform, practical and economical than SPECT/PET or Xe-CT. Acetazolamide-challenged ASL quantifies augmentation of cerebrovascular reserve (CVR), and this serves as a predictor for improvement after revascularization surgeries. The use of transcranial Doppler (TCD) can assess the mean arterial velocity and resistance index before and after surgery which is another method for ascertaining effectiveness of revascularization.

• #47 Moyamoya Disease | Baylor Medicine

  https://www.bcm.edu/healthcare/specialties/neurosurgery/cerebrovascular-and-stroke-surgery/moyamoya-disease

  If moyamoya disease is suspected, it is important to make a diagnosis and consult with a specialist quickly to prevent worsening of the disease. […] Based on the patients symptoms, the physician may order one or all of these tests. All of these tests are often needed before making a decision about surgery. […] Magnetic resonance imaging (MRI) […] Cerebral angiogram […] Perfusion scans (MRI or CT perfusion) […] Neuropsychological assessment.

• #48 Moyamoya Disease | University of Michigan Health

  https://www.uofmhealth.org/conditions-treatments/brain-neurological-conditions/moyamoya-disease

  Early recognition and evaluation of symptoms by an experienced team is critical for an accurate diagnosis and treatment of moyamoya disease. […] Advanced imaging techniques may be performed based on a patients symptoms and history. These may include: MRI: Uses a magnetic field and radiofrequency waves to create a detailed picture of the brain and blood vessels. […] Cerebral angiogram: Uses an X-ray and contrast dye administered by catheter to produce an image of blood vessels of the head and neck. […] Computed tomography (CT) scan: An X-ray imaging technique to view blood vessels in the brain. […] Electroencephalogram (EEG): A test used to evaluate electrical activity in the brain that can help diagnosis seizures or a likelihood to have seizures. […] Transcranial doppler ultrasound: A test that uses sound waves to detect issues with blood flow in the brain.

• #49

  https://www.aurorahealthcare.org/services/neuroscience/stroke-care/stroke-cerebrovascular-conditions/moyamoya-disease

  First, youll meet with your doctor for a physical exam and to discuss your potential moyamoya disease symptoms and family history. Your doctor may also order one of the following tests: […] Angiogram: A special dye is injected into the bloodstream, which offers a better view of the arteries. When combined with an MRI, the test is called magnetic resonance angiogram (MRA); when combined with a CT scan, its a computed tomographic angiogram (CTA). […] Electroencephalography (EEG): Moyamoya disease often causes a distinctive brainwave pattern in children. This test measures the electrical activity in the brain. […] Positron emission topography (PET): A radioactive dye is injected into the body and then monitored as it makes its way through the blood vessels. […] Transcranial Doppler ultrasound (TCD): High-frequency sound waves show the direction and speed of blood flow.

• #50 Moyamoya disease: Causes, symptoms, diagnosis and treatments | Health – Hindustan Times

  https://www.hindustantimes.com/lifestyle/health/moyamoya-disease-causes-symptoms-diagnosis-and-treatments-101675228288905.html

  Dr BK Misra said, MRI Brain with MR Angio of brain vessels is usually diagnostic. Further evaluation can be done by perfusion studies by CT, MRI or PET scan. […] Dr VR Roopesh Kumar elaborated, The diagnosis of MMD is based on anatomical and functional aspects which include qualitative and non-qualitative imaging studies. Non-qualitative imaging techniques include different angiographies such as DSA, CT angiography (CTA), and magnetic resonance angiography (MRA). Digital subtraction angiography (DSA) continues to be the gold standard to confirm the diagnosis and in showing the dynamic vascular changes and excellent for disease staging as the Suzuki classification. […] He added, Qualitative imaging techniques include Single-photon emission CT, PET, xenon-enhanced computed tomography (Xe-CT), perfusion CT, dynamic susceptibility contrast (DSC), and arterial spin labelling (ASL) MRI, Acetazolamide challenged ASL Perfusion studies and quantitative DSA (QDSA). CT and MR perfusion studies are easier to perform, practical and economical than SPECT/PET or Xe-CT. Acetazolamide-challenged ASL quantifies augmentation of cerebrovascular reserve (CVR), and this serves as a predictor for improvement after revascularization surgeries. The use of transcranial Doppler (TCD) can assess the mean arterial velocity and resistance index before and after surgery which is another method for ascertaining effectiveness of revascularization.

• #51 Moyamoya Disease Diagnosis [Neurosurgery Education Wiki]

  https://neurosurgery.education/wiki/doku.php?id=moyamoya_disease_diagnosis

  Diagnosis of Moyamoya disease requires bilateral symmetrical stenosis or occlusion of the terminal portion of the internal carotid arterys (ICA)s as well as the presence of dilated collateral vessels at the base of the brain 1). (If unilateral, the diagnosis is considered questionable, 2) and these cases may progress to bilateral involvement). […] Work-up in suspected cases typically begins with a non-enhanced head CT. Up to 40% of ischemic cases have normal CT. […] In addition to helping to establish the diagnosis, angiography also identifies suitable vessels for revascularization procedures and unearths associated aneurysms. […] Six angiographic stages of MMD are described by Suzuki and Takaku 3) that tend to progress up until adolescence and stabilize by age 20. […] Ultrasound provides a new way to identify stroke in MMD patients. Future prospective cohort studies are needed to verify the clinical value of ultrasound in identifying patients with MMD at high risk of stroke 7). […] This study demonstrated a characteristic metabolic pattern that can distinguish patients with MMD without VCI from those with VCI, namely, hypometabolic lesions in the left hemisphere played a more important role in cognitive decline in patients with MMD 8).

• #52

  https://www.aurorahealthcare.org/services/neuroscience/stroke-care/stroke-cerebrovascular-conditions/moyamoya-disease

  First, youll meet with your doctor for a physical exam and to discuss your potential moyamoya disease symptoms and family history. Your doctor may also order one of the following tests: […] Angiogram: A special dye is injected into the bloodstream, which offers a better view of the arteries. When combined with an MRI, the test is called magnetic resonance angiogram (MRA); when combined with a CT scan, its a computed tomographic angiogram (CTA). […] Electroencephalography (EEG): Moyamoya disease often causes a distinctive brainwave pattern in children. This test measures the electrical activity in the brain. […] Positron emission topography (PET): A radioactive dye is injected into the body and then monitored as it makes its way through the blood vessels. […] Transcranial Doppler ultrasound (TCD): High-frequency sound waves show the direction and speed of blood flow.

• #53 Moyamoya Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17244-moyamoya-disease

  Moyamoya disease is progressive. That means it will gradually get worse without treatment. Healthcare providers use the Suzuki stages to describe the severity of the disease. They’ll use the MRA test to help determine what stage of the disease you’re in. […] Moyamoya disease treatment involves managing your symptoms with certain medications. Your healthcare provider may recommend or prescribe medications like: […] Aspirin: Aspirin can help prevent or reduce blood clots in the smaller, backup blood vessels that develop. […] Antiseizure medications: These medications can prevent seizures that moyamoya disease causes. […] Anticoagulants: Anticoagulants can thin your blood to prevent blood clots. But these drugs have risks, like possible bleeding that’s difficult to stop. Your provider will only prescribe them in certain cases.

• #54 Moyamoya Disease Diagnosis [Neurosurgery Education Wiki]

  https://neurosurgery.education/wiki/doku.php?id=moyamoya_disease_diagnosis

  Diagnosis of Moyamoya disease requires bilateral symmetrical stenosis or occlusion of the terminal portion of the internal carotid arterys (ICA)s as well as the presence of dilated collateral vessels at the base of the brain 1). (If unilateral, the diagnosis is considered questionable, 2) and these cases may progress to bilateral involvement). […] Work-up in suspected cases typically begins with a non-enhanced head CT. Up to 40% of ischemic cases have normal CT. […] In addition to helping to establish the diagnosis, angiography also identifies suitable vessels for revascularization procedures and unearths associated aneurysms. […] Six angiographic stages of MMD are described by Suzuki and Takaku 3) that tend to progress up until adolescence and stabilize by age 20. […] Ultrasound provides a new way to identify stroke in MMD patients. Future prospective cohort studies are needed to verify the clinical value of ultrasound in identifying patients with MMD at high risk of stroke 7). […] This study demonstrated a characteristic metabolic pattern that can distinguish patients with MMD without VCI from those with VCI, namely, hypometabolic lesions in the left hemisphere played a more important role in cognitive decline in patients with MMD 8).

• #55 Moyamoya disease – Wikipedia

  https://en.wikipedia.org/wiki/Moyamoya_disease

  Cerebral angiography is the gold standard of diagnosing moyamoya disease and its progression. According to Suzuki’s system, it can be classified into six stages: […] Magnetic resonance angiography (MRA) is also useful in diagnosing the disease with good correlation with Suzuki’s grading system. […] Often nuclear medicine studies such as SPECT (single photon emission computerized tomography) are used to demonstrate the decreased blood and oxygen supply to areas of the brain involved with moyamoya disease. Conventional cerebral angiography provides the conclusive diagnosis of moyamoya disease in most cases and should be performed before any surgical considerations. […] There is also research that has shown that certain radiographic biomarkers that lead to the diagnosis of moyamoya disease have been identified. The specific radiographic markers are now considered an acceptable key component to moyamoya disease and have been added to the International Classification of Diseases (ICD). […] If this is not the case and the findings are unilateral, it is diagnosed as moyamoya syndrome. […] The occlusion of the ICA results in concomitant diminution of the „puff-of-smoke” collaterals, as they are supplied by the ICA.

• #56 Moyamoya Disease Worldwide-Global Burden East and West | IntechOpen

  https://www.intechopen.com/chapters/75556

  The Suzuki staging system is most widely used for evaluation and staging of Moyamoya disease. The gold standard diagnostic modality is cerebral angiography but magnetic resonance imaging [MRA] has also been employed for diagnosis. […] The disease diagnosis often rests upon angiography, and revascularization procedures are performed to avoid recurrence of symptoms. […] Angiography is the gold standard for diagnosis and assessing disease progression. The hallmark findings of cerebral angiography are occlusion of intracranial internal carotid arteries and abnormal smog-like arteriolar network [moyamoya vessels] at the base of the brain. […] Both the staging systems highlight that with the progression of the disease, the contribution of blood supply from ICA decreases and an intricate collateral network is formed which derives its blood flow from vessels outside the cerebral circulation.

• #57 Moyamoya Diagnosis | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/moyamoya/diagnosis.html

  Magnetic resonance imaging (MRI) […] Cerebral angiogram […] MRI/Nova […] Xenon CT scans […] Based on the patients’ symptoms and history, the physician may order one or all of these tests before making a decision about treatment. […] Neuropsychological assessment As part of the presurgical diagnostic process, an evaluation of mental abilities (such as memory) is performed. These abilities can be affected by moyamoya disease. The evaluation, which consists of paper and pencil tests and questions, provides a baseline and provides the team information about how the brain is functioning.

• #58 Moyamoya Disease | Baylor Medicine

  https://www.bcm.edu/healthcare/specialties/neurosurgery/cerebrovascular-and-stroke-surgery/moyamoya-disease

  If moyamoya disease is suspected, it is important to make a diagnosis and consult with a specialist quickly to prevent worsening of the disease. […] Based on the patients symptoms, the physician may order one or all of these tests. All of these tests are often needed before making a decision about surgery. […] Magnetic resonance imaging (MRI) […] Cerebral angiogram […] Perfusion scans (MRI or CT perfusion) […] Neuropsychological assessment.

• #59 Moyamoya disease: Diagnostic imaging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3389911/

  Before modern angiographic examinations (such as CTA or MRA) were introduced to a wide clinical practice, the final diagnosis of the vascular changes was based on conventional angiography or digital subtraction angiography (DSA). […] At present, the diagnosis of the whole range of vascular changes in the course of the disease is based mainly on MRA and CTA, using multi-row systems. […] A proper qualification for surgery should base on a comprehensive angiographic and imaging evaluation of brain structures.

• #60 Imaging of the Diagnosis, Work-Up, and Treatment of Moyamoya Vasc…: Ingenta Connect

  https://www.ingentaconnect.com/content/10.3174/ng.1900046

  Moyamoya disease is a complex phenomenon with many potential etiologies and can present with a variety of clinical manifestations. Imaging plays a crucial role in the diagnosis of Moyamoya disease, and the radiologist may be the first to suggest the disease. Either invasive or noninvasive angiography can be used to establish the diagnosis, based on defined radiologic criteria. […] Once the diagnosis is established, cerebral perfusion imaging before and after a vasodilatory challenge can be used to assess cerebrovascular reserve. Noninvasive imaging plays a key role in the evaluation of patients following surgical revascularization to assess the effectiveness of surgery as well as for complications. Noncontrast CT or MR imaging can be used to evaluate for early postoperative complications. CTA can be used to assess the patency of bypass grafts. Perfusion imaging can be performed to assess the degree of improvement in cerebral perfusion. […] Learning Objectives: Describe the imaging appearance of Moyamoya disease, identify typical postoperative findings after surgical revascularization, and recognize the complications of Moyamoya disease and its treatments.

• #61 Moyamoya Diagnosis | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/moyamoya/diagnosis.html

  Magnetic resonance imaging (MRI) […] Cerebral angiogram […] MRI/Nova […] Xenon CT scans […] Based on the patients’ symptoms and history, the physician may order one or all of these tests before making a decision about treatment. […] Neuropsychological assessment As part of the presurgical diagnostic process, an evaluation of mental abilities (such as memory) is performed. These abilities can be affected by moyamoya disease. The evaluation, which consists of paper and pencil tests and questions, provides a baseline and provides the team information about how the brain is functioning.

• #62 Moyamoya Disease | Peter O’Donnell Jr. Brain Institute | Condition | UT Southwestern Medical Center

  https://utswmed.org/conditions-treatments/moyamoya-disease/

  While imaging studies usually reveal MMD/MMS, additional diagnostic tools include: […] Medical and family history […] Neuropsychological assessment: Examining a patients memory and ability to think and speak helps physicians determine any cognitive changes the patient might be experiencing as a result of decreased blood flow. Physicians also use this assessment as a baseline of cognitive abilities to compare with those of a second assessment after surgical treatment.

• #63 Moyamoya Disease | Peter O’Donnell Jr. Brain Institute | Condition | UT Southwestern Medical Center

  https://utswmed.org/conditions-treatments/moyamoya-disease/

  While imaging studies usually reveal MMD/MMS, additional diagnostic tools include: […] Medical and family history […] Neuropsychological assessment: Examining a patients memory and ability to think and speak helps physicians determine any cognitive changes the patient might be experiencing as a result of decreased blood flow. Physicians also use this assessment as a baseline of cognitive abilities to compare with those of a second assessment after surgical treatment.

• #64 Orphanet: Moyamoya disease

  https://www.orpha.net/en/disease/detail/2573

  Moyamoya disease (MMD) is a rare intracranial arteriopathy involving progressive stenosis of the cerebral vasculature located at the base of the brain causing transient ischemic attacks or strokes. […] The diagnosis of MMD may be difficult because it is rare and because of the non-characteristic signs and symptoms. Diagnosis is suspected on the basis of the clinical presentation and imaging findings. Cerebral angiography is the standard confirmatory diagnostic method. Moyamoya disease involves in particular the supraclinoid internal carotid arteries and their proximal branches. The term moyamoya means „puff of smoke” in Japanese, in reference to the appearance of abnormal vascular collateral networks on angiography that develop adjacent to the stenotic vessels. Magnetic resonance angiography (MRA) can also be used.

• #65 Moyamoya Disease Workup: Approach Considerations, Laboratory Studies, Angiography

  https://emedicine.medscape.com/article/1180952-workup

  Misdiagnosis and delayed diagnosis of moyamoya disease are particular pitfalls in the treatment of patients with this disorder. Misdiagnosis can occur easily if the physician does not incorporate moyamoya disease into the differential diagnosis of any patient presenting with stroke. […] If moyamoya disease is not considered seriously, then appropriate diagnostic tests may not be performed and a delay in diagnosis could result. […] If an ischemic stroke progresses despite preventative treatment with antiplatelet agents or anticoagulants, then moyamoya disease should be considered as a possible etiology. […] Cerebral angiography is the criterion standard for the diagnosis of moyamoya disease. The following findings support the diagnosis: Stenosis or occlusion at the terminal portion of the internal carotid artery or the proximal portion of the anterior or middle cerebral arteries. […] Magnetic resonance angiography (MRA) or computed tomography angiography (CTA) can be performed. Any of the above findings on MRA or CTA may preclude the need for conventional angiography.

• #66 Moyamoya Syndrome Diagnosis & Treatment – NYC | Columbia Neurosurgery in New York City

  https://www.neurosurgery.columbia.edu/patient-care/conditions/moyamoya-syndrome

  Moyamoya is a rare condition, and physicians who do not specialize in cerebrovascular disorders sometimes do not consider it as a diagnosis. […] Computed tomography (CT) and magnetic resonance imaging (MRI) scans may provide the first indications of the disease. […] If the disease is suspected, cerebral angiography is conducted to establish the diagnosis. For this test, a catheter is inserted in a large blood vessel in the groin and threaded up to the blood supply of the brain. A special dye visible on X-ray is injected into the blood vessels, and a series of X-rays are taken. In this way, a specialist can study the blood flow in the brain. Magnetic resonance angiography (MRA), another imaging study that shows the blood vessels in the brain, also may be used. […] In addition, single photon emission computerized tomography (SPECT) scans may be used to identify the regions of the brain that are not receiving sufficient oxygen.

• #67 Epidemiology, diagnosis and treatment of moyamoya disease (Review)

  https://www.spandidos-publications.com/10.3892/etm.2019.7198

  In the 2012 guidelines, cerebral angiography remains the gold standard for the diagnosis of MMD with the staging performed according to angiographic findings. […] According to the new guidelines, differential diagnoses of MMD, which should be excluded, are the following underlying cerebrovascular diseases: Atherosclerosis, autoimmune diseases, meningitis, brain tumors, Down syndrome, Recklinghausen’s disease, head injury and cerebrovascular damage after head irradiation. […] The clinical symptoms of MMD include transient ischemic attacks, ischemic stroke, hemorrhagic stroke, epilepsy, headache and cognitive dysfunction, with the incidence of each symptom varying depending on the age of the patient. […] MMD has two major symptoms: Cerebral ischemia and cerebral hemorrhage. […] Cerebral angiography is the gold standard for diagnosing MMD and assessing its progression.

• #68 Diagnostic Criteria for Moyamoya Disease – 2021 Revised Version

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9357455/

  Moyamoya disease can be diagnosed when all of the following findings are found on MRI and MRA (time-of-flight; TOF) using a scanner with a static magnetic field strength of 1.5 Tesla (T) or higher (3.0 T is even more useful). […] Moyamoya disease is diagnosed when (1) and (2) of A-1 or (1) to (3) of A-2 are met and B is excluded. […] In the 2021 version of the diagnostic criteria, therefore, we decided to clearly state as follows: Especially in the case of unilateral lesions or lesions complicated by atherosclerosis, it is essential to perform cerebral angiography to exclude other diseases. Thus, the 2021 version of the diagnostic criteria emphasizes the importance of cerebral angiography more than ever. […] In fact, it is well known that the diagnosis of moyamoya disease in the early stage of the disease is not easy by MRA alone. In early-stage cases where the above three findings cannot be obtained by MRI and MRA, we recommend definitive diagnosis by cerebral angiography. […] Quasi-moyamoya disease is also often referred to as moyamoya syndrome. […] The revised criteria cover all aspects of the disease, including a definition of the disease concept, diagnostic imaging, and the concept of quasi-moyamoya disease (moyamoya syndrome).

• #69 Moyamoya disease: Diagnostic imaging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3389911/

  Moyamoya disease is a progressive vasculopathy leading to stenosis of the main intracranial arteries. […] Currently, magnetic resonance angiography and CT angiography with multi-row systems are the main imaging methods of diagnostics of the entire range of vascular changes in moyamoya disease. […] An appropriate qualification to surgery should be based on a comprehensive angiographic and imaging evaluation of brain structures. […] Angiographic criteria of the diagnosis of moyamoya disease were established in 1998. They include stenosis or occlusion of the distal parts of intracranial internal carotid arteries and proximal parts of anterior and middle arteries, as well as the presence of collateral vasculature in the regions of the brain base, without causal disease. […] In ambiguous cases, it is indicated to carry out MRI, which not only helps in establishing the diagnosis, but also allows for a better evaluation of the range and time phase of ischaemic lesions.

• #70 Steinberg Lab Research: Moyamoya | Gary K. Steinberg Lab | Stanford Medicine

  https://med.stanford.edu/content/sm/steinberg-lab/research/moyamoya.html

  Moyamoya Disease (MMD) is characterized by the uni- or bi-lateral occlusion of primarily the internal carotid artery, with subsequent development of fragile, abnormal collateral vessels. Diagnosis is difficult and usually occurs following clinical presentation of hemorrhagic or ischemic stroke, seizures, migraines or developmental issues. […] Our high throughput genetic and epigenetic studies aim to screen for genetic changes that underlie the Moyamoya disease, which will help identify novel candidates for diagnosis of moyamoya disease. […] In collaboration with Dr. Minnie Sarwal (now at the California Pacific Medical Center), we have identified 165 novel autoantibodies present in the serum of patients with MMD compared to control patients. We are continuing this research to further explore why these autoantibodies are being expressed, and if these results can be translated into potential diagnostics. […] The Stanford Moyamoya Center is currently recruiting healthy participants for a research study on Moyamoya disease. This study aims to identify the genetic and molecular basis of Moyamoya disease.

• #71 Steinberg Lab Research: Moyamoya | Gary K. Steinberg Lab | Stanford Medicine

  https://med.stanford.edu/content/sm/steinberg-lab/research/moyamoya.html/

  Moyamoya Disease (MMD) is characterized by the uni- or bi-lateral occlusion of primarily the internal carotid artery, with subsequent development of fragile, abnormal collateral vessels. Diagnosis is difficult and usually occurs following clinical presentation of hemorrhagic or ischemic stroke, seizures, migraines or developmental issues. […] Our high throughput genetic and epigenetic studies aim to screen for genetic changes that underlie the Moyamoya disease, which will help identify novel candidates for diagnosis of moyamoya disease. […] We are continuing this research to further explore why these autoantibodies are being expressed, and if these results can be translated into potential diagnostics.

• #72

  https://link.springer.com/article/10.1007/s00234-012-1020-1

  The purpose of this study was to propose new magnetic resonance (MR) criteria of diagnosing moyamoya disease (MMD) from cisternal moyamoya vessels (MMVs) on 3-T magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and compare the diagnostic accuracy of the existing MR criteria and the proposed MR criteria. […] Diagnostic accuracy was 62.5% with the existing MR criteria and 97.5% with the proposed MR criteria. The proposed MR criteria was more sensitive (1.00) than the existing MR criteria (0.45), but less specific (0.95) than the existing MR criteria (1.00). […] The proposed MR criteria using cisternal MMVs showed significantly higher diagnostic accuracy than the existing MR criteria. We believe that our proposed MR criteria will be beneficial for diagnosing MMD.

• #73 Moyamoya Disease | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/m/moyamoya-disease.html

  Moyamoya disease is most commonly diagnosed in children, but it can affect adults as well. […] Because moyamoya disease can cause severe brain damage or even death, it is important to diagnose and treat the condition as quickly as possible. Diagnosis of the condition generally begins with a physical exam and a review of the patient’s medical history. The patient’s physician may recommend one or more of the following tests or image diagnostic tools in order to more clearly understand the patient’s condition. […] These imaging tools can assist the physician in finding the blockage and the tangle of blood vessels, as well as see how much damage may have been done by the condition.

• #74 Moyamoya Disease – Child Neurology Foundation

  https://www.childneurologyfoundation.org/disorder/moyamoya-disease/

  Moyamoya disease is a common cause of stroke in children. […] It is important that children with moyamoya disease are diagnosed early. […] Moyamoya is diagnosed using pictures of a child’s brain and blood vessels. The most common way to get these pictures is with MRI, or magnetic resonance imaging. However, additional pictures may be needed. […] A procedure called a cerebral angiogram can confirm a moyamoya diagnosis. A cerebral angiogram can also show how severe the condition is. It can help plan for surgery. […] Most children with moyamoya do not need blood tests. But blood testing may be needed for other reasons, including medication monitoring, genetic testing, surgical planning, and in rare cases when other causes are suspected.

• #75 Moyamoya Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17244-moyamoya-disease

  Moyamoya disease is progressive. That means it will gradually get worse without treatment. Healthcare providers use the Suzuki stages to describe the severity of the disease. They’ll use the MRA test to help determine what stage of the disease you’re in. […] Moyamoya disease treatment involves managing your symptoms with certain medications. Your healthcare provider may recommend or prescribe medications like: […] Aspirin: Aspirin can help prevent or reduce blood clots in the smaller, backup blood vessels that develop. […] Antiseizure medications: These medications can prevent seizures that moyamoya disease causes. […] Anticoagulants: Anticoagulants can thin your blood to prevent blood clots. But these drugs have risks, like possible bleeding that’s difficult to stop. Your provider will only prescribe them in certain cases.

• #76 Moyamoya Disease | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/moyamoya-disease

  The standard test for both diagnosing moyamoya and planning for surgery is a cerebral angiogram. This is a minimally invasive procedure that produces a “map” of your child’s carotid arteries. […] Moymoya is treated with surgery to fix the narrowed arteries in the brain and decrease the risk of stroke. While surgery is the only viable treatment for moyamoya disease in the long term, your doctor may also recommend medication to manage some of your child’s symptoms. These may include aspirin (to help prevent blood from clotting) and calcium channel blockers, such as verapamil (to help lower blood pressure). […] Several surgical treatments for moyamoya are effective at bypassing narrowed arteries and creating a new blood supply for the affected areas of the brain.

• #77 Moyamoya Disease | Penn State Health

  https://www.pennstatehealth.org/services-treatments/moyamoya-disease

  The treatment of moyamoya and intracranial stenosis begins with radiological studies to confirm the diagnosis and plan for treatment. […] The surgical treatment for moyamoya is cerebral revascularization with direct cerebral bypass. […] We offer comprehensive and leading-edge treatments for patients with moyamoya disease, other occlusive cerebrovascular conditions, and complex aneurysm and vascular malformation. […] These studies help our scientists improve diagnostic techniques, develop better treatments, and collaborate with other researchers.

• #78 Moyamoya disease: Diagnostic imaging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3389911/

  Before modern angiographic examinations (such as CTA or MRA) were introduced to a wide clinical practice, the final diagnosis of the vascular changes was based on conventional angiography or digital subtraction angiography (DSA). […] At present, the diagnosis of the whole range of vascular changes in the course of the disease is based mainly on MRA and CTA, using multi-row systems. […] A proper qualification for surgery should base on a comprehensive angiographic and imaging evaluation of brain structures.

• #79 Imaging of the Diagnosis, Work-Up, and Treatment of Moyamoya Vasc…: Ingenta Connect

  https://www.ingentaconnect.com/content/10.3174/ng.1900046

  Moyamoya disease is a complex phenomenon with many potential etiologies and can present with a variety of clinical manifestations. Imaging plays a crucial role in the diagnosis of Moyamoya disease, and the radiologist may be the first to suggest the disease. Either invasive or noninvasive angiography can be used to establish the diagnosis, based on defined radiologic criteria. […] Once the diagnosis is established, cerebral perfusion imaging before and after a vasodilatory challenge can be used to assess cerebrovascular reserve. Noninvasive imaging plays a key role in the evaluation of patients following surgical revascularization to assess the effectiveness of surgery as well as for complications. Noncontrast CT or MR imaging can be used to evaluate for early postoperative complications. CTA can be used to assess the patency of bypass grafts. Perfusion imaging can be performed to assess the degree of improvement in cerebral perfusion. […] Learning Objectives: Describe the imaging appearance of Moyamoya disease, identify typical postoperative findings after surgical revascularization, and recognize the complications of Moyamoya disease and its treatments.

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