Multiple progressive intracranial arterial occlusions ('moyamoya' disease)

Area of Science:

• Neurology
• Vascular Medicine
• Radiology

Background:

• Moyamoya disease is a rare cerebrovascular disorder characterized by progressive stenosis of the internal carotid arteries.
• The presence of abnormal vascular networks, termed moyamoya vessels, is a hallmark of the condition.
• Associated risk factors such as hypertension and hyperlipidaemia are frequently observed.

Purpose of the Study:

• To describe clinical findings in adult female patients with moyamoya vessels.
• To highlight the importance of investigating the aetiology of moyamoya vessel formation.
• To explore potential therapeutic avenues, including corticosteroids and risk factor management.

Main Methods:

• Case series describing four adult female patients.
• Clinical assessment including evaluation for hypertension and hyperlipidaemia.
• Vascular imaging to identify moyamoya vessels in specific brain regions (orbital, cortical, basal ganglia).

Main Results:

• Four adult female patients presented with moyamoya vessels.
• Hypertension and hyperlipidaemia were identified in three of the four patients.
• Moyamoya-like vessels were observed in orbital, cortical, and basal ganglia collateral networks.

Conclusions:

• The findings underscore the need to shift focus from descriptive to aetiological aspects of moyamoya vessels.
• An empirical trial of corticosteroids may be beneficial.
• Aggressive management of risk factors including hypertension, hyperlipidaemia, and smoking is crucial.