Read Full Text: Lewy body dementias: Dementia with lewy bodies and Parkinson disease dementia
Journal: CONTINUUM Lifelong Learning in Neurology
Year Published: 2016
Ranked 21st on our 2016-2021 list of the 100 Most Influential OT Research Articles
Lewy body dementias are common, being the second most common form of dementia after Alzheimer’s disease.
That’s why it’s so surprising that Lewy body dementias often slip under the radar in the medical community. Patients often have difficulty finding health professionals familiar with the complex course of the disease. (Case in point: Robin Williams struggled with hallmarks of Lewy body dementia, but he was not diagnosed until after his death.)
This week’s article orients us to Lewy body dementias so we have a baseline understanding to work with—and it also delves into the unique role of OT. There are many ways that OTs can help patients navigate this challenging disease course by staying true to what we do best: listening to patients and prioritizing their dignity and goals.
Let’s dive in.
What was the purpose of this article?
This article was written in Continuum, The American Board of Psychiatry and Neurology has designated as “approved reading” for continuing medical education for doctors. (You can see what doctors are reading about both the disease and our role as OTs!)
The article provides an overview of the following aspects of Lewy body dementias:
- Clinical features
- Neuropathological findings
- Diagnosis
- Treatment
For our purposes, we will focus on clinical features and treatment.
What are Lewy body dementias?
Lewy bodies are abnormal clumps of protein that form in the brain and interfere with normal brain function.
Approximately 1.4 million Americans are diagnosed with one of the Lewy body dementias.
(In contrast, about 5.5 million Americans are diagnosed with the leading cause of dementia, Alzheimer’s disease (AD), which is characterized by a buildup of plaques in the brain.)
There are two main types of Lewy body dementias:
- Dementia with Lewy bodies (DLB)
- Parkinson’s disease dementia (PDD)
The article does not go in depth on this, but a form of Lewy body dementia is also found in Multiple system atrophy (MSA). You can see our previous review on MSA here, as the disorder has its own distinct and challenging course.
Quick side note: While the article did not mention this, but I think it is helpful to know that one major study found that the average survival time of patients with dementia with Lewy bodies was also shorter than that of patients with Alzheimer’s disease. (DLB patients typically live 7.7 years after the onset of cognitive symptoms, while AD patients typically live 9.3 years after the onset of cognitive symptoms.)
Timing of onset matters when differentiating the two primary types of Lewy body dementia
The main difference between the two types of Lewy body dementia has to do with the onset of the parkinsonism symptoms.
DLB patients typically initially develop cognitive impairment and visual hallucinations. Parkinsonism develops simultaneously or soon thereafter.
In PDD patients, the parkinsonism emerges first, by at least a year, and is then followed by cognitive symptoms.
Clinical features of Lewy body dementias
The article breaks down clinical features for both DBL and PDD, but because the presentation is so similar, I am combining them for you. But, for more nuance please read the article in its entirety.
Cognitive symptoms:
Progressive cognitive decline begins relatively early, though it typically begins after age 55.
Multiple domains of cognition are often impacted, including:
- Short-term memory
- Attention
- Executive function
- Visual spatial skills
Patients’ earliest complaints tend to involve things like: difficulty multitasking, losing the thread of conversations, and getting lost while driving.
Interestingly, the short-term memory difficulty seems to impact the retrieval of memories, rather than the process of encoding and storing them. So, cueing can be particularly helpful, as the memory is there it just needs extra help to be accessed.
Neuropsychiatric symptoms:
Hallucinations
Recurrent, complex hallucinations are common. These hallucinations are usually well-formed and animate. It is common for them to include:
- Adults
- Small children
- Deceased family members
- Small animals
They are typically visual and unimodal (meaning they present without sound, smell, touch, etc.) They are frequently well tolerated and emotionally neutral—but, obviously, they can also be fear-provoking and disconcerting.
Delusions
Delusion can occur later in the disease course. Unlike hallucinations (where the patient experiences something that isn’t there), a delusion is a false belief or interpretation that continues to be held despite evidence that it is false. These delusions typically have a paranoid quality and can involve false beliefs of:
- Infidelity
- House intruders
- Theft
Patients may also experience Capgras Syndrome, where they believe that their spouse or caregiver has been replaced by an imposter.
Fluctuations in attention and arousal:
Attention and alertness may fluctuate throughout the day, leading to episodes of “staring and perturbed flow of ideas” and/or frequent daytime drowsiness and napping.
See the assessments section below for a screen related to this.
Motor features of parkinsonism:
The parkinsonism symptoms that often occur alongside or shortly after cognitive decline can include:
- Bradykinesia
- Gait impairments
- Resting tremor
- Pill rolling tremor
Medication sensitivity—especially to neuroleptics
OTs typically don’t spend too much time thinking about medication. But, I wanted to highlight the fact that patients with LBD often have sensitivity to neuroleptics (anti-psychotics). This is one of the reasons that arriving at a correct diagnosis is so important.
If a patient were to arrive at an ER with some of the psychiatric symptoms listed above, they may likely be given an antipsychotic. But, DLB patients tend to have an extreme negative reaction to these medications, and they are at increased risk for neuroleptic malignant syndrome.
The article goes so far as to say that patients and their caregivers should be taught that LBD patients are considered allergic to neuroleptics.
Other associated symptoms:
Rapid eye movement (REM) sleep behavior disorder, loss of smell, and constipation are also common. These associated symptoms may start years before onset of the other hallmark symptoms. Here’s a little more info on two broad categories of associated symptoms:
Autonomic Impairment
Autonomic impairment is common in Lewy body dementias, but no as profound as in multiple system atrophy (MSA). These symptoms can include:
- Constipation
- Orthostatic hypotension
- Resulting in syncope and falls
- Neurogenic urinary frequency or incontinence
Rapid eye movement sleep disorder
In this disorder, the normal paralysis of REM sleep is impaired. As a result, patients may act out their dreams, with behaviors such as kicking, punching and yelling.
Treatment
There is no cure for Lewy body dementia, nor are there options to modify the overall disease progression. So, treatment is focused on managing symptoms.
Both medication and nonpharmacological approaches are recommended for symptom management. The article has a helpful chart that breaks down management options based on symptoms.
With all conditions, it is generally wise to try nonpharmacological options before medications, but you’ll see that this approach is particularly important for LBD patients. They tend to be not only sensitive to neuroleptics, but to medication in general.
For our purposes, I am going to highlight where OT is specifically mentioned, and then I’ll discuss areas where nonpharmacological treatment is mentioned and I believe OTs can play an important role in care.
Occupational therapy for motor symptoms:
Occupational therapy is specifically mentioned for managing motor symptoms. The article calls out our ability to help with basic functions, including feeding. It also highlights the benefit of an OT home evaluation. Here’s the precise wording:
“Home safety evaluations can be useful to identify and remove fall hazards, such as loose throw rugs, to add aids such as grab bars and shower stools, and in the setting of dementia, to evaluate for the need to remove hazards such as gas stoves.”
The article also mentions exercise as being highly beneficial.
Management of insomnia:
The article states that before medication is started, sleep hygiene should be optimized. I believe OTs have a lot to offer in the realm of sleep management. And, I can see this being a very valuable part of a home visit.
Management of dysautonomia:
Again, behavioral strategies is the first option listed. The article specifically mentions:
- Promoting hydration
- Transitioning slowly from various body positions
- Leg crossing (in OT we call these countermeasures)
- Compression stockings or abdominal binders
Author’s conclusions
The author concluded that disease-modifying therapies remain a major unmet need in this population.
And, in the meantime, treatment strategies targeting specific symptoms need to be carefully selected to avoid the worsening of other domains of impairment.
Takeaways for OT practitioners
(Please note: these are my personal takeaways and were not mentioned specifically in the article.)
1. For patients and caregivers, it may be powerful simply to have a healthcare professional (you) who understands so many aspects of the disease course.
Throughout this article, I kept thinking of the many patients and caregivers who have had to manage this complex condition without an accurate diagnosis.
It made me hope that each patient and family member in the future will have someone like you to help walk alongside them throughout the progression of this disease. Robin Williams’ wife, Susan, outlined their story of navigating the condition without an accurate diagnosis in her article, The terrorist inside my husband’s brain. This line from the article stood out to me:
“How I wish he could have known why he was struggling, that it was not a weakness in his heart, spirit, or character.”
2. As an OT, there are many ways you can help these patients stay safe, engaged, and connected.
The range of symptoms really calls on you to draw on your full skill set—from helping create cognitive cues, to assisting patients as they manage sleep and dysautonomia.
But, with all of the specific strategies you can take, it is important to bring it back to the big overall question that I find incredibly helpful to ask our dementia patients:
How can we help you stay safe, connected, and engaged?
3. Plan to involve the caregiver in all aspects of your therapy.
I thought that a major oversight of this article was the failure to bring up the tremendous role that is so often played by informal caregivers—with LBD and with all types of dementia.
I thought our Club member, Brandy Archie, had very useful advice on involving the caregiver in this podcast episode. And, I definitely heard her voice and advice in my head as I was reading this article. If you haven’t yet listened, I recommend the episode: Supporting Informal Caregivers of People with Dementia.
Here’s the full APA citation for this article:
Gomperts SN. Lewy Body Dementias: Dementia With Lewy Bodies and Parkinson Disease Dementia. Continuum (Minneap Minn). 2016 Apr;22(2 Dementia):435-63. doi: 10.1212/CON.0000000000000309. PMID: 27042903; PMCID: PMC5390937.