Binswanger disease, a type of vascular dementia, is characterized by atherosclerosis of blood vessels supplying the deep white matter of the brain. Hypertension, being the principal risk factor, is universally present, and a variable degree of cognitive impairment, gait disturbances and other neurological deficits are encountered in patients. A thorough physical examination and magnetic resonance imaging (MRI) of the brain are necessary to make the diagnosis.
Presentation
Binswanger disease (BD) is a distinct form of vascular dementia in which atherosclerosis and hypertension affecting the smaller blood vessels in the white matter cause a slowly progressive ischemia of the subcortical areas of the brain[1] [2] [3]. Hypertensive vascular disease is, by far, the single most important risk factor for the development of BD, and many individuals report prior episodes of stroke or transient ischemic attacks (TIAs), whereas diabetes mellitus, pre-diabetic states, cigarette smoking, hyperlipidemia and sleep apnea are mentioned in certain reports as well [1] [4]. Progressive reduction of blood flow through these areas explains the gradual course of BD, and the initial symptoms are related to alterations in cognitive status - decline in executive functions, reduction in the speed of processing information and memory dysfunction [1] [2] [5] [6]. Moreover, deficits in attention, behavioral changes and emotional lability are clinical hallmarks of BD and other forms of dementia [3] [5]. In addition, focal neurological deficits are commonly observed, most prominent being those involving the upper motor neurons (UMNs) - muscle rigidity, hyperreflexia (often asymmetric), signs of parkinsonism, ataxia and gait disturbances [1] [2] [3] [6]. Urinary incontinence is reported in many individuals as well [4] [6].
Workup
To diagnose BD, a detailed patient history is the first step in the workup. The course and progression of symptoms are noted in the history. If patients give unreliable data regarding their illness, a heterogeneous anamnesis from either family members of close friends can be highly useful in obtaining an objective view about the condition of the patient. History of cardiovascular disease and the presence of additional risk factors and comorbidities is integral in order to make a presumptive diagnosis. The second step is a meticulous physical examination, with an emphasis on cognitive assessment and neurological examination, both equally important procedures when there is a clinical suspicion of any type of dementia. The Montreal Cognitive Assessment (MOCA), preferred over the mini-mental status examination (MMSE) is recommended for evaluation of cognitive function [1] [7]. Once valid clinical criteria for dementia are noted, the diagnosis of BD can be confirmed with imaging studies, especially magnetic resonance imaging (MRI) [1] [2] [3] [5] [7]. One of the most important features of BD on MRI are 5-10 mm lacunar infarcts, principally located in the brain stem and the majority of the white matter with signs of atrophy [1] [4]. Together with lacunar infarcts, hyperintensity of the white matter (known as leukoaraiosis), usually in the periventricular space, is diagnostic for BD [1] [4] [6]. In addition to MRI, computed tomography (CT) can also identify leukoaraiosis, and is preferred over MRI in certain circumstances [1] [4] [6].
Treatment
There is no cure for Binswanger Disease, but treatment focuses on managing symptoms and slowing disease progression. Controlling vascular risk factors, such as high blood pressure, high cholesterol, and diabetes, is essential. Medications may be prescribed to address cognitive symptoms, mood disorders, or motor difficulties. Physical therapy and occupational therapy can help improve mobility and daily functioning. Supportive care, including counseling and social support, is also important for patients and their families.
Prognosis
The prognosis for Binswanger Disease varies depending on the severity of symptoms and the effectiveness of managing risk factors. The disease typically progresses slowly, but cognitive and physical decline can significantly impact the quality of life. Early diagnosis and intervention can help slow progression and improve outcomes. However, as with other forms of dementia, Binswanger Disease is ultimately a progressive condition.
Etiology
Binswanger Disease is primarily caused by chronic damage to the small blood vessels in the brain, leading to reduced blood flow and subsequent white matter damage. Hypertension is a major risk factor, as it can cause changes in the blood vessels over time. Other contributing factors include diabetes, high cholesterol, smoking, and a history of stroke. Genetic predisposition may also play a role in some cases.
Epidemiology
Binswanger Disease is considered a rare form of dementia, although its exact prevalence is not well-documented. It is more common in older adults, particularly those over the age of 60. The condition is often underdiagnosed or misdiagnosed due to its overlap with other types of dementia and the subtlety of early symptoms. It is more prevalent in individuals with a history of vascular risk factors.
Pathophysiology
The pathophysiology of Binswanger Disease involves chronic ischemia, or reduced blood flow, to the brain's white matter. This ischemia results from damage to the small blood vessels, leading to demyelination, or loss of the protective covering of nerve fibers. The resulting white matter lesions disrupt communication between different parts of the brain, contributing to cognitive and motor symptoms. The disease process is gradual and progressive, with cumulative damage over time.
Prevention
Preventing Binswanger Disease involves managing vascular risk factors to maintain healthy blood vessels. This includes controlling blood pressure, maintaining healthy cholesterol levels, managing diabetes, and avoiding smoking. Regular physical activity, a balanced diet, and routine medical check-ups can also help reduce the risk. Early intervention and lifestyle modifications are key to preventing or delaying the onset of symptoms.
Summary
Binswanger Disease is a form of vascular dementia caused by damage to the small blood vessels in the brain, leading to cognitive decline and motor impairment. It is associated with vascular risk factors such as hypertension and diabetes. While there is no cure, managing these risk factors and providing supportive care can help slow disease progression and improve quality of life. Early diagnosis and intervention are crucial for better outcomes.
Patient Information
If you or a loved one is experiencing symptoms such as memory loss, difficulty walking, or mood changes, it is important to seek medical evaluation. Binswanger Disease is a type of dementia that affects the brain's white matter due to blood vessel damage. Managing risk factors like high blood pressure and diabetes can help slow the progression of the disease. Support from healthcare professionals, family, and friends is essential in managing the condition and maintaining quality of life.
References
- Huisa BN, Rosenberg GA. Binswanger’s disease: Diagnosis and Management. Expert Rev Neurother. 2014;14(10):1203-1213.
- Huisa BN, Caprihan A, Thompson J, et al. Long-Term Blood-Brain Barrier Permeability Changes in Binswanger’s Disease. Stroke; a journal of cerebral circulation. 2015;46(9):2413-2418.
- Kovács T, Szirmai I, Papp M. Clinico-pathology and differential diagnosis of Binswanger's disease [Article in Hungarian]. Ideggyogy Sz. 2005;58(3-4):78-87.
- Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
- Akiguchi I, Budka H, Shirakashi Y, et al. MRI features of Binswanger’s disease predict prognosis and associated pathology. Annals of Clinical and Translational Neurology. 2014;1(10):813-821.
- Bennett DA, Wilson RS, Gilley DW, Fox JH. Clinical diagnosis of Binswanger's disease. J Neurol Neurosurg Psychiatry. 1990;53:961-965.
- Moretti R, Torre P, Antonello RM, Manganaro D, Vilotti C, Pizzolato G. Risk factors for vascular dementia: Hypotension as a key point. Vasc Health Risk Manag. 2008;4(2):395-402.