Moyamoya disease is a sporadic cerebrovascular malady categorized by progressive stenosis of the internal carotid arteries and successive development of friable collateral vessels in the brain. The primary root of Moyamoya disease remains undefined, with a genetic predisposition playing an essential role. Familial cases show autosomal dominant inheritance patterns, Chained to particular genetic mutations. Genetic factors cooperating with environmental factors add to disease development. The moyamoya disease mostly occurs in East Asian countries like Korea and Japan. It has global impact. Its occurrence percentage shows demographic variations, with a greater prevalence among females and diverse age-related occurrence in childhood and adulthood. These patterns show genetic and environmental factors' interconnected linkage. Clinical Manifestations show transient, ischemic strokes, ischemic attacks and intracranial hemorrhages. Clinical signs vary between children and adults, with the later more susceptible to strokes. Cognitive impairment and motor discrepancies also arise due to compromised cerebral perfusion. Diagnosis depends on clinical assessment, neuroimaging, and angiography. Imaging techniques like Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) scans identify cerebral ischemic or hemorrhagic events. Cerebral angiography confirms the diagnosis by revealing a characteristic collateral vessel formation resembling a "puff of smoke." Treatment management includes revascularization procedures, such as direct or indirect bypass surgeries, to improve cerebral blood flow. Medical therapies, including antiplatelet agents, aim to reduce thrombotic risks. Continuing follow-up is crucial to calculate treatment-efficacy and disease progression.
Primary Language | English |
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Subjects | Neurology and Neuromuscular Diseases |
Journal Section | Review Articles |
Authors | |
Publication Date | March 29, 2024 |
Submission Date | August 25, 2023 |
Acceptance Date | February 25, 2024 |
Published in Issue | Year 2024 Volume: 41 Issue: 1 |
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