Lewy Body Dementia

Is Lewy body dementia a rare disease?
Lewy body dementia (LBD) affects 1.3 million Americans and their families and many millions more worldwide, making it the second leading cause of progressive dementia after Alzheimer’s disease. Lewy body dementia is an umbrella term for two related diseases: dementia with Lewy bodies and Parkinson’s disease with dementia. Over time, people with both diagnoses will develop very similar symptoms.
If it’s the second leading cause of progressive dementia, why haven’t I ever heard of it?
Because Lewy body dementia can closely resemble both Alzheimer’s disease and Parkinson’s disease, it is widely under-diagnosed. In a caregiver survey conducted by the Lewy Body Dementia Association, it took an average of eighteen months and visits to three different physicians to arrive at a diagnosis of Lewy body dementia. Many people with Lewy body dementia are mistakenly given a diagnosis of Alzheimer’s disease or Parkinson’s disease or both.
What are Lewy bodies and who is Lewy?
Friederich Lewy (1885-1950) was a German scientist who discovered abnormal protein deposits that interfere with brain activity in people with Parkinson’s disease and Lewy body dementia. In Parkinson’s disease, Lewy body proteins appear in the part of the brainstem that produces the neurotransmitter dopamine and inhibits its production. In Lewy body dementia, the abnormal proteins appear in other parts of the brain as well, including the cerebral cortex where it can affect memory, thought, language, attention, and awareness. In Parkinson’s disease dementia, physical symptoms generally occur first. In Lewy body dementia, cognitive symptoms more often precede the Parkinson-like physical symptoms.
Why is a correct diagnosis of LBD important?
Many people with LBD are highly sensitive to medications, especially to antipsychotic drugs also called neuroleptics. Adverse reactions to antipsychotics may include worsening cognition, heavy sedation, and irreversible worsened Parkinsonian symptoms in some people who have Lewy body disease. Other medications, such as those used for Parkinson’s disease, surgical anesthetics, and some over-the-counter medications can have significant adverse reactions as well.
What are the main features of Lewy body dementia?
The central feature is dementia—a progressive decline of cognitive abilities, including the ability to plan or perform abstract or analytical thinking.
The three core features are fluctuating cognition (wide variations in attention and alertness), recurrent visual hallucinations (often detailed and usually present in the early stages of the disease), and Parkinsonism (muscle stiffness and rigidity, slow movements, problems with balance, shuffling gait, stooped posture, tremor, and blank facial expression).
Suggestive features include REM sleep behavior disorder, severe sensitivity to certain neuroleptics, and abnormal results on PET or SPECT brain scans.
Other possible features include: weak voice, difficulty swallowing, delusions, fainting, repeated falls, urinary problems, depression, and restless leg syndrome.
Are there any treatments for Lewy body disease?
Some medications for Alzheimer’s disease and Parkinson’s disease may help people with Lewy body disease, though others may suffer serious adverse side effects from these drugs, as mentioned above. An LBD-experienced physician should manage treatment and the administration of all medications for a person with LBD. Some additional helpful treatments include massage, music, aromatherapy, exercise, and physical therapy.
What is the prognosis?
Lewy body dementia is a progressive neurological disease, though the rate of progression varies greatly. There is no way to predict the path of the disease for any given individual. On average however, the disease has a duration of 5 to 7 years after diagnosis, though progression can vary from 2 to 20 years.

For more information, please contact:

Lewy Body Dementia Association, Inc.
P.O. Box 451429
Atlanta, GA 31145-9429

Website: lbda.org
Caregiver helpline: 800.LEWY.SOS (800.539.9767)