Introduction

Binswanger’s disease (BD) is a rare form of dementia that is also known as subcortical vascular dementia, subcortical arteriosclerotic encephalopathy, lacunar dementia, or ischemic periventricular leukoencephalopathy. BD is a progressive neurological disorder caused by thickening and narrowing of the arteries, which affects the blood vessels that supply the white matter of the brain. As the arteries become hardened and narrowed, the blood supplied by those arteries is reduced, and eventually, the brain tissue dies.¹ 

The symptoms associated with BD are mostly related to cognitive functioning, such as:²

• Psychomotor slowness
• Short-term memory
• Behaviour and mood changes
• Reduced ability to act or make decisions quickly
• Forgetfulness
• Changes in speech
• Unsteady gait
• Clumsiness or frequent falls

While some people with BD experience sudden symptoms, most people present with a more gradual decline in symptoms. People with BD also often present with high blood pressure or a history of stroke and cardiovascular diseases. Stroke may also cause sudden worsening of the existing symptoms of BD, and then stabilise and improve briefly, although the patients’ overall condition continues to deteriorate as the blood vessels become more obstructed over time. Uncontrolled high blood pressure is an especially important risk factor as it causes the narrowing of the small blood vessels and subsequently leads to a reduction in blood flow, particularly to the brain. Other risk factors of BD are diabetes mellitus, dyslipidemia, smoking, sleep apnoea, and atrial fibrillation.³

Diagnosis of Binswanger’s disease

Magnetic resonance imaging (MRI) is a more sensitive technique for detecting white matter abnormalities, compared to computed tomography (CT) scan. However, the lesions visualised by MRI are also similar to those of typical Alzheimer’s disease in 30% of cases. Therefore, while imaging is an important tool to diagnose BD, it is not used independently. The clinical features, along with history taking, are crucial for the diagnosis of the disease.⁴

Essentially, patients with BD have gradual progress and varying degrees of cognitive impairment. The first symptom usually begin in the sixth decade of life, and usually evolve over a five to ten-year period. There are notable cognitive and behavioural changes, characterised by dementia and dysexecutive syndrome, motor and gait disturbances, falls, and urinary incontinence with periods of exacerbation and alleviation. Most often, patients become slow and abulic. Changes in mental status (euphoric, elated, aggressive, depressive) and gradual, subtle worsening of memory loss were most frequently observed. Slight confusion, changes in personality, apathy, loss of interest in usual activities, or paranoia are some of the terms used to describe intellectual deterioration. Difficulties in judgment and orientation, and dependence on others for daily activities, are usually seen in later stages. A more complex clinical feature of pseudobulbar palsy, hemiparesis, rigidity, or gait ataxia, are helpful in diagnosing BD. A patient’s past history often reveals high blood pressure and episodes of “mini-strokes”. A history of persistent high blood pressure is particularly important, and if absent, the diagnosis of BD is questionable.^5,6,7,8

Management and treatment of BD

There are currently no treatment options available specifically for BD patients. BD patients are treated symptomatically to prevent progression, improve behavior, and reduce complications.

Pharmacological treatment

Antihypertensive medications

Antihypertensive medications are used to reduce blood pressure levels, and are used more aggressively in younger patients compared to older patients. Besides being a major risk factor in BD, high blood pressure is also a major risk factor for stroke, therefore, the treatment is used as both primary and secondary prevention of stroke. Controlling blood pressure has been shown to reduce the progression of cognitive impairment and the onset of dementia associated with stroke and white matter hyperintensities (WMHs).^9,10,11

Antiplatelet and anticoagulant therapy

Antiplatelet and anticoagulant therapy, such as aspirin, is used to prevent further strokes. Individuals with WMHs have a three times higher risk of stroke compared to the general population. However, antiplatelet monotherapy for BD patients is only initiated when patients present with symptoms of stroke to prevent intracranial hemorrhages (ICH).¹²

Cholesterol-lowering medications

Cholesterol-lowering agents like statins are used as a secondary prevention of stroke in BD patients. It is highly recommended for use in patients with a previous history of stroke. Statins reduce the “bad cholesterol” in the body responsible for atherosclerosis and cardiovascular diseases, which are the risk factors in BD.^13,14

Cognitive enhancers

Cognitive enhancers such as cholinesterase inhibitors and NMDA receptor antagonists may be used to improve global function and behavioural symptoms.^15,16,17

Antidepressant medications

Antidepressant medications such as clomipramine, sertraline, citalopram, risperidone, and olanzapine may be used in patients who present with depressive symptoms, agitation, anxiety, or disruptive behaviours.¹⁸ 

Non-pharmacological management

Lifestyle modifications

Since there is no cure, the best treatment is preventive, starting early in adulthood, by controlling risk factors such as hypertension, diabetes, and smoking. These risk factors can be controlled or reduced through lifestyle modifications that include a healthy and balanced diet, regular physical activity and exercise, quitting smoking, stress, and weight management. By adopting and maintaining healthy habits early in life, individuals can significantly reduce their risk of developing cardiovascular diseases (CVDs) in the future, thus reducing the risk of developing dementia.¹⁹

Cognition‐focused interventions

The impaired ability to function in daily life can vary from mild difficulty with instrumental activities to dependence on others for basic activities. However, even a mild impairment can impact an individual’s independence, resulting in loss of confidence and withdrawal from activities. Therefore, cognitive-focused interventions are introduced, aimed at patients’ general enhancement of cognitive and social functioning. These are classified as cognitive rehabilitation and cognitive training.^20,21

Cognitive rehabilitation

Cognitive rehabilitation is a personalised approach that enables patients to improve their functioning in areas that they recognised as important, and to maintain as much independence as possible. The rehabilitation may suggest patients new ways of doing daily tasks which could include the use of memory aids like an alarm for an unattended stove, or pillbox with pre-packed and pre-labelled medications to avoid missing a dose.

Cognitive training

Cognitive training usually involves a set of standardised tasks that are designed to target specific cognitive functions such as memory, attention, or problem-solving. It includes doing repeating exercises with a gradual increase in difficulty to improve particular thinking skills and attention span. Other activities in cognitive training include playing cards, jigsaw puzzles, crosswords, anagrams, and more.

FAQs

Is Binswanger’s disease similar to Alzheimer’s disease?

Both Binswanger’s disease and Alzheimer’s disease are under the dementia umbrella, and although they share some similarities in certain signs and symptoms, Binswanger’s disease is a type of vascular dementia that is caused by reduced blood flow to the brain while Alzheimer’s disease is caused by protein build-up in the brain. Binswanger’s disease is also the second most common type of dementia after Alzheimer’s disease.

What is the life expectancy of Binswanger’s disease?

On average, the life expectancy is about five years after the symptoms begin. It varies from one patient to another depending on the other conditions the patients have. Although there is no cure, making changes to control the risk factors helps to slow down the progress of the disease. Because risk factors include stroke and heart attack, the patient’s death could also be due to stroke or heart attack.

Summary

Binswanger’s disease (BD) is a progressive form of vascular dementia that causes a gradual decline in one’s cognitive function and gait disturbances. Since there is no cure or specific treatment, the management of BD focuses on controlling risk factors and alleviating symptoms. The major risk factor is persistent, uncontrolled high blood pressure, which could also lead to stroke and other cardiovascular diseases that could worsen BD, hence, an early intervention that includes lifestyle modification is beneficial for a good prognosis.