Moyamoya Disease

Original Editor - User:Wendy Walker

Top Contributors - Wendy Walker

Clinically Relevant Anatomy

Moyamoya Disease is a disorder of some of the blood vessels in the brain: the internal carotid arteries, and the arteries which branch from them. It is a rare cause of Stroke and TIAs.

The image on the left: MIP reconstructed MR angiography of a 11 year old girl with Moyamoya Disease. The image on the right: a healthy subject for comparison.

It can also be referred to as Progressive Intracranial Occlusive Arteropathy, or Progressive Intracranial Arterial Occlusion.

Mechanism of Injury / Pathological Process

The affected arteries become increasingly constricted with time, and a collateral circulation develops around the constricted arteries to compensate for the blockages. The condition is an idiopathic, non-inflammatory, non-atherosclerotic progressive vaso-occlusive disease involving the terminal supraclinoid internal carotid arteries and circle of Willis.

Clinical Presentation

The patient often presents either:

• in early childhood, aged around 5 years (two-thirds of patients) OR
• in adults in their 30s or 40s (one-third of patients)

In children, hemispheric ischaemic strokes are the most common presentation; in adults haemorrhage from the abnormal vessels is more common.

Stroke or Transient Ischaemic Attack are the most common presentations, although some patients present with headaches or seizures.

Outcome Measures

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Management / Interventions

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Differential Diagnosis

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Resources

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