Although moyamoya disease may occur by itself in a previously healthy individual, many disease states have been reported in association with moyamoya disease, including the following:
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Immunologic -Graves disease/thyrotoxicosis [7]
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Infections - Leptospirosis and tuberculosis
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Hematologic disorders - Aplastic anemia, Fanconi anemia, sickle cell anemia, and lupus anticoagulant
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Congenital syndromes - Apert syndrome, Down syndrome, Marfan syndrome, tuberous sclerosis, Turner syndrome, von Recklinghausen disease (Neurofibromatosis Type 1), and Hirschsprung disease
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Vascular diseases - Atherosclerotic disease, coarctation of the aorta and fibromuscular dysplasia, cranial trauma, radiation injury, parasellar tumors, and hypertension
These associated conditions may not be causative, but they do warrant consideration due to their impact on treatment. In the presence of these risk factors, the condition is referred to as moyamoya syndrome.
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Schematic representation of the circle of Willis, arteries of the brain, and brain stem.
