Dementia with Lewy bodies (patient information)

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Editor-in-Chief: C. Michael Gibson, M.S.,M.D. [mailto:mgibson@perfuse.org] Phone:617-632-7753; Associate Editor-In-Chief: Lakshmi Gopalakrishnan, M.B.B.S.

Overview

 * Dementia with Lewy bodies (DLB) is one of the most common types of progressive dementia.
 * Lewy body disease usually begins between the ages of 50 and 85. The disease gets worse over time.
 * The criteria for DLB is dementia symptoms and 2 or 3 of the below:
 * Parkinsonism: slowness and stiffness
 * Recurrent detailed visual hallucinations, delusions (false beliefs)
 * Symptoms fluctuate markedly from day to day

What causes Dementia with Lewy bodies?

 * The symptoms of DLB are caused by the build-up of Lewy bodies which are 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.


 * DLB usually occurs sporadically, in people with no known family history of the disease. However, rare familial cases have occasionally been reported.

What are the symptoms of Dementia with Lewy bodies?

 * Brief episodes of confusion and orientation to time or place
 * Mild memory problems
 * Well defined, vivid hallucinations
 * Tremors or rigidity (especially if Haldol or other anti-psychotics given)
 * Person has rigid, false beliefs that can’t be changed by convincing
 * Unexplained loss of consciousness, frequent falls
 * Symptoms can be temporary - vary from day to day or even throughout the day

Diagnosis

 * The central feature of DLB is progressive cognitive decline, combined with three additional defining features:
 * Pronounced fluctuations in alertness and attention, such as frequent drowsiness, lethargy, lengthy periods of time spent staring into space, or disorganized speech;
 * Recurrent visual hallucinations, and
 * Parkinsonian motor symptoms, such as rigidity and the loss of spontaneous movement.


 * People may also suffer from depression.

Treatment options

 * 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.

Diseases with similar symptoms

 * 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.

Where to find medical care for Dementia with Lewy bodies?
Directions to Hospitals Treating Dementia with Lewy bodies

What to expect (Outlook/Prognosis)?

 * Like Alzheimer’s disease and Parkinson’s disease, DLB is a neuro-degenerative 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.