A case of progressive vasculopathy: angiographic characteristics and moyamoya syndrome

Abstract

Moyamoya Disease and Syndrome are neurological conditions that affect the blood vessels in the brain, characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches, and the consequent network of collateral vessels at the base of the skull whose angiographic pattern simulates a "smoke cloud". Both conditions have a universal distribution, although Moyamoya Disease is more frequently seen in the Asian population. The term "Moyamoya" originates from Japanese, meaning puff of cigarette smoke, reflecting the characteristic cerebral angiogram pattern seen in these conditions. The etiology of both conditions is not yet fully understood. Moyamoya Disease corresponds to isolated vasculopathy, without risk factors for cerebrovascular disease, whereas in Moyamoya Syndrome, there is an underlying pathology. In this case report, the angiographic study and the presence of autoimmune pathology (Graves' Disease) suggest the hypothesis of Moyamoya Syndrome. The natural history of Moyamoya Disease and Syndrome varies, and they may have a slow progression with rare intercurrent events or a fulminant course with rapid neurological decline. In the absence of treatment, the progression of the disease is associated with a high risk of recurrence of neurological events. Therefore, it is crucial to consider these conditions in cases of cerebrovascular diseases.