Fact-sheet: Cerebral microangiopathy
- Chronic white matter ischemia
- Cerebral small vessel disease (CSVD)
Updated on 10/14/2026 at 11:39 AM
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Definition
Cerebral small vessel disease is an umbrella term encompassing brain lesions attributed to pathological changes affecting small arteries, arterioles, capillaries, venules, or small veins.
It is characterized by structural changes: tortuous vessel walls, reduced caliber and flow, enlarged Virchow-Robin spaces, and white matter demyelination.
The most common cause of vascular dementia (Binswanger disease) and a major cause of both ischemic and hemorrhagic strokes.
Clinical features
- Asymptomatic: Often an incidental finding on imaging.
- Cognitive impairment: A major cause of cognitive decline and vascular dementia (with a reported prevalence of 100% in some dementia studies).
- Binswanger disease: A term used to describe a progressive clinical syndrome characterized by cognitive impairment associated with compatible neuroimaging findings (diffuse white matter involvement).
- Risks: Stroke and all-cause mortality.
Laboratory findings
Cardiovascular risk factors accelerate the progression of lesions: arterial hypertension, diabetes mellitus, hypercholesterolemia, and smoking.
Ultrasound
Doppler ultrasound: used for associated macrovascular assessment
CT
Less sensitive than MRI
.- Non-enhancing white matter hypodensities (leukoaraiosis), predominantly involving the periventricular regions around the atria of the lateral ventricles and the subcortical white matter.
- Ischemic lacunes: Focal hypodensities that are more pronounced than the areas of diffuse demyelination.
MRI
Imaging modality of choice. The STRIVE-2 recommendations advocate the following MRI sequences: T1-weighted, T2-weighted, T2-FLAIR, diffusion-weighted imaging (DWI), GRE or SWI, and time-of-flight MR angiography (TOF-MRA).
- White matter hyperintensities on T2-weighted and T2-FLAIR images, appearing iso- or hypointense on T1-weighted images (leukoaraiosis).
- Lacunes: Fluid-filled cavities measuring 3–15 mm in diameter. On T2-FLAIR images, lacunes appear hypointense (CSF-like signal intensity), sometimes with a peripheral hyperintense rim, distinguishing them from areas of demyelination, which remain hyperintense on T2-FLAIR images.
- Enlarged perivascular spaces: Visible on T2-FLAIR images as hypointense lacune-like structures, particularly in the insular region and lentiform nucleus.
- Cerebral microbleeds: Detected on susceptibility-sensitive sequences (GRE/SWI), predominantly involving the basal ganglia, suggesting an underlying hypertensive etiology.
- Cerebral atrophy (cortical and subcortical).
The coexistence of leukoaraiosis and cerebral microbleeds is an adverse prognostic factor for the occurrence of intracerebral hemorrhage in hypertensive patients.
Classification
Fazekas scale (or Fazekas and Schmidt scale): Assesses periventricular and deep white matter lesions.Periventri
- cular white matter hyperintensities:0 = Absent
- 1 = Pencil
- -thin lining or caps2 = Smooth
- periventricular halo3 = Irregu
- lar periventricular hyperintensities extending into the deep white matterDeep white
- matter hyperintensities:0 = Absent
- 1 = Puncta
- te hyperintense foci2 = Beginn
- ing confluence of hyperintensities3 = Large
- confluent areas of hyperintensityWahlund sc
ale: Grading of white matter and basal ganglia signal abnormalities associated with aging and vascular risk factors.Le texte transmis ne contient pas de balises HTML visibles. J'ai conservé sa structure et sa numérotation, sans ajouter de balises.
- White matter lesions
- Grade 0: No signal abnormalities
- Grade 1: Focal signal abnormalities (hyperintense on FLAIR and T2-weighted images)
- Grade 2: Early confluence of lesions
- Grade 3: Diffuse involvement
- Basal ganglia lesions
- Grade 0: No signal abnormalities
- Grade 1: A single focal lesion > 5 mm
- Grade 2: More than one focal lesion
- Grade 3: Confluent lesions
Differential diagnosis
- CADASIL
- Cerebral amyloid angiopathy: Cortical-subcortical microbleeds, cortical superficial siderosis, and convexity subarachnoid hemorrhage are suggestive of cerebral amyloid angiopathy.
- Ependymitis granularis: Juxtaventricular changes (within 3 mm of the ventricular wall) related to cerebrospinal fluid (CSF) leakage rather than cerebral small vessel disease.
- Other causes: Central nervous system (CNS) vasculitis, radiation-induced vasculopathy, and genetic disorders (Fabry disease, MELAS syndrome).
- Non-vascular causes: White matter hyperintensities may also reflect inflammatory disorders, such as multiple sclerosis, or toxic leukoencephalopathy.
References
- Fazekas F, Chawluk JB, Alavi A, Hurtig HI, Zimmerman RA. MR signal abnormalities at 1.5 T in Alzheimer's dementia and normal aging. AJR Am J Roentgenol. 1987 Aug;149(2):351-6. doi: 10.2214/ajr.149.2.351. PMID: 3496763.
