Binswanger Disease

Disease Overview

Binswanger disease is a progressive neurological disorder caused by arteriosclerosis and thromboembolism affecting the blood vessels that supply the white-matter and deep structures of the brain (basal ganglia and thalamus). Most patients experience progressive loss of memory and intellectual abilities (dementia), urinary urgency or incontinence, and an abnormally slow, shuffling, unsteady pattern of walking, usually over a 5-10 year period. Due to their vascular etiology, the symptoms and physical findings associated with Binswanger disease may suddenly worsen due to stroke, stabilize and then improve for a brief time, but the patient’s overall condition continues to progress as the blood vessels become increasingly obstructed.

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Synonyms

• Binswanger encephalopathy
• multi-infarct dementia, Binswanger type
• subcortical arteriosclerotic encephalopathy, SAE
• subcortical dementia
• subcortical ischemic vascular disease
• vascular dementia, Binswanger type

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Signs & Symptoms

Affected individuals often become depressed, uncaring (apathetic), inactive, and unable to act or make decisions (abulic). They become withdrawn, and exhibit poor judgement, reduced planning and organizational skills, and less spontaneous communication. In addition, affected individuals may have difficulty with speech (dysarthria), swallowing (dysphagia), and urinary bladder control (incontinence). Some patients exhibit abnormalities that are similar to those seen in Parkinson disease, such as slowness, poor balance and short, shuffling steps (Parkinsonism). Tremor is usually not a feature.

Many individuals with Binswanger disease have a history of strokes or transient ischemic attacks. Consequently, the symptoms and signs of this disease develop in a stuttering or stepwise fashion; in contrast to the insidious, gradually progressive course of neurodegenerative diseases (see Related Disorders).

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Causes

Binswanger disease is caused by arteriosclerosis, thromboembolism and other diseases that obstruct blood vessels that supply the deep structures of the brain. Hypertension, smoking, hypercholesterolemia, heart disease and diabetes mellitus are risk factors for Binswanger disease. Rare hereditary diseases such as CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) also cause Binswanger disease. Thus, Binswanger disease is actually a clinical syndrome of vascular dementia with multiple causes, not a specific disease. The reduced blood flow in brain tissue appears to produce secondary inflammation that may be a target for treatment.

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Affected populations

Binswanger disease affects males and females in equal numbers and usually occurs in individuals age 50 years or older.

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Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Binswanger disease. Comparisons may be useful for a differential diagnosis.

Alzheimer Disease
Alzheimer disease is a common progressive disorder of the brain affecting memory, intellect and language function. Alzheimer patients are at increased risk of falling, but most exhibit little or no gross locomotor disturbance until very late in the disease.

Dementia with Lewy Bodies (DLB)
Patients with DLB have Parkinsonism and dementia that begin more or less simultaneously. Patients with DLB have difficulty with attention, concentration and multitasking (executive function). They are also prone to depression, visual hallucinations, sleep disturbances and day-to-day fluctuations in cognitive function. Memory is often less affected than in Alzheimer disease. The Parkinsonism in DLB is gradually progressive and consists of slowness (bradykinesia), muscle stiffness (rigidity), stooped posture, and slow shuffling gait that is often poorly responsive to levodopa. Tremor is frequently mild or absent, compared to classic Parkinson disease. REM sleep behavior (vocalizations and movement during dreams) is common and may be violent.

Frontotemporal Degeneration
Pick disease and other forms of frontotemporal degeneration typically begin before age 65. The initial presentation is often personality change (e.g., impaired judgement, impulsive behavior, and blunted emotions), which may lead to psychiatric consultation. Loss of communication skills is another common presentation. Motor disturbances may occur early or late in the disease. Gradually progressive dementia occurs in all patients. (For more information on this disorder, choose “frontotemporal” as your search term in the Rare Disease Database.)

Normal Pressure Hydrocephalus
Binswanger disease may be difficult to distinguish from so-called normal pressure hydrocephalus (NPH). Both conditions produce the clinical triad of gait disturbance, dementia and urinary incontinence. However, NPH progresses gradually, not in the stuttering or stepwise fashion of vascular disease. The ventricular system of the brain commonly enlarges in Binswanger disease and neurodegenerative dementias (hydrocephalus ex vacuo), so distinguishing these conditions from NPH is often difficult.

There are many other neurological disorders that can cause dementia and memory disturbances.

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Diagnosis

The diagnosis of Binswanger disease is usually based on a thorough clinical evaluation, including a detailed patient history, physical examination, and magnetic resonance imaging (MRI) or computerized tomography (CT) scanning of the brain. MRI and CT reveal nerve fiber (white matter) degeneration and multiple small strokes in the deep structures of the brain.

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Standard Therapies

Treatment

The ischemic brain damage in Binswanger disease is not reversible, so treatment is focused on reducing risk factors for stroke, thereby retarding progression of the disease. Treatment usually involves the use of anti-hypertensive drugs to control blood pressure, antiplatelet drugs (e.g., aspirin) or warfarin to reduce thromboembolism, statins to reduce atherosclerosis, smoking cessation and diabetic control. Antidepressant drugs are helpful in the management of depression associated with Binswanger disease. Other treatment is symptomatic and supportive.

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Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov . All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Toll free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

Contact for additional information about Binswanger disease:
Rodger J. Elble, MD, PhD
Professor of Neurology
Director, Parkinson Disease and Movement Disorders Center
Southern Illinois University School of Medicine
PO Box 19645
Springfield, IL 62794-9645
Phone: (217) 545-7194
FAX: (217) 545-1903
email: [email protected]

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References

REVIEW ARTICLES

Huisa BN, Rosenberg GA. Binswanger’s disease: toward a diagnosis agreement and therapeutic approach. Expert Rev Neurother. 2014;14:1203-13.

Erkinjuntti T. Diagnosis and management of vascular cognitive impairment and dementia. J Neural Transm Suppl. 2002;63:91-101.

Erkinjuntti T. Subcortical vascular dementia. Cerebrovasc Dis. 2002;13 Suppl 2:58-60.

Loeb C. Binswanger’s disease is not a single entity. Neurol Sci. 2000;21:343-48.

Olsen CG, Classen ME. Senile dementia of the Binswanger’s type. Am Fam Physician. 1998;58:2068-74.

Caplan LR. Binswanger’s disease–revisited. Neurology 1995;45:626-633.

JOURNAL ARTICLES

Rosenberg GA. Binswanger’s disease: biomarkers in the inflammatory form of vascular cognitive impairment and dementia. J Neurochem. 2018;144:634-43.

Dichgans M. A new cause of hereditary small vessel disease: angiopathy of retina and brain. Neurology 2003;60:8-9.

Jellinger KA. The pathology of ischemic-vascular dementia: an update. Journal of Neurological Sciences 2002;203-204:153-157.

Erkinjuntti T, Inzitari D, Pantoni L, et al. Limitations of clinical criteria for the diagnosis of vascular dementia in clinical trials. Is a focus on subcortical vascular dementia a solution? Ann N Y Acad Sci. 2000;903:262-72.

Davous P. CADASIL: a review with proposed diagnostic criteria. Eur J Neurol. 1998;5:219-33.

CADASIL syndrome: a genetic form of vascular dementia. J Geriatr Psychiatry Neurol. 1998;11:71-77.

INTERNET

NINDS Binswanger’s Disease Information Page. Last Update 2019-03-27. https://www.ninds.nih.gov/Disorders/All-Disorders/Binswangers-Disease-Information-Page Accessed April 29, 2019.

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Programs & Resources

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