Lewy Body Disease: Walking The Dementia Tightrope

Lewy Body Dementia

by Paul Raia, PhD

Life itself is a question of balance. The equilibrium of our solar system, the ebb and flow of the tides, the fragile relationships between ecosystems, the intricacies of maintaining physiological and emotional wellness, all a question of balance.

Lewy Body Disease causes many systems in our body to spiral out of balance. Abnormal proteins called Lewy bodies are thought to be involved in, if not the cause of, two separate but related diseases — Parkinson’s disease and Dementia with Lewy bodies (DLB). Both conditions have these abnormal protein deposits named for the physician, Fredrick H. Lewy, MD, who first saw them under the microscope in the early 1900s in the parts of the brain that control thinking, memory and movement.

The presence of these Lewy bodies can cause degenerative Parkinson’s disease with no, or only slight, cognitive problems; a progressive and terminal dementia; or a combination of the two: full-blown dementia with significant degenerative motor problems.

Typically, the early symptoms of DLB include:

  • visual hallucinations;
  • problems with attention and carrying out multiple stepped tasks;
  • spontaneous symptoms of Parkinson’s disease that come and go;
  • significant sleep disorder;
  • frequent falls;
  • unpredictable loss of consciousness;
  • dramatic sensitivity to drugs, especially antipsychotic drugs that might control hallucinations;
  • problems with visual-spatial tracking of objects, depth perception and other visual deficits.

The main hallmark of DLB is that there is no balance. The symptoms fluctuate dramatically and unpredictably over the 6- to 10-year life expectancy of the disease. Family caregivers and patients call it the “roller coaster” disease: for one day, one week, one month, you’re better than you were earlier, and then your symptoms worsen and your ability to function plummets.

Medical management of the disease is difficult. Patients can benefit from the type of medicine typically given to Alzheimer’s patients that increases the amount of neurotransmitters (natural chemicals that serve as information pathways in the brain). However, these patients are typically very sensitive to other medications that could be helpful in their treatment of symptoms, particularly antipsychotics, hypnotics (sleep aides), certain antidepressants and drugs to help movement disorders. Physicians have to walk that tightrope, balancing a drug that may help one symptom against the negative side effects on another symptom, and, to make matters all the more difficult, symptoms change without warning, by the day, by the week, by the month.

DLB is a veritable Magic Mountain of a roller coaster ride for the patients and the caregivers because there is no predictability in the trajectory of the disease. One week you hit your spouse and fall out of bed at night, and the next week you might sleep like a baby. It’s the unpredictability, the imbalance of one’s life that wears down the family and the patient in time.

Yet another lopsidedness of DLB is the lack of awareness within the medical community and amongst the public at large. Even though DLB affects 1.5 million Americans and is the second most common form of dementia, it is rarely diagnosed and there is little research on treatments or a cure. This is most likely because all forms of neurodegenerative brain diseases, like DLB in elderly populations, are lumped together as dementia or as Alzheimer’s disease. My guess is that we will find many more cases of DLB as we educate primary care doctors about the unique symptoms and develop better diagnostics, particularly brain-imaging technologies.

Take Nora, for example, a close friend of mine. She was 59 when she first showed changes in her emotional status. She became very anxious and sad. She had difficulty organizing and carrying out tasks. She had visual problems, especially focusing on or following objects. Her sense of balance was slightly off, but that was attributed to her visual problem. Her short-term memory was impaired but not dramatically so. The family history showed several close relatives who died of Alzheimer’s disease. Her psychiatrist and her neurologist were convinced she had Alzheimer’s disease. No hallucinations, no good days/bad days, no sleep disturbance, no movement disorder, but a slight tremor that we attributed to the anxiety. After needed surgery for a broken arm, Nora died rather suddenly. Post mortem autopsy showed no plagues or tangles (abnormal proteins in the brain cells) characteristic of Alzheimer’s disease, but a brain riddled with Lewy bodies. In retrospect, it may have been the characteristic allergic reaction to the anesthesia used during surgery to repair the shattered arm that killed her. This can happen in individuals with DLB. Nora had only minor symptoms of DLB, but a brain full of Lewy bodies.

Distinguishing between various forms of dementia is important for families and patients. For example, a physician may not want to use an antipsychotic medication typically used to treat visual hallucinations because of the risk that class of medication carries for those with Lewy body dementia. Each form of dementing illness has its own set of symptoms, its own prognosis, its own rate of decline, its own genetic consequences for families, and its own unique problems to overcome. Knowing what form of dementia, or combination of dementias, the individual is coping with enables a family to better plan for the future, to better understand and address specific behaviors, and for medical teams to better treat the affected individual.

For family members, it is important in most cases to accompany the person being diagnosed for a confirmation of the symptoms reported by the patient. Family members should write out the symptoms they want the doctor to know about, as well as any new medications or any medical issues in general, and bring their notes to the appointment. Key symptoms to report: changes in balance, tremors, muscle jerks, sleep movements, visual hallucinations, any change in cognitive abilities.

Various types of brain scans can identify specific areas of the brain that are damaged, and that information can be useful in determining between Dementia with Lewy bodies and Alzheimer’s. However, for the most part, the diagnosis is made clinically, that is, from an accurate reporting of symptoms, a family history, and observations made by the doctor. A neurologist that specializes in geriatrics or dementia may be the most clinically experienced to pick up the subtle differences between the various types of dementing illnesses in their early stages. Unfortunately, at this point in time, only an autopsy can identify the dementing illness with certainty.


Dr. Paul Raia is Vice President, Professional Clinical Services of the Alzheimer’s Association, MA/NH chapter, and is also the chapter’s Director of Patient Care and Family Support.


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