Research progress in unilateral moyamoya disease

Area of Science:

• Neurology
• Vascular Medicine
• Radiology

Background:

• Unilateral moyamoya disease (U-MMD) is a progressive stenosis of internal carotid arteries and branches.
• It presents with characteristic moyamoya vessels at the brain base, with unknown etiology.
• U-MMD accounts for 9.7-17.8% of moyamoya disease cases, with a familial incidence of 5.5-13.3%.

Purpose of the Study:

• To review current research on unilateral moyamoya disease (U-MMD).
• To cover etiology, epidemiology, clinical and radiological features, progression, and treatment.
• To highlight U-MMD's potential progression to bilateral disease and associated risk factors.

Main Methods:

• Literature review of recent studies on unilateral moyamoya disease.
• Analysis of epidemiological data, clinical presentations, and radiological findings.
• Evaluation of treatment strategies, focusing on surgical interventions and prognosis.

Main Results:

• U-MMD shares similarities with typical moyamoya disease but has distinct characteristics.
• There is a significant probability (median 29.01%) of U-MMD progressing to bilateral moyamoya disease.
• Surgical treatment shows promise in reducing ischemic stroke incidence and improving outcomes.

Conclusions:

• Further research is needed to clarify the optimal timing and indications for surgical intervention in U-MMD.
• Understanding U-MMD's progression and risk factors is crucial for patient management.
• This review synthesizes current knowledge on U-MMD, guiding future research and clinical practice.