Dementia With Lewy Bodies

What is Dementia With Lewy Bodies?

Dementia with Lewy bodies (DLB)( diffuse Lewy body disease, Lewy body variant of Alzheimer's disease) is becoming increasingly noted as a common dementia after that of Alzheimer's disease. Depending on clinical settings, it is thought to be the third most common neurodegenerative dementia. ( The first is Alzheimer's disease, the second mixed dementia ( AD/VaD) and then LBD). In some studies LBD accounted for 15% to 20%of autopsy cases, and 35% of the dementia cases in population based studies. The central feature of DLB is progressive cognitive decline, combined with three additional defining features: (1) pronounced “fluctuations” in alertness and attention, such as frequent drowsiness, lethargy, lengthy periods of time spent staring into space, or disorganized speech; (2) recurrent visual hallucinations, and (3) parkinsonian motor symptoms, such as rigidity and the loss of spontaneous movement. Most of the time, the dementia and parkinsonian signs appear to develop simultaneously.

Similar to AD the incidence of LBD also appears to increase with age,and may be more common in men. Clinically one sees a more rapid deterioration in persons with LBD, thought to be due to the emergence and deterioration of motor functioning due to parkinsonian signs. It is not uncommon to see more disability, including falls in persons with LBD.

To date, there have been two workshops on establishing diagnositic criteria for LBD. Further, in addition to the dementia and parkinsonian signs, these patients often have fluctuating cognition, depression, REM ( rapid eye movement) sleep disturbances, hallucinations, sensitivity to neuroleptics, and changes on PET scanning.

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.

Is there any 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.

What is the 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 can be similar to that in Alzheimer’s disease, however in most cases due to progressively increasing disability, it continues at a more rapid rate, with death in 6-8 years.

What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS) conducts research related to DLB in laboratories at the NIH and also supports 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.

With Lewy Bodies%22 Select this link to view a list of studies currently seeking patients.