Stephanie Bowens | StarNews Correspondent
When someone has dementia, or severe loss of memory and impairment in mental and behavioral abilities significant enough to interfere with daily function, it can dramatically impact the persons life and the lives of their loved ones. Dementia with Lewy Bodies, or DLB, is one of the most common causes of dementia and affects over a million people in the US.
Dr. Mark Pippenger, a behavioral neurologist at Novant Health Memory Care, has worked in behavioral neurology for 20 years. He said DLB is a form of dementia, a degenerative brain disease that affects memory and thinking, characterized by the presence of Lewy bodies in brain cells in certain regions of the brain.
Ive seen a lot of people who have the disease, and it has a devastating impact on patients and their families, he said, adding hes hopeful the boost in interest and contributions toward research efforts that followed the diagnosis of actor Robin Williams will lead to more effective treatment soon.
According to the U.S National Library of Medicine (NLM), Lewy bodies are abnormal deposits of a protein called alpha-synuclein. Pippenger said Lewy Bodies, named after Dr. Friederich Lewy, a neurologist who discovered them in 1912, are found inside brain cells or neurons.
For many years we only associated Lewy bodies with Parkinsons disease until the 1970s when it started to become clear that some people had a (different) form of dementia that sometimes included symptoms similar to Parkinsons disease, Pippenger said.
National Institute on Aging said Lewy bodies affect several brain regions including the cerebral cortex, which controls many functions, including information processing, perception, thought, and language, the limbic cortex, which plays a major role in emotions and behavior, the hippocampus, essential in forming new memories, the midbrain and basal ganglia, which are involved in movement, the brain stem, which is vital in maintaining alertness and regulating sleep, and brain regions needed to recognize smells.
DLB is characterized by a decline in intellectual function (dementia), movement problems called parkinsonism, visual hallucinations, fluctuations in behavior and intellectual ability, alertness and attention, and REM sleep behavior disorder.
Earliest signs well typically see include the REM behavior (Disorder), the acting out dreams during sleep, Pippenger said. That can come on years before any of the other signs of dementia with Lewy bodies.
Pippenger said sometimes hallucinations are the first symptoms people have with DLB. According to NIA, early in the course of the disease people with DLB, may have trouble with mental activities such as multitasking and problem solving. NLM said dementia, or intellectual decline, may start mild or seem to come and go, and problems with memory typically occur later.
Parkinsonism or movement changes seen in Parkinsons disease, is another common symptom of DLB. These include tremor or muscle stiffness, unusually slow movement, impaired balance and coordination and difficulty walking.
DLB is usually clinically diagnosed by looking at symptoms and medical history. Currently, while tests can be performed to support a diagnosis, no single test can conclusively diagnose the disease, except an autopsy.
Pippenger said to be diagnosed with DLB, patients must have two out of four core features.
Those four features include spontaneous visual hallucinations seeing things that are not really there, he said. The second is spontaneous parkinsonism, symptoms similar to Parkinsons disease, usually slowness of movement and stiffness in the muscles, but sometimes also shaking or tremor. The third core feature is called REM behavior disorder, and thats when people act out their dreams during their sleep, and the fourth core feature is strong fluctuation or change in the level of impairment or level of consciousness over time. So, people are a lot better one week and a lot worse the next week and better again the week after that, so it fluctuates.
Researchers dont know exactly why alpha-synuclein accumulates into Lewy bodies or how Lewy bodies cause the symptoms of DLB, but Pippenger said while the exact cause of DLB is unknown, some risk factors are aging and gender. He said the typical age of onset is between 50 and 85 years old, and men are more likely to get DLB than women.
Treatment plans may include medications, various types of therapy, including physical therapy or speech therapy, and counseling.
Drugs that we use to treat Alzheimers disease appear to have benefits for people who have Dementia with Lewy Bodies, and those drugs include one called Donepezil … It doesnt appear to affect the underlying disease, but it appears to delay symptoms, Pippenger said.
Donepezil may also reduce hallucinations.
Lewy bodies can often be found in brains of people diagnosed with other forms of dementia. DLB shares similar symptoms with Alzheimers disease and Parkinsons Disease, making differentiating between these diseases challenging.
According to NIA, someones decline in thinking ability in DLB may look similar to Alzheimer's disease, but over time development of movement issues and other distinguishing symptomssuggest a DLB diagnosis. Early symptoms also differ.
Alzheimers disease rarely produces hallucinations early in the disease, Pippenger said. Alzheimers also usually doesnt cause parkinsonism early in the disease.
People who have dementia with Lewy Bodies early on have a lot more trouble with processing visual input, so they have more trouble seeing things correctly, Pippenger added.
He said people with DLB also tend to have a harder time figuring how to do multi-step tasks, such as making a sandwich.
Timing of symptoms helps distinguish between Parkinsons, dementia and DLB.
Dementia comes within a year of the parkinsonism in Dementia with Lewy bodies, but with Parkinsons disease dementia, people have Parkinsons disease for over a year before they get dementia or memory and thinking problems, Pippenger said.
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HEALTH WATCH: 5 Things to Know About Dementia with Lewy Bodies - StarNewsOnline.com
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