Binswanger's Disease

Binswanger's Disease

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Binswanger's Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

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

Disorder Subdivisions

  • None

General Discussion

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.

Symptoms

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 They become withdrawn, and exhibit poor judgement and less spontaneous communication.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).

Causes

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.

Affected Populations

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

Standard Therapies

Diagnosis

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.



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.

Investigational Therapies

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

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



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: relble@siumed.edu or relble@msn.com

References

REVIEW ARTICLES

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

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.

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.

Resources

National Association for Continence

P.O. Box 1019

Charleston, SC 29402-1019

United States

Tel: (843)377-0900

Fax: (342)377-0905

Tel: (800)252-3337

Email: memberservices@nafc.org

Internet: http://www.nafc.org



Alzheimer's Association

225 N. Michigan Avenue

17th Floor

Chicago, IL 60601

USA

Tel: (312)335-8700

Fax: (866)699-1246

Tel: (800)272-3900

TDD: (312)335-5886

Email: info@alz.org

Internet: http://www.alz.org



Alzheimer's Disease Education and Referral Center

P.O. Box 8250

Silver Spring, MD 20907-8250

Tel: (301)495-3311

Fax: (301)495-3334

Tel: (800)438-4380

Email: adear@nia.nih.gov

Internet: http://www.nia.nih.gov/alzheimers



NIH/National Institute on Aging

31 Center Drive, MSC 2292

Building 31

Room 5C27

Bethesda, MD 20892

Tel: (301)496-1752

Fax: (301)496-1072

Tel: (800)222-2225

TDD: (800)222-4225

Email: bapquery@nia.nih.gov

Internet: http://www.nih.gov/nia



NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801

Bethesda, MD 20824

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

TDD: (301)468-5981

Internet: http://www.ninds.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use . How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.