Dementia With Lewy Bodies

Dementia with Lewy bodies (DLB) is one of the most common types of progressive dementia. The central features of DLB include progressive cognitive decline, “fluctuations” in alertness and attention, visual hallucinations, and parkinsonian motor symptoms, such as slowness of movement, difficulty walking, or rigidity.  People may also suffer from depression.  The symptoms of DLB are caused by the build-up of Lewy bodies – accumulated bits of alpha-synuclein protein -- inside the nuclei of neurons in areas of the brain that control particular aspects of memory and motor control.  Researchers don’t know exactly why alpha-synuclein accumulates into Lewy bodies or how Lewy bodies cause the symptoms of DLB, but they do know that alpha-synuclein accumulation is also linked to Parkinson's disease, multiple system atrophy, and several other disorders, which are referred to as the "synucleinopathies." The similarity of symptoms between DLB and Parkinson’s disease, and between DLB and Alzheimer’s disease, can often make it difficult for a doctor to make a definitive diagnosis. In addition, Lewy bodies are often also found in the brains of people with Parkinson's and Alzheimer’s diseases.  These findings suggest that either DLB is related to these other causes of dementia or that an individual can have both diseases at the same time.  DLB usually occurs sporadically, in people with no known family history of the disease. However, rare familial cases have occasionally been reported.

Treatment

There is no cure for DLB.  Treatments are aimed at controlling the cognitive, psychiatric, and motor symptoms of the disorder. Acetylcholinesterase inhibitors, such as donepezil and rivastigmine, are primarily used to treat the cognitive symptoms of DLB, but they may also be of some benefit in reducing the psychiatric and motor symptoms.  Doctors tend to avoid prescribing antipsychotics for hallucinatory symptoms of DLB because of the risk that neuroleptic sensitivity could worsen the motor symptoms.  Some individuals with DLB may benefit from the use of levodopa for their rigidity and loss of spontaneous movement.   

Prognosis

Like Alzheimer’s disease and Parkinson’s disease, DLB is a neurodegenerative disorder that results in progressive intellectual and functional deterioration.  There are no known therapies to stop or slow the progression of DLB.  Average survival after the time of diagnosis is similar to that in Alzheimer’s disease, about 8 years, with progressively increasing disability. 

Research

The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health conduct research related to DLB in laboratories at the NIH and support additional research through grants to major medical institutions across the country.  Much of this research focuses on searching for the genetic roots of DLB, exploring the molecular mechanisms of alpha-synuclein accumulation, and discovering how Lewy bodies cause the particular symptoms of DLB and the other synucleinopathies. The goal of NINDS research is to find better ways to prevent, treat, and ultimately cure disorders such as DLB.

Organizations

Lewy Body Dementia Association
Supports those affected by Lewy body dementias through outreach, education and research.

912 Killian Hill Road, S.W.
Lilburn, GA 30047
lbda@lbda.org
http://www.lbda.org
Tel: Lilburn
Fax: 480-422-5434

Alzheimer's Disease Education and Referral Center (ADEAR)

National Institute on Aging
P.O. Box 8250
Silver Spring, MD 20907-8250
adear@nia.nih.gov
http://www.nia.nih.gov/alzheimers
Tel: Silver Spring
Fax: 301-495-3334

Alzheimer's Association
National voluntary health organization committed to finding a cure for Alzheimer’s and helping those affected by the disease.

225 North Michigan Avenue
Floor 17
Chicago, IL 60601-7633
info@alz.org
http://www.alz.org
Tel: Chicago
Fax: 866.699.1246

Family Caregiver Alliance/ National Center on Caregiving
Supports and assists families and caregivers of adults with debilitating health conditions. Offers programs and consultation on caregiving issues at local, state, and national levels. Offers free publications and support online, including a national directory of publicly funded caregiver support programs.

785 Market St.
Suite 750
San Francisco, CA 94103
info@caregiver.org
http://www.caregiver.org
Tel: San Francisco
Fax: 415-434-3508

Content Provided By

logo

NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.

Neurological Disorders and Stroke »