Binswanger disease

Definition

Binswanger disease is a rare form of progressive dementia that develops after age 60 and involves degeneration of the brain's white matter.

Description

Also known as subcortical arteriosclerotic encephalopathy , Binswanger disease is a form of subcortical dementia. Dementia is a general term used to describe a generalized deterioration of thinking and reasoning skills. In the case of Binswanger disease, the deterioration is due to physiological problems (i.e., organic factors). While many dementias result from damage to cortical areas of the brain, some diseases, including Binswanger disease, Alzheimer's disease , Parkinson's disease , Huntington disease , and dementia associated with AIDS , result from damage to subcortical areas of the brain (specifically, to subcortical connections).

Alternate names for Binswanger disease include Binswanger-type multi-infarct dementia , Binswanger encephalopathy, and Binswanger-type vascular dementia.

As with other individuals suffering subcortical dementia, people with Binswanger experience difficulties in maintaining attention to tasks and show depressed levels of motivation often accompanied by mood swings or apathy.

Demographics

Although Binswanger disease may occur in younger groups, the symptoms usually become pronounced in patients over 60 years of age.

Causes and symptoms

The exact cause of Binswanger disease is unknown, however, lesions in cerebrovascular tissue located in the inner white matter of the brain cause most of the symptoms. Prominent symptoms include rapid mood changes, loss of the ability to focus on tasks, a deterioration in thought processes (e.g., loss of memory and cognition), and mood changes.

Individuals with Binswanger disease may also have elevated blood pressure or suffer from stroke . Binswanger disease is found to be associated with blood (hematological) abnormalities with regard to the types and numbers of cells present, diseases of large blood vessels (especially in the upper chest and neck regions), and diseases of the heart. Abnormal electrical disturbances in the brain may cause seizures .

Binswanger's symptoms may be elusive in both appearance and degree. Not all people experience all the symptoms normally associated with the disease, and patients may experience symptoms for a period of time, followed by brief periods in which they are relatively symptom free.

As with other dementias, patients often present evidence of forgetfulness, memory loss, confusion and/or confabulation of events in terms of time and space (e.g., having a memory of two events that occur on different days as a combined memory of one event).

People with Binswanger disease often suffer depression and withdraw from family, friends, and co-workers (social withdrawal). Although clinical depression is a psychiatric term and requires a separate diagnosis, Binswanger patients suffering depression show a marked loss of interest in activities they once found pleasurable.

As the dementia progresses, people with Binswanger disease may initially lose the ability to perform tasks involving fine motor coordination, such as tying shoes or writing by hand, followed by a loss of broader function. Loss of bladder control (urinary incontinence) may develop, as well as generalized clumsiness or difficulty in walking. Later, patients often develop a blank-like stare and may have difficulty speaking or swallowing.

Diagnosis

Binswanger disease is identified by detection and characterization of lesions in the cerebrovascular tissue located in the inner white matter of the brain, which are usually visible on computed tomography (CT ) scan or magnetic resonance imaging (MRI) .

A tentative diagnosis of Binswanger disease is made upon an evaluation of patient history and symptoms. A definitive diagnosis is made upon autopsy that reveals lesions in cerebrovascular tissue lying in the subcortical regions of the brain. Lesions are not always confined to subcortical areas and additional lesions also may extend into cortical areas.

Treatment team

The treatment team for patients suffering from dementia, either cortical or subcortical, usually includes physicians, nurses, and physical, speech, and occupational therapists.

The diagnosis of Binswanger disease is often made by a neurologist . Physical therapists evaluate deficits in strength, movement, and gait, and supervise exercises to improve these deficits. Speech-language pathologists evaluate deficits in the ability to eat and speak, and provide adaptive strategies to minimize their effects. Occupational therapists evaluate a person's ability to maintain posture and focus while executing normal activities of daily living (such as reaching for and using a toothbrush) and devise strategic movements and equipment to adapt to deficits.

An expanded network of professionals, including mental health counselors and social service workers, may be beneficial. Caregivers are often required for personal care during the late stages of the disease.

Treatment

There is no known cure or specific treatment for Binswanger disease. Patients are treated symptomatically, i.e., treated for the symptoms such as high blood pressure, seizures, or heart disease often associated with Binswanger disease.

In most cases, specialized treatment plans include medications to control mood swings and depression, blood pressure (both elevated and low), seizures, and rhythm irregularities in the heart. Treatment is designed to reduce the adverse effects of these associated conditions.

Recovery and rehabilitation

Although currently no cure exists for dementias such as the Binswanger type, the goal of therapy is to maintain the highest state of physical health by managing the symptoms, along with maintaining the highest possible state of functional activity and well being. In addition to physical and occupational therapy, treatment for mood swings or depression helps the person with Binswanger disease to remain active, socially engaged, and mobile for as long as possible.

When the disease progresses and mobility, along with mental ability, decreases, the person with Binswanger or

other dementias will likely require a nurturing environment that provides for medical care and safety. Whether at home or in a care facility, personal care assistance may be necessary for many or all hours of the day.

Many communities have adult daycare centers with targeted, stimulating activities for persons with dementia in the early stages. Long-term care facilities that specialize in dementia can provide an environment that fosters mobility in a soothing environment, where staff provides cues to orient the person with dementia to memories and surroundings.

Clinical trials

Research on a wide range of neurological diseases, including dementias, is conducted by agencies of the National Institutes of Health such as the National Institute of Neurological Disorders and Stroke (NINDS), and other institutes and research organizations such as the National Institute on Aging and the National Institute of Mental Health. As of November 2003, scientists at the National Institute of Neurological Disorders and Stroke are reevaluating the definitions for many forms of dementia, including Binswanger disease.

Prognosis

Because there is no known specific cure for Binswanger disease, in most cases the disease follows a slowly progressing course during which a patient may suffer progressive strokes interspersed with periods of partial recovery. Once symptoms become visible (manifest), persons with Binswanger disease often die within five years of the onset of the disease.

Resources

OTHER

BBC News: Health and Medical Notes. "Binswanger's Disease." April 12, 1999. (November 13, 2003 [June 1, [2004].) <http://news.bbc.co.uk/1/hi/health/medical_notes/317488.stm>.

National Institute of Neurological Disorders and Stroke (NINDS)/National Institutes of Health. "Binswanger's Disease." November 8, 2002. (November 13, 2003 [June 1, 2004].) <http://www.ninds.nih.gov/health_and_medical/disorders/binswang_doc.htm>.

ORGANIZATIONS

Alzheimer's Association. 919 North Michigan Avenue, Suite 1100, Chicago, IL 60611-1676. (312) 335-8700 or (800) 272-3900; Fax: (312) 335-1110. info@alz.org. <http://www.alz.org>.

Alzheimer's Disease Education and Referral Center (ADEAR). P.O. Box 8250, Silver Spring, MD 20907-8250. (301) 495-3311 or (800) 438-4380; Fax: (301) 495-3334. adear@alzheimers.org. <http://www.alzheimers.org>.

Family Caregiver Alliance. 690 Market Street / Suite 600, San Francisco, CA 94104. (415) 434-3388 or (800) 445-8106; Fax: (415) 434-3508. info@caregiver.org. <http://www.caregiver.org>.

National Institute of Neurological Disorders and Stroke (NINDS) at the National Institutes of Health. P.O. Box 5801, Bethesda, MD 20824; (301) 496-5751 or (800) 352-9424; TTY (301) 468-5981. braininfo@ninds.nih.gov. <http://www.ninds.nih.gov/>.

National Organization for Rare Disorders (NORD). 55 Kenosia Avenue, Danbury, CT 06813-1968. (203) 744-0100 or (800) 999-NORD; Fax: (203) 798-2291. orphan@rarediseases.org. <http://www.rarediseases.org>.

Paul Arthur