NORD gratefully acknowledges Rodger J. Elble, MD, PhD, Professor and Chair of Neurology, Director, Parkinson Disease and Movement Disorders Center, Neurology Residency Director, 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 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.
Affected individuals often become depressed, uncaring (apathetic), inactive, and unable to act or make decisions (abulic). They become withdrawn, and exhibit poor judgement and less spontaneous communication.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).
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).
Binswanger disease is caused by atherosclerosis, 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.
Binswanger disease affects males and females in equal numbers and usually occurs in individuals age 50 years or older.
The diagnosis of Binswanger disease is usually based on a thorough clinical evaluation, a detailed patient history, identification of characteristic findings, 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.
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.
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:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
Contact for additional information about Binswanger’s disease:
Rodger J. Elble, MD, PhD
Professor and Chair of Neurology
Director, Parkinson Disease and Movement Disorders Center
Neurology Residency Director
Southern Illinois Univeristy School of Medicine
PO Box 19643
Springfield, IL 62794-9643
Phone: (217) 545-7182
FAX: (217) 545-1903
email: firstname.lastname@example.org or email@example.com
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.
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.
NINDS Binswanger’s Disease Information Page.
http://www.ninds.nih.gov/disorders/binswangers/binswangers.htm.Updated December 28, 2011 . Accessed February 9, 2012.
Rarer Causes of Dementia. Alzheimer’s Society Information Sheet. http://www.alzheimers.org.uk/site/scripts/documents_info.php?documentID=135.Updated December 2010. Accessed February 9, 2012.
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