Binswanger’s disease: Causes, Symptoms,Treatment, Prognosis

Binswanger’s disease is a type of vascular dementia that is caused by microscopic damage within the deep layers of the brain’s white matter. It is secondary to atherosclerosis, also known as hardening of the arteries, characterized by thickening and narrowing of the arteries, which provide blood supply to the subcortical areas of the brain. As the blood vessels become narrow, the blood supply to the brain tissues decreases leading to degeneration of the brain tissues. Binswanger’s disease usually affects during the fourth decade of life and the condition worsens with increasing age. The symptoms of Binswanger’s disease are usually noted between the ages of 54 to 66 years of age, with the earliest signs being mental deterioration.

It was first described in 1894 by Otto Binswanger and was later renamed by Alois Alzheimer as “Binswanger’s disease” in 1902. Further studies and most of the modern day discoveries were made by Olszewski in 1962.

Binswanger’s disease is also known by the following terminologies:

• Multi-infarct dementia, Binswanger type.
• Binswanger encephalopathy.
• Subcortical dementia.
• Vascular dementia, Binswanger type.
• Subcortical ischemic vascular disease.
• Subcortical arteriosclerotic encephalopathy, SAE.

The symptoms of Binswanger’s disease are due to disruption of the subcortical neural areas. The most commonly encountered symptoms include psychomotor slowness. Symptoms related to cognitive functioning includes:

• Short term memory loss and mental deterioration.
• Mood disorder including apathy, depression and irritability.
• Difficulty with organization and regulation of attention.
• Inability to act or make appropriate decision.
• Behavioural issues.

Other symptoms of Binswanger’s disease include:

• Speech difficulties.
• Language issues.
• Unsteady balance and gait.
• Frequent falls and clumsiness.
• Forgetfulness.
• Changes in personality.
• Urinary disturbances such as uncontrollable bladder.
• Transient ischemic attack.
• Muscle ataxia.
• Slow movements.
• Postural changes.
• Frequent episodes of fainting.
• Epilepsy.

Patients affected by Binswanger’s disease often have difficulty in carrying out daily activities such as management of daily finances, driving, cooking etc.

Binswanger’s disease affects both males and females equally. It is commonly seen in individuals above 50 years of age.

Binswanger’s disease is a progressive disease with no cure at present. The symptoms of Binswanger’s disease may appear suddenly or gradually and there after progress in a step wise manner. It is one of the most severe forms of vascular dementia associated with severe impairment. Binswanger’s disease is often accompanied by Alzheimer’s disease. It may be associated with other diseases such as dementia with Lewy bodies, frontotemporal degeneration, normal pressure hydrocephalus etc.

Binswanger disease is primarily caused by atherosclerosis, thromboembolism and disorders in the blood vessels that supply the deep tissues of the brain. Other predisposing risk factors of Binswanger’s disease include elevated cholesterol levels, heart disorders, diabetes, elevated blood pressure and history of smoking. Certain rare hereditary diseases such as cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) may also lead to Binswanger’s disease. Binswanger’s disease is not a specific disease. It is a clinical syndrome of a vascular dementia with multiple causes.

Diagnosis of Binswanger’s disease is done after obtaining a detailed case history followed by clinical examination. Investigative studies such as CT scan, MRI and proton MR spectrography are done as confirmatory tests. CT scan and MRI of brain show a characteristic pattern of Binswanger’s disease damages to brain tissue. Indicative signs include presence of infarction, lesions, loss of intensity of central white matter, enlargement of ventricles and leukoaraiosis. Leukoaraiosis or LA is an imaging finding of the white matter that is commonly found in imagining tests of patients with Binswanger’s disease. This finding is found in other neurological conditions as well and thus cannot be taken as a confirmatory sign. A Mini Mental Test (MMT) is usually done for assessment of cognitive impairment and vascular dementia. The condition is best diagnosed by a team of experts which includes experienced neurologist and psychiatrist to have in depth understanding of the condition and thus can rule out other neurological or psychological conditions.

Binswanger’s disease does not have a specific treatment. The current treatment modality is symptomatic management of the condition. The most accepted treatment modality for Binswanger’s disease includes management of vascular risk factors that promotes poor perfusion in the brain. This includes management of the underlying issues such as diabetes and hypertension to limit progression of atherosclerosis, which in turn will slow down the progression of Binswanger’s disease. Oral medications may be prescribed based on the symptoms demonstrated by the patient. Binswanger’s disease patients with anxiety and depression may require anti-depressant medication such as SSRI (serotonin specific reuptake inhibitors) such as sertraline or citalopram. In patients with agitation issues and disruptive behavior, antipsychotic drugs such as risperidone and olanzapine may be prescribed. A number of drugs trials have been carried out, which have shown that the drug Memantine provides significant improvement in cognition and provides stabilization of global functioning and behavior. It has been noticed that medications for Alzheimer’s disease such as Aricept (donepezil) helps in management of Binswanger’s disease. In case of psychological issues, behavioral counseling and psychiatric consultation may be recommended.

Treatment of hypertension involves prescription of anti-hypertensive drugs. Antiplatelet drugs or warfarin may be used to control thromboembolisms and statin therapy may be recommended for management of hypercholesterolemia.

The only precautionary methods that can be taken to prevent development of Binswanger’s syndrome is following a healthy lifestyle and seek early treatment for controlling conditions such as hypertension and diabetes to avoid development of atherosclerosis. Behavior such as following a healthy diet, maintaining regular sleep/wake up schedule, regular exercising, limiting alcohol intake and smoking help in slowing down the progression of Binswanger’s disease.

Binswanger’s disease is a vascular dementia secondary to arteriosclerosis of the blood vessels that supply the brain leading to degeneration of the brain tissues. Binswanger’s disease affects male and females equally and usually occurs above 50 years of age. Being a very rare disease not much is known about this condition. A large number of researches are being conducted by the National Institute of Neurological Disorder and Stroke (NINDS) to study and understand Binswanger’s disease better. Binswanger disease may be associated with other conditions such as Alzheimer’s disease, dementia with Lewy bodies, frontotemporal degeneration, and normal pressure hydrocephalus. Binswanger’s disease is caused due to presence of underlying issues such as hypertension, hypercholesterolemia, diabetes, arteriosclerosis and thromboembolism, which limits the blood supply to the structures of the brain. Unhealthy lifestyle such as smoking and alcohol consumption can also lead to Binswanger’s disease. It can also be caused due to genetic inheritance and hereditary conditions such as CADASIL. Binswanger’s disease does not have a specific cure and the only way to prevent this condition is to follow a healthy lifestyle to avoid development of known risk factors.

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