What is Moyamoya disease and why does it occur?
Moyamoya angiopathy is a rare, progressive cerebrovascular disease characterized by the narrowing or complete occlusion of the terminal portions of the internal carotid arteries [3], [4]. In response to chronic ischemia, the body attempts to compensate for the lack of blood supply by forming a network of fragile collateral vessels at the base of the brain [3], [4]. On angiograms, this specific network resembles puffs of smoke, which gave the condition its name ("moyamoya" translates from Japanese as "puff of smoke"). The exact causes are not fully understood, but studies point to an important role for genetic and autoimmune factors, including associations with certain HLA alleles and concomitant thyroid disorders such as Graves' disease [5], [8].
Main symptoms and clinical manifestations
The clinical presentation of the disease largely depends on the patient's age and the degree of hemodynamic impairment. The most frequent manifestations are transient ischemic attacks and ischemic strokes, which can recur even with standard drug therapy [4]. In addition, patients frequently experience headaches, ranging in intensity and character from migraine-like to tension-type attacks, which are associated with microvascular ischemia and impaired autoregulation [3].
It is important to note that pathological changes in this disease affect not only the intracranial vasculature, but also systemic vessels. Recent scientific data show a tendency toward coronary artery involvement with the risk of acute myocardial infarction due to fibrous intimal thickening and a propensity for spasms [1].
Systemic complications and therapeutic challenges
The diagnosis and treatment of Moyamoya disease require a comprehensive multidisciplinary approach. Modern imaging techniques, such as perfusion computed tomography and digital subtraction angiography, play a key role in assessing hemodynamic disorders and planning surgical interventions (such as creating arterial bypasses) [4].
Of particular complexity is the concomitant cardiovascular pathology. Standard approaches to treating coronary artery disease, including the use of statins and antiplatelet agents, may prove insufficiently effective for stabilizing inflamed plaques in such patients [2]. This underscores the need to develop specialized management protocols for patients taking into account the systemic nature of the vasculopathy [1].
Modern treatment approaches
The therapeutic strategy for Moyamoya angiopathy aims to prevent recurrent ischemic events and improve cerebral perfusion [4]. It includes:
- Blood pressure control and correction of risk factors [4].
- Administration of antiplatelet therapy under the strict supervision of specialists [4].
- Surgical revascularization (bypass surgery) to restore normal blood flow in ischemic zones [4].
- Monitoring of concomitant endocrine and autoimmune disorders (e.g., thyroid pathology) [5], [8].
Sources
- 1. ST-Elevation Myocardial Infarction in a Young Man With Moyamoya Disease. JACC. Case reports, 2026
- 2. Effect of Standard Drug Treatment on Coronary Artery Disease Secondary to Moyamoya Disease. Catheterization and cardiovascular interventions, 2025
- 3. The Spectrum of Headaches in Moyamoya Angiopathy: From Mechanisms to Management Strategies. European journal of neurology, 2025
- 4. Adult-onset moyamoya disease presenting with recurrent ischemic stroke. Radiology case reports, 2026
- 5. Clinical features of moyamoya disease with Graves' disease: a retrospective study. Endocrine journal, 2023
- 8. Identification of HLA-DRB1*04:10 allele as risk allele for Japanese moyamoya disease. PloS one, 2019
Disclaimer: The information presented in this article is for informational purposes only and does not replace an in-person consultation with a qualified physician. If symptoms of the disease appear, you must consult a specialist.