Clinical Management of Moyamoya Patients

Area of Science:

• Neurology
• Vascular Medicine
• Radiology

Background:

• Moyamoya angiopathy (MMA) is a rare cerebrovascular disease causing progressive stenosis of internal carotid arteries and collateral vessel formation.
• It presents as either idiopathic Moyamoya disease (MMD) or Moyamoya Syndrome (MMS) linked to other conditions, with unclear pathophysiology.
• Clinical manifestations include ischemic/hemorrhagic strokes, headaches, seizures, cognitive decline, and movement disorders.

Purpose of the Study:

• To provide a comprehensive overview of Moyamoya angiopathy, encompassing its diagnosis, pathophysiology, clinical features, and current management strategies.
• To highlight the diagnostic role of radiological angiographic techniques.
• To discuss the limitations in understanding the underlying pathophysiology and its impact on patient management.

Main Methods:

• Diagnosis relies on radiological angiographic techniques.
• Treatment strategies are based on observational studies and expert consensus due to a lack of randomized clinical trials.
• Management includes pharmacological (antithrombotics) and surgical interventions.

Main Results:

• Surgical revascularization techniques are the most effective treatment for symptomatic MMA, improving long-term outcomes and reducing stroke risks.
• No current pharmacological treatment can reverse the arterial stenosis.
• Antithrombotic agents are used for ischemic MMA, but the hemorrhagic risk must be considered.

Conclusions:

• Moyamoya angiopathy requires a multidisciplinary approach, integrating pharmacological and surgical options.
• Surgical revascularization is the current gold standard for symptomatic patients.
• Further research is needed to elucidate pathophysiology and develop targeted therapies.