Information on the following diseases can be found in the Related Disorders section of this report:

• Alzheimer Disease
• Pick's Disease
• Creutzfeldt-Jakob Disease

The range and severity of symptoms associated with Binswanger's disease vary greatly from case to case. In most affected individuals, the disorder slowly progresses during a five to 10 year period. In some cases, symptoms and physical findings associated with Binswanger's disease may stabilize or improve for a brief time; however, in most cases, progression of the disorder usually returns.

Affected individuals experience progressive memory loss and deterioration of intellectual abilities (dementia), strokes, paralysis of one or another side of the body (hemiparesis), electrical disturbance in the brain (seizures), and/or an abnormally slow, unsteady walk (abnormal gait).

Affected individuals often become depressed, uncaring (apathetic), inactive, unable to make decisions (abulic), hardly speak, and show poor judgment. In addition, affected individuals may exhibit difficulty forming words (dysarthria), swallowing difficulties (dysphagia), and inability to control the release of urine (incontinence). In some cases, affected individuals may demonstrate abnormalities that are similar to those seen in Parkinson syndrome, such as tremors; short, shuffling steps; loss of trunk mobility; and/or loss of coordination (synergy) between upper limb and trunk movements when walking. Individuals with Binswanger's disease may be at greater risk to develop narrowing and hardening of blood vessels (arteriosclerosis) and high blood pressure (hypertension) than the general population. The exact cause of Binswanger's disease is not known.

In many cases, individuals with Binswanger's disease may have a history of strokes and/or paralysis of one side of the body (hemiparesis). Eventually other signs of the disorder develop including progressive memory loss and deterioration of intellectual abilities (dementia), electrical disturbance in the brain (seizures), and/or an abnormally slow, unsteady walk (abnormal gait).
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The exact cause of Binswanger's Disease is not known. There is patchy or diffuse loss (degeneration) of the white matter of the brain, resulting in the behavioral and physical problems associated with senile dementia. This condition may develop in the aging brain as a result of many different factors including lack of blood flow (ischemia) to the brain. Other factors that may be associated with the development of Binswanger's Disease include stroke, high blood pressure, blood abnormalities or other causes not yet fully understood.
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Binswanger's Disease is thought to affect males and females in equal numbers and usually occurs in individuals between 55 and 75 years of age.
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Symptoms of the following disorders can be similar to those of Binswanger's Disease. Comparisons may be useful for a differential diagnosis:

Alzheimer's Disease is a common progressive disorder of the brain affecting memory, thought and language. Groups of nerve endings in the cortex of people with Alzheimer's degenerate, which disrupts the passage of electrochemical signals between the cells. These areas of degeneration are called "plaques". Changes known as "neurofibrillary tangles" also occur in nerve cells of the brain's cortex. The number of plaques and tangles appear to be directly proportional to the disturbance in intellectual function and memory. Recently scientists discovered that Alzheimer's Disease can be inherited through a dominant genetic trait in at least ten percent of affected individuals. Some researchers believe symptoms may be caused by excessive amounts of a protein that builds up in the brains of persons affected by this disorder, as well as abnormally low levels of the brain chemical (neurotransmitter) acetylcholine. As a result of these discoveries, tests to identify people at high risk, and drugs to treat Alzheimer's may be developed in the future. (For more information on this disorder, choose "Alzheimer" as your search term in the Rare Disease Database.)

Dementia with Lewy Bodies (DLB)
Among the rarer dementias is Dementia with Lewy Bodies (DLB) which is characterized by microscopic deposits in particular tissues of the brain (Lewy bodies) that cause damage to nerve cells. Many of the symptoms of DLB, such as loss of memory and reasoning skills, are similar to those in Alzheimer’s disease. Many people with DLB also develop Parkinson-type symptoms such as slowness, stiffness and tremor. Falls are not infrequent nor are hallucinations and mood swings.

Pick's Disease, also known as Lobar Atrophy of the brain, is characterized by neurological disturbances and usually begins between the ages of forty and sixty. This disorder appears to affect females more often than males, and the exact cause is not known. The area of the brain affected is the major different in Pick's Disease from other forms of senile dementia. This disease involves atrophy limited to the lobes of the brain. Areas of degeneration are identified by the presence of Pick cells and Pick inclusion bodies instead of plaques and tangles. (For more information on this disorder, choose "Pick" as your search term in the Rare Disease Database.)

Creutzfeldt-Jakob Disease in its early stages is marked with memory failure, changes in behavior, inability to concentrate, imperfect articulation of speech, lack of coordination, or visual disturbances. As the illness progresses, mental deterioration becomes pronounced. Involuntary movements (especially muscle jerks) appear, the patient may become blind, and develop weakness or stiffness in the arms or legs. Paralysis, sensory disturbances, and progressive muscular atrophy are other symptoms. Involuntary muscle contractions may be a prominent symptom throughout the course of the disease. General intellectual decline becomes more pronounced as the disorder progresses. Advanced symptoms may include mutism, absence of movement (akinesia), epileptic seizures, involuntary muscular contractions, rigidity, and semicoma. (For more information on this disorder, choose "Creutzfeldt-Jakob" as your search term in the Rare Disease Database.)

There are many other neurological disorders that can cause dementia and memory disturbances.
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Diagnosis
The diagnosis of Binswanger's Disease is usually based upon a thorough clinical evaluation, a detailed patient history, identification of characteristic findings, and a variety of tests, particularly advanced imaging techniques. Magnetic Resonance Imaging (MRI) and computerized tomography (CT) Scan may reveal the presence of lesions that affect the white matter of the brain and/or degeneration of the white matter of the brain. During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of tissue structure. During MRI, a magnetic field and radio waves are used to create cross-sectional images of the brain.

Treatment
Treatment of Binswanger's Disease often involves the use of anti-hypertensive drugs to control blood pressure. Anti-depressive drugs have been found to be very helpful in the management of depression associated with Binswanger's Disease. Other treatment is symptomatic and supportive.

TEXTBOOKS
Adams RD, Victor M, Ropper AA. Eds. Principles of Neurology. 6th ed. McGraw-Hill Companies. New York, NY; 1997:831-32.

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1399.

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.

Erkinjuntti T, Inzitari D, Pantoni L, et al. Research criteria for subcortical vascular dementia in clinical trials. J Neural Transm Suppl. 2000;59:23-30.

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

JOURNAL ARTICLES
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.

FROM THE INTERNET
NINDS Binswanger’s Disease Information Page. Nd. 3pp.
www.ninds.nih.gov/health_and_medical/disorders/binswan_doc.htm

Chui H. Dementia Due to Subcortical Ischemic Vascular Disease. Medscape. 2001. 11pp.
www.medscape.com/viewarticle/407405_Print

Alzheimer’s Disease and Related Dementias Fact Sheet. Alzheimer’s Association. ©1999. 2pp.
http://www.sialzheimers.com/52/cat52.htm?778

Rarer Causes of Dementia. Alzheimer’s Society Information Sheet. nd. 4pp.
www.alzheimers.org.uk

Other Dementias. Alzheimer Society TORONTO. June 2, 2003. 7pp.
www.asmt.org/RD2.htm

Olsen CG, Clasen ME. Senile Dementia of the Binswanger’s Type. 1998. 8pp.
http://aafp.or/afp/981200ap/olsen.html

Facts about dementia. Alzheimer’s Society. July 2001. 7pp.
www.alzheimers.org.uk.org/Facts_about_dementia/What_is_dementia/info_rarer.htm