Overview

According to the NHS, over 180,000 people in the UK are estimated to live with a type of vascular dementia.¹ Binswanger disease (BD) is one of those pathologies characterised by deterioration of the brain's white matter due to a lack of blood supply, resulting in vascular cognitive impairment and dementia (VCID).² More specifically, Binswanger is a type of cerebral small vessel disease (CSVD), where the smaller arteries are damaged. VCID significantly impacts patients' quality of life and life expectancy and puts a heavy burden on their families and other carers. Unfortunately, the diagnosis of Binswanger disease and CSVD can be fairly complex as it shares various characteristics with other dementia and vascular dementia types.² Although symptoms tend to worsen over time, with lifestyle changes, people with BD can act on critical risk factors to stabilise their cognitive decline and overall health.¹

What is binswanger's disease?

What is cerebral small vessel disease?

Binswanger disease (BD), clinically known as subcortical ischaemic vascular dementia, is a type of cerebral small vessel disease (CSVD).³ In those pathologies, the smallest blood vessels (arteries) supplying the brain are deteriorating. Because those are partially (or entirely in some cases) destroyed, it leads to reduced blood flow to the tissue. As the cerebral tissues do not receive the required oxygen and other nutrients, white matter is destroyed in surrounding areas - this is generically referred to as ‘white matter disease’. The final result is that the brain does not function properly, and symptoms of cognitive decline appear, such as confusion and locomotive dysfunction.²

Diagnostic criteria

Symptoms of Binswanger disease appear around 55–65 years old, with the disease progressing steadily.² Preliminary symptoms such as slower cognition are often attributed to the brain's normal ageing. Still, those should not be disregarded as they indicate that the disease mechanism has already started.⁴ Disease-specific symptoms may notably include:

• Executive dysfunction (evaluated via neuropsychological testing), e.g., patients have trouble with short-term memory, managing emotions and actions and volition
• Hyperreflexia (limbs have an increased reflex response) and other gait disturbances
• Urinary urgency or incontinence
• Apathy and depression⁴

It is important to note that Binswanger disease, like any other form of vascular dementia, shares characteristics with other neurodegenerative disorders such as Parkinson's and Alzheimer's, which impedes the straightforward diagnosis of the disease in primary healthcare settings (GP). Other CSVD pathologies can also be present alongside Binswanger Disease, and those are referred to as mixed-type dementias.^2,4

As recently outlined by the vascular impairment of cognition classification consensus study or VICCCS, Binswanger disease has overlapping clinical signs with what is defined as ‘subcortical ischaemic vascular dementia’, or more simply CSVD. Within the classification system, BD and CSVD are only categories within the VCI spectrum.⁵ Indeed, diagnosis is very complicated for physicians to establish solely on symptoms established during clinical assessment and neuropsychological testing. It is solely through the visualisation of cerebral lesions via imaging techniques that a definitive diagnosis of Binswanger disease can be confirmed.^4,6

Imaging techniques for Binswanger disease diagnosis

Gold standard imaging techniques for diagnosing BD and CSVD include computed tomography (CT), but, more importantly, magnetic resonance imaging (MRI), as it allows visualising potential pathological tissues with outstanding clarity and precision. Fluid-attenuated inversion recovery (FLAIR) is a newer MRI technique that allows one to differentiate the brain's grey and white matter clearly.⁷ Typical MRI features for Binswanger Disease include;

• Diffuse white matter lesions (WMLs), which are hyperintense zones of white matter, 
• Scattered lacunar infarcts^6,7

Causes of binswanger's disease and development of the disease (pathophysiology)

As with any pathology, understanding how Binswanger disease first develops is necessary to find treatments that slow its progression.

Pathophysiology

Recent studies indicate that the cause of the death of the brain tissue, more than the generic lack of blood flow to the area, would be due to the ‘hypertensive lipo- and fibro-hyalinosis’ of the small vessels of the brain.⁷ Indeed, the brain, like any organ and tissue of the body, requires constant oxygen and nutrients delivered within the blood supply via arteries. It means that, within the brain, the smallest arteries themselves harden, and the outer capsule loses its integrity and can also accumulate deposits. The lumen (inner circumference of the vessel) is reduced, further reducing blood flow, and the artery may even rupture. What is called the blood-brain barrier is disrupted, allowing leakage of blood content, such as plasma, within the brain.⁸ In the surrounding white matter, a region called a lacuna (because of its usual rounded shape) thus presents signs of infarction (blood deprivation).⁷ Those areas are even visible in medical images such as the ones produced by MRI; hence, WMLs and lacunar artefacts are imaging markers for BD and CSVD.

Causes

Specifically, for Binswanger disease, the development of the disease is progressive rather than sudden. This means rather than a hemorrhagic incident, it is the slow process of ischemia (oxygen restriction) that results in damage to the white matter of the brain.² Although causes for this may be multifactorial, hypertension has been acknowledged as a major contributing factor for the development of hyalinosis of small cerebral arteries.⁷ Hypertension could even cause ‘mini-strokes’ that further damage the integrity of the blood vessels.⁹

Risk factors and treatment

There is no given treatment for vascular dementia. To stabilise the deterioration of the brain tissue, it is suggested that the cause be targeted.⁴ Because the cause of Binswanger disease is the deterioration of the vascular network supplying the brain, risk factors for the disease include common cardiovascular pathologies such as:

• Hypertension 
• Diabete mellitus and pre-diabete 
• Obesity
• Smoking
• Hyperlipidemia (high levels of lipids, such as cholesterol and triglycerides, in the blood)
• Sleep apnoea
• Atrial fibrillation
• Other cardiovascular pathologies^2,4

How can we address these risk factors?

Lifestyle changes such as eating a healthy, balanced diet and regular exercise can greatly improve an individual's overall health and act on important risk factors for Binswanger's Disease and cerebral small vessel disease.¹ Indeed, modification of such behaviours can directly impact cardiovascular health, which is the cause of the development of these diseases.¹⁰

Current research in binswanger disease (BD) and cerebral small vessel disease (CSVD) 

Currently, the field of vascular dementia research is undergoing some changes. Indeed, in the past, an array of scientific terms had been used within the literature, with little to no consensus on the criteria for said diseases. This has resulted in overlapping terms, slowing the research progress as it did not allow scientists to use universal terminology. The recent VICCCS has allowed it to provide a framework on terminology and provided a rigorous classification system for vascular dementia/vascular cognitive impairment and dementia (VCID).⁵

Additionally, regarding diagnostic criteria, the emerging field of biomarkers may allow for the early diagnosis of BD and CSVD. A biological marker is the measure of a given component in a tissue, which acts as an objective measure for the likeness of pathological processes occurring within the body - it is a warning sign. It can be used as a preliminary diagnostic tool.¹¹ In the case of vascular dementia, and for BD and CSVD, the blood or cerebrospinal fluid (CSF) will contain important biomarkers for disease. For example, the albumin index can be calculated. An elevated number will indicate disruption of the blood-brain barrier (BBB), a sign of Binswanger disease and cerebral small vessel disease.¹²

Summary

Binswanger disease, a subtype of vascular dementia, is closely linked to cerebral small vessel disease (CSVD). It is marked by white matter damage, lacunar infarcts and symptoms such as executive dysfunction, gait disturbance, urinary incontinence and apathy or depression. These changes stem from chronic small vessel damage – primarily due to hypertension, diabetes and other cardiovascular risk factors.

Diagnosis relies on CT and, more importantly, MRI findings, in addition to clinical presentation. While there is no cure, early recognition and targeted management of vascular risk factors can slow progression and improve quality of life.

Ultimately, Binswanger disease highlights the critical role of vascular health in preserving cognitive function, highlighting the importance of protecting small vessels when aiming to prevent dementia.