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Last updated:
6/8/2023
Years published: 1989, 1996, 1997, 2003, 2007, 2009, 2011, 2012, 2016, 2019, 2023
NORD gratefully acknowledges Rodger J. Elble, MD, PhD, Professor of Neurology, Southern Illinois University School of Medicine, for assistance in the preparation of this report.
Binswanger disease is a progressive neurological disorder caused by arteriosclerosis and thromboembolism affecting the blood vessels that supply the white-matter (nerve fiber pathways) and clusters of nerve cells (basal ganglia and thalamus) beneath the surface of the brain (cerebral cortex). 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.
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 They become withdrawn and exhibit poor judgement, reduced planning and organizational skills, and less spontaneous communication.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 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, some symptoms and signs of this disease may develop in a stuttering or stepwise fashion, but there is also gradual deterioration due to progressive destruction of nerve fiber pathways deep in the brain (subcortical white matter). Therefore, Binswanger disease may be difficult to discern from neurodegenerative diseases like Alzheimer disease and dementia with Lewy bodies, which begin insidiously and progress gradually (see Related Disorders). Furthermore, Binswanger disease and neurodegenerative diseases commonly coexist in the same patient because these conditions are so common in older people.
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.
Binswanger disease affects males and females in equal numbers and usually occurs in individuals aged 50 years or older.
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.
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. Inflammation appears to play a role in small vessel damage, but there is no proven way of treating this.
It is important to remember that Binswanger disease often coexists with neurodegenerative diseases such as Alzheimer disease, dementia with Lewy bodies and Parkinson disease. Consequently, patients often receive trials of cholinesterase inhibitors (e.g., donepezil, rivastigmine, galantamine) for cognitive impairment and levodopa for motor impairment.
Other treatment is symptomatic and supportive. Antidepressant drugs are helpful in the management of depression associated with Binswanger disease. Good sleep hygiene and regular exercise are very important. Speech therapy is helpful in the assessment and treatment of speech and swallowing difficulties. Occupational therapy can perform in-home safety evaluations and recommend useful aids and strategies to improve safety and independence. Patients often benefit from some of the same physical therapy and exercise programs that are useful in Parkinson disease.
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: prpl@cc.nih.gov
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/for-patients-and-families/information-resources/news-patient-recruitment/
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
Southern Illinois University School of Medicine
PO Box 19645
Springfield, IL 62794-9645
Phone: (217) 545-7194
FAX: (217) 545-1903
email: relble@siumed.edu
REVIEW ARTICLES
Rosenberg GA, Wallin A, Wardlaw JM, Markus HS, Montaner J, Wolfson L, Iadecola C, Zlokovic BV, Joutel A, Dichgans M, Duering M, Schmidt R, Korczyn AD, Grinberg LT, Chui HC, Hachinski V. Consensus statement for diagnosis of subcortical small vessel disease. J Cereb Blood Flow Metab. 2016;36:6-25.
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. Reviewed Feb 7, 2023. Last. https://www.ninds.nih.gov/Disorders/All-Disorders/Binswangers-Disease-Information-Page Accessed May 15, 2023.
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Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.
Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/
