Cerebral Small Vessel Disease
Original Editor - Rahma Ahmed Ahmed Bahbah
Top Contributors - Rahma Ahmed Ahmed Bahbah
Introduction[edit | edit source]
Small vessel disease is a systemic condition affects primarily the brain, the kidney, and the retina as they have a high percentage from cardiac output. So, SVD is a major cause in conditions such as renal failure, blindness, lacunar infarcts, and dementia.[1]
It's a condition -from its name- affect the small vessels such as the small arteries, arterioles, venules, and capillaries. The main target of SVD is the endothelium, as it's the barrier between circulating blood and the vessel wall. Endothelial cells may become damaged when inflammation exits.[1]
Cerebral small vessel disease (CSVD) is an umbrella term that refers to intracranial vascular disease based on various pathological and neurological processes, which lead to manifestations and neuroimaging findings as a result of the structural changes of vascular and brain parenchyma.[2]
CSVD mainly affect grey as well as white matter of the deep structures such as basal ganglia, thalamus,
Subtypes[edit | edit source]
Most commonly, two types are identified: amyloid and non-amyloid related (Table 1).[3]
CSVD can be divided into six groups
○Type I: arteriosclerosis/age-related CSVD
○Type II: amyloid-related CSVD;
○Type III: genetic CSVD distinct from amyloid angiopathy
○Type IV: inflammatory/immunologically mediated CSVD
○Type V: venous collagenosis
○Type VI: other CSVD.
Cerebral small vessel disease (CVSD) classification. AD—Alzheimer’s disease; CADASIL—cerebral autosomal dominant arteriopathy with subcortical ischemic stroke and leukoencephalopathy; CARASIL—cerebral autosomal recessive arteriopathy with subcortical ischemic and leukoencephalopathy; MELAS—mitochondrial encephalopathy with lactic acidosis and stroke-like episodes; CNS—central nervous system; SLE—systematic lupus erythematosus; HIV—human immunodeficiency virus.
| Type: | Description: | Associated Diseases: |
|---|---|---|
| Type I | Arteriosclerosis-related CSVD | • Hypertension |
| • Diabetes | ||
| Type II | Amyloid-related CSVD | • AD |
| • Down’s syndrome | ||
| Type III | Genetic CSVD (distinct from amyloid angiopathy) | • Fabry’s disease |
| • CADASIL | ||
| • CARASIL | ||
| • MELAS | ||
| • Small vessel disease with COL4A1 mutation | ||
| • Retinal vasculopathy with leukodystrophy with TREX1 mutation | ||
| • Hereditary multi-infarct dementia of Swedish type | ||
| Type IV | Inflammatory/immunologically mediated CSVD | • Systematic Vasculitis: |
◦ ○IgA vasculitis
◦ ○Eosinophilic granulomatosis with polyangiitis
◦ ○Granulomatosis with polyangiitis
◦ ○Cryoglobulinemic vasculitis
◦ ○Cutaneous leukocytoclastic
◦ ○Microscopic polyangiitis
Primary Central Nervous System Vasculitis • Vasculitis secondary to CNS infections tuberculosis, syphilis, HIV, leptospirosis • Vasculitis Secondary to Connective Tissue Disorders (SLE, scleroderma, rheumatoid vasculitis, dermatomyositis, Sjogren’s syndrome) | | Type V | Venous collagenosis | | | Type VI | Other CVSD | • Post radiation CVSD • Non-amyloid microvessel degeneration in AD |
Clinical manifestations[edit | edit source]
The main clinical manifestations of CSVD include stroke, cognitive decline, dementia, psychiatric disorders, abnormal gait, and urinary incontinence, as according to the location of the lesion.[1]
Neuroimaging[edit | edit source]
Neuroimaging features of CSVD include recent small subcortical infarcts, lacunes, white matter hyperintensities, perivascular spaces, microbleeds, and brain atrophy.[1]
Whereas lacunar infarction, critical stenosis and hypoperfusion involving multiple small arterioles, mainly in deep white matter, lead to incomplete ischemia which are visualized as White Matter Hyperintensities (WMH) on neuroimaging. So lacunes and white matter lesions often coexist in the same patient.
Inflammatory markers have been associated with periventricular white matter hyperintensities (WMH).
Risk factors[edit | edit source]
The Conventional risk factors are hypertension, diabetes, smoking, obesity, depression and dyslipidemia.
Pathophysiology process[edit | edit source]
The pathophysiologic mechanisms of CSVD are not yet clear. These various pathologic changes cited by the European expert group are damages of brain parenchyma including neuronal apoptosis, diffuse axonal injury, demyelination and loss of oligodendrocytes.
The main target of SVD is the endothelium, as it's the barrier between circulating blood and the vessel wall. Endothelial cells may become damaged when inflammation exits.[1]
Poor sleep is associated with increased systemic inflammation and maybe develop cortical atrophy.
Diabetes as known is a very good environment to develop inflammation
Management[edit | edit source]
References[edit | edit source]
- Antoine M. Hakim , Small Vessel Disease , Volume 10 - 2019 | Frontiers, https://doi.org/10.3389/fneur.2019.01020
- Amita Singh, Gabriel Bonnell, Justin De Prey, Natalie Buchwald, Kyrillos Eskander, Keith J. Kincaid, Christina A. Wilson, Small-vessel disease in the brain ,American Heart Journal Plus: Cardiology Research and Practice https://doi.org/10.1016/j.ahjo.2023.100277.
