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Hemoglobin SC Sickle Cell Disease Presenting as Acute Ischemic Stroke With Moyamoya Syndrome in an Adult: A Case
Tejashree Nidiginti1, Basil Afzal1, Reshmi Roy1
1General Internal Medicine, Calderdale and Huddersfield NHS Foundation Trust, Halifax, GBR.
Abstract:
Moyamoya disease is a rare condition where blood vessels at the base of the brain become narrow or blocked. This leads to the growth of small, fragile vessels that look like a "puff of smoke" on imaging tests like CT or MRI scans. These vascular changes predominantly involve the terminal portion of the internal carotid arteries and the proximal segments of the anterior and middle cerebral arteries, leading to progressive stenosis and the development of collateral networks. Although the exact cause of moyamoya disease is unknown, it increases the risk of having a stroke. In this disease, there is partial or complete occlusion or narrowing in the terminal part of the carotid artery, seen in arterial imaging. When these intracerebral vessel changes happen along with other conditions, such as autoimmune diseases, vasculitis, hematological conditions like sickle cell anemia, renal problems like polycystic kidney disease, renal artery narrowing, and any metabolic causes, it is called moyamoya syndrome (MMS). Our case report describes how we diagnosed and managed a 26-year-old male from Nigeria with MMS.
Insights
Moyamoya syndrome (MMS) involves brain vessel narrowing and stroke risk, often linked to other conditions. This case report details the diagnosis and management of MMS in a young Nigerian male.
Area of Science:
- Neurology
- Vascular Medicine
- Radiology
Background:
- Moyamoya disease is a rare cerebrovascular condition characterized by progressive stenosis of the internal carotid arteries and their branches.
- This stenosis leads to the formation of fragile collateral vessels, often described as a 'puff of smoke' on imaging.
- Moyamoya syndrome (MMS) occurs when these vascular changes are associated with other systemic conditions.
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