Moyamoya disease and syndrome: a review

Abstract

Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.

Unusual presentation of acquired thrombotic thrombocytopenic purpura (TTP) versus catastrophic antiphospholipid syndrome in a patient with Moya‐Moya disease, case report, and literature review

“…It is characterized by chronic progressive stenosis of the terminal portion of the bilateral internal carotid arteries or the proximal portion of the anterior and/or middle cerebral arteries, which leads to the formation of an abnormal vascular network composed of collateral pathways at the base of the brain. 1 Moyamoya is the Japanese term for a “puff of smoke,” used to describe the appearance of these collateral vessels on cerebral angiograms. 2 Patients with moyamoya spectrum vasculopathy have been associated with multiple autoimmune diseases, such as systemic lupus erythematosus, autoimmune thyroid disease, antiphospholipid syndrome, and congenital TTP.…”

“…Moyamoya disease is a cerebrovascular condition that predisposes affected patients to stroke. It is characterized by chronic progressive stenosis of the terminal portion of the bilateral internal carotid arteries or the proximal portion of the anterior and/or middle cerebral arteries, which leads to the formation of an abnormal vascular network composed of collateral pathways at the base of the brain 1 . Moyamoya is the Japanese term for a “puff of smoke,” used to describe the appearance of these collateral vessels on cerebral angiograms 2 .…”

White and Gray Matter Perfusion in Children with Moyamoya Angiopathy after Revascularization Surgery

“…As previously shown, brain perfusion parameters improve after surgery in MMA patients, which subsequently leads to clinical improvement, especially in children [15]. There are multiple papers in the field that focus exclusively on gray matter perfusion alterations before and after surgical revascularization [16]. On the other hand, white matter perfusion changes remain underestimated.…”

A case of acute ischemic stroke in moyamoya syndrome associated with Graves’ disease: Is there a role for anti-dsDNA antibodies?

“…Sumaira Rafique 1 , Koutaiba Rida Obaid 2 , Islam Ahmed Hassan 3 From 1 Consultant, 2 Resident, Department of Medicine, 3 Consultant Radiologist, Department of Radiology, Hamad General Hospital, Doha, Qatar M oyamoya disease (MMD) is a disorder characterized by bilateral stenosis or occlusion of the terminal internal carotid artery (ICA) and proximal portion of the anterior and middle cerebral arteries and the formation of collaterals called moyamoya vessels to compensate for the steno-occlusion [1]. The term MMD is reserved for cases in which the intracranial vascular changes are primary and not associated with any known condition, while the term Moyamoya syndrome (MMS) is used for intracranial vascular changes that occur in association with other conditions, such as postcranial radiotherapy, neurofibromatosis Type 1, Down syndrome, and sickle cell anemia [1,2]. The diagnosis of both MMD and MMS is based on characteristic smoky appearance on angiography like a puff of smoke in the air [1,2].…”

“…The term MMD is reserved for cases in which the intracranial vascular changes are primary and not associated with any known condition, while the term Moyamoya syndrome (MMS) is used for intracranial vascular changes that occur in association with other conditions, such as postcranial radiotherapy, neurofibromatosis Type 1, Down syndrome, and sickle cell anemia [1,2]. The diagnosis of both MMD and MMS is based on characteristic smoky appearance on angiography like a puff of smoke in the air [1,2].…”