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Smooth muscle cells of intracranial vessels: from development to disease
Juhana Frösen1,2, Anne Joutel3,4
1Hemorrhagic Brain Pathology Research Group, NeuroCenter, Kuopio University Hospital, Kuopio 70029, Finland.
Insights
Cerebrovascular diseases like stroke cause significant suffering and economic burden. This review explores how intracranial vessel development, structure, and function relate to brain arteriovenous malformations, aneurysms, and small-vessel diseases.
Area of Science:
- Neurology
- Vascular Biology
- Pathology
Background:
- Cerebrovascular diseases, including ischemic and hemorrhagic strokes, are leading causes of death and disability.
- Intracranial vascular pathologies such as brain arteriovenous malformations, saccular aneurysms, and cerebral small-vessel diseases contribute significantly to stroke incidence and outcomes.
- These conditions impose a substantial human and economic burden globally.
Purpose of the Study:
- To review the developmental, structural, and functional characteristics of intracranial vessels.
- To elucidate the role of smooth muscle cells in the pathogenesis of key cerebrovascular diseases.
- To explore the relationship between unique intracranial vessel properties and disease development.
Main Methods:
- Literature review of developmental biology, vascular structure, and function.
- Analysis of smooth muscle cell involvement in specific intracranial vascular pathologies.
- Synthesis of current knowledge linking vessel ontogeny and structure to disease mechanisms.
Main Results:
- Brain arteriovenous malformations are common causes of intracranial hemorrhage in younger populations.
- Saccular intracranial aneurysms pose risks in middle-aged and older adults, with rupture carrying a poor prognosis.
- Cerebral small-vessel disease is implicated in the majority of intracerebral hemorrhages and a significant proportion of ischemic strokes and dementias in the elderly.
Conclusions:
- The unique developmental, structural, and functional aspects of intracranial vessels are intrinsically linked to the pathogenesis of major cerebrovascular diseases.
- Understanding these relationships is crucial for developing targeted therapeutic strategies.
- Smooth muscle cell behavior is a key factor in the development of these debilitating conditions.
Abstract:
Cerebrovascular diseases that cause ischaemic or haemorrhagic stroke with subsequent loss of life or functional capacity due to damage of the brain tissue are among the leading causes of human suffering and economic burden inflicted by diseases in the developed world. Diseases affecting intracranial vessels are significant contributors to ischaemic and haemorrhagic strokes. Brain arteriovenous malformations, which are a collection of abnormal blood vessels connecting arteries to veins, are the most common cause of intracranial haemorrhage in children and young adults. Saccular intracranial aneurysms, which are pathological saccular dilations mainly occurring at bifurcations of the large intracranial arteries near the circle of Willis, are highly prevalent in the middle-aged population, causing significant anxiety and concern; their rupture, although rare, is a significant cause of intracranial haemorrhage in those past middle age that is associated with a very sinister prognosis. Cerebral small-vessel disease, which comprise all pathological processes affecting vessels <500 microns in diameter, account for the majority of intracerebral haemorrhages and ∼25% of ischaemic strokes and 45% of dementias in the elderly. In this review, we summarize the developmental, structural, and functional features of intracranial vessels. We then describe the role of smooth muscle cells in brain arteriovenous malformations, intracranial aneurysms, and small-vessel diseases, and discuss how the peculiar ontogeny, structure, and function of intracranial vessels are related to the development of these diseases.
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