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Moyamoya Disease
Introduction
Moyamoya disease was first described in Japanese literature in 1957; however, Suzuki and Takaku first coined the term “moyamoya disease” in 1969.[1] Moyamoya disease is an isolated chronic, usually bilateral, vasculopathy of undetermined etiology characterized by progressive narrowing of the terminal intracranial portion of the internal carotid artery (ICA) and circle of Willis. Moyamoya syndrome corresponds to the same moyamoya arteriopathy, but in the context of either neurological or extraneurological conditions, whether inherited or acquired. A fragile network of abundant collateral vessels develops in response to chronic brain ischemia, known as moyamoya vessels, which appear as a “puff of smoke” on angiography.[2] Patients most often present with ischemic strokes or transient ischemic attacks (TIAs); however, intracranial hemorrhages are also common due to the fragility of the abnormal moyamoya vessels. Surgical revascularization is the mainstay of treatment for both ischemic and hemorrhagic presentations of moyamoya disease.
Etiology
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Etiology
Moyamoya disease is associated with several inherited and acquired conditions, including:
- Inherited conditions
- Acquired conditions
Epidemiology
Moyamoya disease occurs far more frequently in Asia, a trend reflected in the majority of available epidemiological data. The condition appears most commonly in East Asian countries, particularly Japan and Korea, though an increasing incidence has also been documented in Western nations. In Japan, incidence rates range from 0.35 to 0.94 per 100,000 individuals, with a reported prevalence of 3.16 per 100,000 population.[26][27][28] A positive family history has been identified in 11% to 12% of cases, according to prior studies.[29][27]
A population-based study conducted in California and Washington state involving 298 patients reported an incidence of 0.086 per 100,000 individuals.[30] Another study using data from the Nationwide Inpatient Sample Database found a higher incidence of 0.57 per 100,000 population.[31] This same study revealed a female predominance in the United States, with women accounting for 72% of cases. Moyamoya disease generally follows a bimodal age distribution, with incidence peaks between 5 and 9 years and 45 and 49 years of age.[27] Pediatric patients more frequently present with ischemic symptoms, while adults more commonly exhibit intracranial hemorrhage at onset.[31]
Pathophysiology
The pathophysiology of moyamoya disease remains unclear, though genetic predisposition is theorized in East Asian countries. The RNF213 gene (Ring Finger Protein) is a well-known susceptibility gene that is often found in patients of Japanese and Korean descent.[32] The homozygous variant has been shown to predict an earlier onset and a more severe presentation.[33]
In a study involving the Midwestern United States population, an unusually high prevalence of type 1 diabetes, autoimmune thyroid disorders, and other autoimmune disorders was found in the moyamoya cohort, which may suggest an autoimmune association.[34][35] Similar results were seen in the Western Chinese population, where 31% of patients with moyamoya disease also had an autoimmune disease, mostly diabetes mellitus type 1 and Graves’ disease.[36]
Chronic brain ischemia resulting from narrowing is believed to cause an overexpression of proangiogenic factors, including vascular endothelial growth factor, fibroblast growth factor, and hepatocyte growth factor, among others, which, in turn, promotes the development of a fragile network of collateral vessels.[37] Furthermore, the association between moyamoya disease and inflammatory disease has been studied, with inflammatory disease clusters seen in both pediatric and adult cohorts.[38]
The following types of moyamoya disease with the chromosome involved have been described in the literature:
- MYMY1 (chromosome 3p)
- MYMY2 (RNF213 gene on chromosome 17q25)
- MYMY3 (chromosome 8q23)
- MYMY4 (X-linked recessive condition characterized by moyamoya disease, short stature, hypergonadotropic hypogonadism, and facial dysmorphism)
- MYMY5 (ACTA2 gene on chromosome 10q23) [39]
- MYMY6 with achalasia (GUCY1A3 gene on chromosome 4q32)
Mutations in BRCC3/MTCP1 [40] and GUCY1A3 [41] genes have also been implicated in moyamoya syndrome.
Histopathology
Prior studies have shown that the arteries affected in moyamoya disease have fibrocellular thickening of the intimal layer, which contains smooth muscle cells, as well as duplicated internal elastic lamina.[42][43][44] The tunica media eventually becomes thinner, leading to a decrease in the outer vessel diameter, and is seen in conjunction with luminal stenosis.[45][46]
History and Physical
Cerebral ischemic events in the form of TIA or ischemic infarcts are the most common presentation of all moyamoya patients.[47] Intracerebral hemorrhage occurs more frequently among adult patients with moyamoya disease, while seizures can present in both adults and children. Symptoms can be categorized based on etiology: those due to cerebral ischemia (eg, stroke, TIA, and seizure) and those due to the growth of collateral vessels that compensate for ischemia (eg, hemorrhage and headache).
Cerebral Ischemia-Associated Presentations
In the pediatric population, moyamoya typically manifests with TIA or ischemic stroke. These events are often precipitated by hyperventilation (ie, crying). Hyperventilation decreases carbon dioxide, leading to cerebral vasoconstriction and worsening cerebral hypoperfusion. Children may also show intellectual disabilities of various degrees.[48][49][50] Deterioration of cognition is in a linear relationship with the number of strokes and chronic hypoxemia from a progressive narrowing of the cerebral vasculature.
Collateral Growth-Associated Presentations
In addition to TIA or ischemic strokes, adults also frequently present with hemorrhagic strokes. Hemorrhage primarily results from the rupture of fragile moyamoya collaterals and is typically observed in deep areas of the brain, eg, the basal ganglia and the periventricular deep white matter. Intraventricular hemorrhage is common due to the proximity of the primary site of intracerebral hemorrhage. Seizures are commonly seen after either ischemic or hemorrhagic events.[51]
Migraine-like headaches are common in both children and adults and are thought to occur from the stimulation of dural nociceptors by dilated transdural collaterals.[52][53] Tension-type headaches, hemiplegic migraines, and cluster headaches can also be seen.[19] Recent studies have also shown psychiatric symptoms, eg, depression, anxiety, and psychosis in adult patients with Moyamoya disease.[54][55][56]
Although rare, several reports have described choreoathetosis and other movement disorders as a manifestation of moyamoya disease.[57][58][59] A retrospective review of 316 consecutive patients found that only 3.2% of patients presented with chorea.[60]
Evaluation
Like all neurovascular conditions, the evaluation of moyamoya disease relies on different imaging modalities to help characterize the lesion, guide management strategies, and help predict postoperative recovery. A brief description of various imaging modalities is discussed here; however, a more thorough review was published by Du and colleagues.[61]
Magnetic Resonance Imaging
Magnetic resonance imaging (MRI) typically serves as an initial diagnostic tool due to its high sensitivity and noninvasive nature. MRI effectively detects hemorrhages and strokes, while older ischemic lesions frequently appear as white matter hyperintensities in distal vascular territories or border zone regions on FLAIR and T2-weighted sequences. A notable radiographic feature, the "ivy sign," reflects leptomeningeal enhancement or increased signal intensity on T2-FLAIR and appears in nearly half of pediatric patients with Suzuki stage III or IV disease.[62] This sign likely indicates slow cortical blood flow, and its resolution may serve as a prognostic marker following revascularization.[62] Incorporating vessel wall imaging into MRI protocols may also aid in differentiating Moyamoya disease from atherosclerotic lesions.[63]
Magnetic Resonance Angiography
Magnetic resonance angiography (MRA) provides information on cerebral arteries and the degree of narrowing (see Image. Angiographic Findings in Moyamoya Disease). MRA also demonstrates the development of collaterals (ie, moyamoya vessels). In the 2021 Japanese guidelines, diagnosis can be made if the following are seen on MRI/MRA:
- Stenosis or occlusion of the terminal portion of the intracranial internal carotid artery.
- Decrease in the outer diameter of the terminal portion of the internal carotid artery and the horizontal portion of the middle cerebral artery bilaterally on heavy T2-weighted MRI.
- Abnormal vascular networks in the basal ganglia and periventricular white matter on MRA.[64]
"Quasi-moyamoya disease" (also known as moyamoya syndrome) is a unilateral or bilateral occlusive process associated with an underlying disease.[65]
Conventional cerebral angiography
Based on the 2021 Japanese guidelines, the diagnosis of moyamoya disease can be made with the following criteria:
- Stenosis or occlusion in the arteries centered on the terminal portion of the intracranial internal carotid artery.
- Moyamoya vessels (abnormal vascular networks) are present in the vicinity of the occlusive or stenotic lesions in the arterial phase.[64]
Both bilateral and unilateral cases can be diagnosed as moyamoya disease according to the following 2021 guidelines.[64] Cerebral angiography is considered the gold standard in the diagnosis of moyamoya disease. Suzuki and Kodoma classified the severity of moyamoya disease by progression of an occlusive process and the eventual appearance of collaterals based on serial cerebral angiographic evaluations and staged them, known as "Suzuki stages of moyamoya disease", which are mentioned under staging. Various types of collaterals have been described on angiography, each progressing from extracranial to the intracranial vasculature, including:
- Basal moyamoya: Abnormal dilation of the perforating arteries, such as the lenticulostriates and thalamo-perforating arteries
- Choroidal and pericallosal: Dilatation of the anterior choroidal and posterior pericallosal arteries
- Ethmoidal moyamoya: Collaterals are perfused from the ophthalmic artery and ethmoidal (anterior and posterior) arteries.
- Vault moyamoya: Last pathway in which transdural collaterals supply the pial arteries.[66]
Transcranial Doppler
Transcranial Doppler (TCD) is an adjunctive method for monitoring cerebral hemodynamics, and data on its ability to determine the stage and treatment method are scarce. Additionally, TCD is operator-dependent. Hence, this modality is not as useful as MRI, MRA, or conventional angiography. The primary parameters of monitoring using TCD are mean blood flow velocity and the pulsatility index.[67]
Electroencephalography
Electroencephalography (EEG) evaluations are necessary for patients presenting with seizures. Suzuki and Kodoma mentioned a distinctive EEG finding among ~50% of moyamoya patients, known as the "rebuild-up" phenomenon. The rebuild-up phenomenon is referred to as a reappearance of slow waves of higher amplitude (usually seen during hyperventilation), within 20 to 60 seconds following termination of hyperventilation, which is not seen in any other pathology. Rebuild-up is different than initial slowing due to hyperventilation and signifies the diminished blood flow. The slowdown due to rebuilding is expected to be resolved in approximately 10 minutes.[68]
Cerebral Perfusion Measurement
Important tools for measuring cerebral perfusion include single-photon emission computed tomography (SPECT), xenon-enhanced CT, and MRI-based methods. Xenon-enhanced CT and MRI-based methods include positron emission tomography (PET) scans and arterial spin labeling. Both typically show the following findings:
- Increase in oxygen fraction extraction
- Reduction of global cerebral blood flow with posterior cerebral flow distribution
- Impaired cerebrovascular reactivity to carbon dioxide and acetazolamide in ICA territory, suggesting low cerebrovascular reserve [69]
Treatment / Management
Management Approaches
Early diagnosis of moyamoya disease, coupled with timely surgical intervention, is of utmost importance as medical therapies act only as secondary prevention and do not halt disease progression. In asymptomatic patients, the risk of stroke has to be weighed against the risks of surgery. In a multicenter, nationwide survey in Japan, 40 patients were enrolled in a prospective cohort study, which demonstrated an annual risk of 3.2% of any stroke.[70] In another multicenter prospective cohort study, Kuroda et al found a yearly risk of stroke of 1.4% per person.[71] An interim analysis of the AMORE study revealed a 5.9% incidence of disease progression in asymptomatic patients, suggesting the need for careful decision-making when treating asymptomatic patients.[72] (B3)
In patients who present with ischemia, some studies suggest delaying revascularization, as patients who underwent surgery within 90 days after the last stroke had lower overall postoperative stroke incidence and lower mortality rates.[73] However, other authors suggest performing revascularization within 2 months of diagnosis, especially in patients younger than 4 years of age.[74] In patients presenting with intracranial hemorrhage, studies have demonstrated evidence that surgical revascularization may have a preventative effect against rebleeding, suggesting that surgical intervention is better than nonoperative management.[75] A thorough review of the recommendations from the Japanese guidelines, European Stroke Organization, and American Heart Association can be found in a review by Rifino and colleagues.[76](B3)
Medical Management
Acute therapy for strokes or intracranial bleeding is performed as per standard protocols.[77] Conservative management is directed towards maintaining cerebral blood flow and preventing further ischemic and hemorrhagic events. Aspirin has been conventionally used among patients with moyamoya disease. A meta-analysis evaluated the benefits of antiplatelet therapy and reported a reduced risk of hemorrhagic stroke, but did not decrease the risk of ischemic strokes.[78] The 2021 Japanese guidelines report that oral antiplatelet agents may be considered in patients with ischemic moyamoya disease, although they cite a low level of evidence.[77] Other studies have reported increased rates of bypass patency with perioperative aspirin use, without a significant increase in bleeding risk.[79][80][81](A1)
Headaches and seizures are usually managed by symptomatic treatments using analgesics and antiepileptic medications, respectively.[82]
Surgical Revascularization
Surgery is the mainstay of treatment for moyamoya disease to improve cerebral blood flow and prevent future strokes and hemorrhages. The main surgical approaches to revascularization include indirect, direct, or combined approaches.[83][84](A1)
Indirect revascularization
Indirect revascularization is the more common surgical approach in the pediatric population, as the donor and recipient vessels in children are much smaller than in adults, making a direct bypass technically more challenging. Indirect techniques include encephalomyosynangiosis (EMS), encephaloduroarteriosynangiosis (EDAS), encephalomyoarteriosynangiosis (EMAS), encephaloduroarteriomyosynangiosis (EDAMS), and encephalo-galeosynangiosis (EGS). Other methods include pial synangiosis, dural inversion, multiple burr holes, or omental transposition.
Direct revascularization
The superficial temporal artery is used as the donor vessel to the superficial branches of the MCA. The benefit of direct bypass is the immediate restoration of blood flow to the ischemic regions of the brain.
Perioperative Management
The precise management of patients with moyamoya disease both before and after surgical revascularization plays a vital role in optimizing outcomes for these patients. Because the affected cerebral hemispheres are at risk for ischemic events, special considerations must be made when undergoing anesthesia. The primary risk of undergoing anesthesia is the development of a mismatch between cerebral oxygen demand and supply. Consequently, the goals from an anesthesia standpoint include maintaining normocarbia, normotension, normovolemia, and normothermia.[85]
Expert consensus has recommended the following for pediatric patients undergoing indirect revascularization:
- Children with sickle cell disease should be preadmitted before surgical revascularization.
- Intravenous isotonic fluids should be given for at least 4 hours before and 24 hours after surgery.
- Antiplatelet therapy should not be stopped during the immediate preoperative and postoperative periods.
- Arterial lines should be continued for 24 hours after surgery for continuous blood pressure monitoring and until active interventions for blood pressure control are not needed.
- Hourly vital sign checks for 24 hours and hourly neurological assessments for at least 12 hours after surgery, with special attention to maintaining normoxia and normotherapy and avoiding hypotensive episodes.
- Intravenous fluid boluses should be considered first-line interventions for the treatment of new focal neurological deficits.[86]
A detailed care pathway has been described for pediatric patients undergoing revascularization.[87] Other protocols have been established specifically for perioperative nausea and vomiting in pediatric patients.[88] Another important consideration in the perioperative period is the management of relative anemia. One study analyzing 53 surgeries showed that larger decreases in hemoglobin, hematocrit, and blood urea nitrogen were associated with an increased risk of perioperative ischemic strokes.[89] Beyond assessing vitals and the neurological exam in the perioperative period, continuous EEG has been recommended by some authors to detect early clinical and subclinical ischemia, which may lead to the initiation of treatment before clinical symptoms manifest.[90](B3)
Differential Diagnosis
Differential diagnoses that should be considered when evaluating moyamoya disease include:
- Large vessel occlusion
- Cerebral vasculitis
- Intracranial atherosclerotic disease
- Basilar artery thrombosis
- Blood dyscrasia
- Cavernous sinus syndromes
- Cerebral aneurysms
- Dissection syndromes
- Fibromuscular dystrophies
- Spontaneous intracranial hemorrhage
Staging
The Suzuki staging system describes the progression from the initial stenosis in the terminal portion of the ICA to the formation of a deep but fragile network of collaterals (moyamoya) and the subsequent reduction of moyamoya vessels, accompanied by the development of supply from external carotid artery branches. This fragile network of collaterals mainly develops from thalamoperforating and lenticulostriate perforating arteries. Suzuki stages of moyamoya disease are the following:
- Stage 1
- Narrowing of carotid fork: On the angiographic exam, only the terminal portion of the internal carotid artery is stenosed.
- Stage 2
- Initiation and appearance of basal moyamoya: On the angiographic exam, stenosis of all the terminal branches of ICA (ACA and MCA) and deep moyamoya vessels are seen.
- Stage 3
- Intensification of basal moyamoya: On the angiographic exam, deep moyamoya vessels are intensified. MRA taken during this stage shows a "puff of smoke" appearance.
- The deflection of the anterior cerebral artery (ACA) and middle cerebral arteries (MCA) is noted.
- Stage 4
- Minimization of basal moyamoya: On the angiographic exam, deep moyamoya vessels begin to regress while transdural collaterals begin to appear.
- The deflection of the PCA is noted.
- Stage 5
- Reduction of moyamoya: On the angiographic exam, continued regression of deep moyamoya vessels and progression of transdural collateral vessels are noted.
- Stage 6
- Disappearance of moyamoya: On the angiographic exam, deep moyamoya vessels have vanished, and complete occlusion of the ICA is noted.
- Blood supply to the ACA and MCA areas is derived mainly from the external carotid artery.[2]
Prognosis
The prognosis of moyamoya disease depends on multiple factors. Patients initially presenting with ischemia, those diagnosed before the age of 3, individuals with diabetes mellitus, and those with PCA involvement face an increased risk of recurrent ischemic symptoms.[91] Identified risk factors for recurrent hemorrhages include an initial hemorrhagic presentation, smoking, choroidal collaterals, and decreased cerebrovascular reserve.[91] A large retrospective study from Korea demonstrated a reduced risk of death and recurrent hemorrhagic stroke in adult patients who underwent revascularization compared to those managed conservatively. However, an elevated risk of hemorrhagic stroke was observed in asymptomatic moyamoya disease patients who received revascularization.[92]
In a meta-analysis of pediatric moyamoya patients, revascularization correlated with lower odds of ischemic stroke and intracranial hemorrhage. Similar rates of ischemic stroke, TIAs, new or worsened seizures, and symptomatic improvement were observed when comparing direct or combined techniques to indirect methods. Another meta-analysis found superior long-term angiographic revascularization with direct or combined approaches, particularly when paired with indirect bypasses.[93] However, stroke recurrence, morbidity, and mortality did not significantly differ between techniques.[94] Postoperative monitoring remains essential, as contralateral progression frequently occurs and may require additional treatment.[95][96][97]
Complications
Complications that are associated with moyamoya disease include:
- Perioperative ischemic stroke
- Hemorrhagic stroke
- Hyperperfusion syndrome after direct vascularization
- New or worsening seizures
- Epidural hematoma
- Wound healing issues
Postoperative and Rehabilitation Care
Patients who experience perioperative ischemic or hemorrhagic strokes may require inpatient rehabilitation as they recover.
Consultations
Consultations that may be involved in the management of this condition:
- Neurosurgery
- Neurology
- Interventional radiology
- Genetics
- Rehab
- Physical, occupational, and speech therapies
Deterrence and Patient Education
Although no preventive treatments for moyamoya disease or moyamoya syndrome have been established, early recognition and treatment can improve long-term outcomes. Deterrence and patient education for moyamoya disease focus on early recognition, risk reduction, and proactive management to prevent neurological deterioration. Since no curative treatment exists, educating patients and caregivers about the importance of timely diagnosis and surgical intervention remains crucial.
Clinicians must emphasize adherence to follow-up appointments, imaging surveillance, and medication regimens, particularly antiplatelet therapy when indicated. Patients should receive counseling on modifiable risk factors, including blood pressure control, smoking cessation, weight management, and lipid regulation, to minimize the risk of ischemic and hemorrhagic events. For pediatric patients, families should understand the potential impact of hyperventilation-induced symptoms and the importance of monitoring for cognitive or behavioral changes. A clear explanation of the disease process, treatment options, and long-term expectations empowers patients and supports informed decision-making, fostering greater engagement and adherence to care plans.
Enhancing Healthcare Team Outcomes
Effective management of Moyamoya disease requires seamless collaboration across an interprofessional healthcare team, including physicians, advanced practitioners, nurses, pharmacists, radiologists, anesthesiologists, and surgical specialists. Neurologists and neurosurgeons play central roles in diagnosis, staging, and determining the appropriate surgical intervention, while advanced practitioners and nurses ensure timely follow-up, monitoring, and patient education. Accurate interpretation of advanced imaging modalities, such as MRI, MRA, and cerebral angiography, requires coordination between radiologists and treating clinicians to inform evidence-based decision-making. Pharmacists support optimal use of antiplatelet agents and seizure medications, carefully monitoring for drug interactions, adherence, and perioperative considerations. Effective communication during preoperative and postoperative phases—particularly around anesthetic risks and neurological status—enhances safety and minimizes complications.
Patient-centered care relies on aligning treatment strategies with the patient's age, presentation, and risk profile, while also integrating education on lifestyle modifications, such as smoking cessation, blood pressure control, and stroke prevention. Nurses and allied health professionals provide ongoing support and health coaching, especially for pediatric patients and families coping with neurocognitive or psychosocial challenges. Structured care coordination meetings and standardized care pathways—particularly for perioperative care—optimize team performance and reduce variability in outcomes. By fostering open dialogue, shared goals, and role clarity, interprofessional teams ensure safer interventions, more efficient transitions of care, and improved long-term outcomes for individuals affected by Moyamoya disease.
Media
(Click Image to Enlarge)
Angiographic Findings in Moyamoya Disease. Magnetic resonance angiography (MRA) provides information on cerebral arteries and the degree of narrowing.
Contributed by S Munakomi, MD
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