Moyamoya: An Update and Review.

Area of Science:

• Neurology
• Neurosurgery
• Vascular Neurology

Background:

• Moyamoya disease (MMD) is a rare, progressive stenosis of intracranial arteries, primarily affecting the anterior circulation.
• Moyamoya syndrome (MMS) is a secondary condition with similar angiographic findings caused by underlying pathologies like radiation.
• Both conditions present with a characteristic

Purpose of the Study:

• To provide a comprehensive clinical review of Moyamoya disease and syndrome.
• To discuss the incidence, epidemiology, pathology, and clinical presentation of MMD and MMS.
• To evaluate the effectiveness of various treatment modalities, including medical, interventional, and surgical options.

Main Methods:

• Review of existing literature on Moyamoya disease and syndrome.
• Analysis of diagnostic imaging modalities such as catheter-based diagnostic cerebral angiography (DCA), CT perfusion, and MRI (DWI/ADC).
• Discussion of surgical treatment options, including direct and indirect cerebrovascular bypass.

Main Results:

• MMD is characterized by progressive stenosis of the internal carotid arteries and their branches, leading to compensatory angiogenesis.
• Clinical presentation often includes stroke signs and symptoms due to cerebral ischemia, and movement disorders from collateral vessel proliferation.
• Catheter-based DCA remains the gold standard for diagnosis, while surgical intervention is the generally accepted treatment.

Conclusions:

• Medical and neuro-interventional treatments have shown limited efficacy in halting disease progression for MMD and MMS.
• Surgical revascularization techniques, such as direct and indirect bypass, are the mainstay of treatment for Moyamoya disease and syndrome.
• This review aims to guide clinicians in managing this rare and complex cerebrovascular disorder.