Category Archives: LBD

Red flags suggesting atypical parkinsonism, and treatment of DLB, MSA, PSP, and CBD

Date
Posted by
Red flags suggesting atypical parkinsonism, and treatment of DLB, MSA, PSP, and CBD

In a recent issue of the “Handbook of Clinical Neurology,” a chapter is devoted to the four atypical parkinsonism disorders — dementia with Lewy bodies (DLB), multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The authors — both movement disorder specialists at the University of Florida — indicate that “diagnosis is critical as the atypical parkinsonisms herald symptom profiles and prognoses distinct” from Parkinson’s Disease (PD). They also indicate it’s important to identify the suspected underlying pathology — whether it be alpha-synuclein (for DLB and MSA) or tau (for PSP and CBD). Identifying the underlying pathology influences clinical trial enrollment.

Here’s a link to an abstract on PubMed about the chapter:

https://www.ncbi.nlm.nih.gov/pubmed/31753139
Recognizing and treating atypical Parkinson disorders.
Armstrong MJ, McFarland N.
Handb Clin Neurol. 2019;167:301-320.

(There is a $32 fee to download the full article.)

The authors, Drs. Melissa Armstrong and Nikolas McFarland, point out that one reason to know whether you are dealing with an atypical parkinsonism disorder rather than PD is that the survival time is less than 10 years on average and that most die from disease-related issues such as falls, aspiration pneumonia, and failure to thrive.

This statement of concern about caregivers is interesting:

“Given patients’ mounting disability over time and the need for increasing support for both instrumental activities of daily living and basic activities of daily living, caregiver support is critical. In some circumstances, clinicians will need to assist couples in identifying backup caregivers to prepare for the possibility that older caregivers may themselves experience health concerns.”

PHARMACOLOGIC TREATMENT

Pharmacologic treatment is entirely symptomatic, and includes:

MOTOR

* Parkinsonian symptoms: levodopa

* Dystonia: dopaminergic agents; botulinum toxin injections; anticholinergic agents (in younger people with MSA)

* Myoclonus: valproic acid, levetiracetam, clonazepam

The authors note that deep brain stimulation is “rarely helpful” to those with atypical parkinsonism.

NON-MOTOR

* Depression: avoid tricyclic antidepressants in those with orthostatic hypotension

* Depression and Pain: duloxetine

* Depression, Sleep, and Weight Loss: mirtazapine

NONPHARMACOLOGIC TREATMENT

Nonpharmacologic treatment is focused on dysphagia (swallowing problems) and fall prevention. Treatment also includes physical therapy, occupational therapy, and speech-language pathology.

“The role of exercise in the atypical parkinsonisms is not well-researched to date, but increasing evidence of the benefits of exercise in people with PD suggests that there may be an important role for exercise in the atypical parkinsonisms, as well. Safety is a priority and guides selection of appropriate physical interventions.”

The authors encourage neurologists to discuss advance care directives with patients and families early on so that personal preferences are known. These directives should be re-discussed along the way. Palliative care and hospice can be helpful.

The chapter includes a helpful table for physicians on “Red flags suggesting an atypical parkinsonism.” That’s copied below.

Robin

————————-

Recognizing and treating atypical Parkinson disorders.
Armstrong MJ, McFarland N.
Handb Clin Neurol. 2019;167:301-320.

Table 16.1
Red flags suggesting an atypical parkinsonism (a)

Red flag Suggested atypical parkinsonism
Rapid disease progression Any atypical parkinsonism
Lack of a robust levodopa response Any atypical parkinsonism
Bilateral symmetric parkinsonism DLB, PSP
Early gait impairment, falls Any atypical parkinsonism (b)
REM sleep behavior disorder DLB, MSA
Early bulbar dysfunction PSP
Irregular, jerky tremor MSA, CBD
Myoclonus MSA, CBD (less common in DLB, PSP)
Supranuclear gaze palsy PSP, CBD
Dysautonomia DLB, MSA
Cerebellar signs MSA
Laryngeal stridor MSA
Perioral/facial levodopa-induced dyskinesias MSA
Early dementia DLB, PSP, CBD (b)
Apraxia of speech or progressive nonfluent aphasia PSP, CBD
Apraxia CBD, PSP
Alien limb phenomenon CBD
Higher cortical findings (e.g., agraphesthesia) CBD

(a) Most commonly associated diagnoses are listed but this does not exclude the possibility that an unlisted atypical parkinsonism could be associated with the red flag (for example, RBD is described as occurring in people with PSP, but it is more commonly associated with the synucleinopathies).

(b) Specific details of impairments may suggest a specific atypical parkinsonism.

CBD, corticobasal degeneration; DLB, dementia with Lewy bodies; MSA, multiple system atrophy; PSP, progressive supranuclear palsy.

Nine tips for traveling with family members impacted by dementia (AFA)

Date
Posted by
Nine tips for traveling with family members impacted by dementia (AFA)

The Alzheimer’s Foundation of America (AFA, alzfdn.org) offers nine tips for traveling with family members impacted by dementia.  These tips apply whether you are traveling near or far.

Robin

———————-

https://alzfdn.org/nine-thanksgiving-travel-tips-families-impacted-dementia/

Nine Thanksgiving Travel Tips for Families Impacted by Dementia

AFA offers the following tips for family caregivers to consider:

  • Advise airlines and hotels that you’re traveling with someone who has memory impairment and inform them of safety concerns and special needs.
  • Inquire in advance with airports/train stations about security screening procedures.  This way, you can familiarize the person beforehand about what will happen at the checkpoint to reduce potential anxiety.
  • Plan the travel mode and timing of your trip in a manner that causes the least amount of anxiety and stress.  Account for the person and their needs when making arrangements; if they travel better at a specific time of day, consider planning accordingly.
  • Preserve the person’s routine as best as possible, including eating and sleeping schedules. Small or unfamiliar changes can be overwhelming and stressful to someone with dementia.
  • Take regular breaks on road trips for food, bathroom visits, or rest.
  • Bring snacks, water, activities and other comfort items (i.e., a blanket or the person’s favorite sweater), as well as an extra, comfortable change of clothing to adapt to climate changes.
  • Consider utilizing an identification bracelet and clothing tags with your loved one’s full name and yours to ensure safety.
  • Take important health and legal-related documentation, a list of current medications, and physician information with you.
  • Depending on the trip duration and/or the stage of the person’s illness, consult with their physician to make sure travel is advisable.

3-step Framework for Diagnosing Dementia (Brad Dickerson, MD)

Date
Posted by
3-step Framework for Diagnosing Dementia (Brad Dickerson, MD)

In this five-minute video, Brad Dickerson, MD, a neurologist at Mass General, describes a 3-step framework for diagnosing dementia:

www.mdmag.com/peer-exchange/early-diagnosis-dementia/recommendations-for-diagnosing-alzheimer-disease

Here are excerpts from the transcript:

Currently, I think we advocate for a 3-step framework that starts by describing the person’s overall cognitive functional status. What we mean by that is, does the person have mild cognitive impairment? Does the person have dementia? … What we really need to do is interview the person and, ideally, an informant, and find out what they are lacking in terms of independent functioning. What have they lost? What do they need help with?

[This] ultimately has major implications for the care plan. Establishing whether the person has mild cognitive impairment or dementia is very important, and I think that threshold varies from person to person and can be quite an arbitrary decision that really takes some clinical experience. Ultimately, what I like to ask people is, if you, as the care partner, can leave the person and go on a trip for a weekend or a week, would they function independently at the things that they need to try to get done to get by in daily life? If the care partner says, “No, I would never do that,” you can pretty comfortably say that the person probably has crossed the threshold into dementia. I think that’s the starting point, No. 1.

No. 2 is, what’s the particular cognitive behavioral syndrome that the person is experiencing? …[Is] the main problem memory loss? Is the main problem executive function? Is the main problem language? Are there multiple problems? A lot of times we see, I think, this common presentation of a person who has memory loss. They’re just not holding on to information, and they also have executive dysfunction. They’re not able to reason. They’re not able to perform tasks to the level that they used to be able to in order to get the job done to reach goals in a valid way.

I think that the syndrome is really meant to capture the major symptoms and signs that the person has of their illness. And that communicates important information to our colleagues and to the patient and family about where their problems are. I think it also allows you to highlight…what their strengths still may be. If this person has a primary memory loss syndrome but their executive function is still good, maybe they can make use of strategies to compensate for some of the problems that they’re having with memory. If they have executive dysfunction, they’re probably not going to be able to do that. Ultimately, that cognitive behavioral syndrome, that second level of specificity in our diagnostic formulation, communicates, in shorthand, to us and to others what the person’s problems are and maybe what they can still do.

And then the third level is, what’s the brain disease that is the cause of the problem? Sometimes it’s multiple diseases. Often, it’s compounded by other medical problems or things like medication effects that affect brain function but are not necessarily a disease in and of itself. The most common, I think, is Alzheimer disease mixed with cerebrovascular pathology in an older adult population—people over the age of 70, 75. In the younger people, I think it can sometimes be a more pure condition, whether it’s Alzheimer disease, or frontotemporal degeneration, or Lewy body disease. Those can often be primary diseases, especially in younger people.

That’s really the 3-step formulation that we advocate that we try to follow. It’s not always possible to be 100% confident in any 1 of those levels, and I think that’s where we have to talk about likely due to Alzheimer disease or likely due to cerebrovascular disease, and rate our level of certainty so we can think about whether we need some additional specialty involvement. If so, what does that involve, and how important is that in thinking about the management? We don’t necessarily have to have the sophisticated biomarkers that we talk a lot about in every individual with dementia likely due to Alzheimer disease. I think there are plenty of people we can all diagnose with fairly straightforward assessments and tests and not do the multimillion-dollar work-up on, that we often end up spending time talking about in the more specialized cases.

 

Lewy Body Dementia – Panel and “Sue’s Story” (Nov 13, Los Altos)

Date
Posted by
Lewy Body Dementia – Panel and “Sue’s Story” (Nov 13, Los Altos)

There’s a local program in Los Altos, CA next Wednesday, November 13th, at 6:30pm, that some of our Lewy body dementia (LBD) group members may be interested in attending.

Sue Berghoff has Lewy Body Dementia.  Her husband Chuck Berghoff is a member of the Brain Support Network LBD caregiver support group.  They are visiting libraries and senior centers in Santa Clara County to increase awareness and discuss caregiving issues. 

As part of the event, “Sue’s Story,” a documentary about Sue’s journey, will be shown.  (For more info on the documentary, see thesuesstoryproject.com.)  Following the movie, there will be a panel discussion with a Kaiser neurologist and Brain Support Network volunteer Denise Dagan.  Denise cared for her father with LBD.  She also cared for her mother with Alzheimer’s.

The program will be held at the Los Altos Library, 13 South San Antonio Rd., Los Altos, CA 94022.

“Cognition in PD and LBD” – Presentation by Stanford MD (Nov 2018)

Date
Posted by
“Cognition in PD and LBD” – Presentation by Stanford MD (Nov 2018)

I stumbled across the slides from a presentation given by Kathleen Poston, MD, movement disorder specialist, Stanford, at a conference for laypeople in November 2018. The presentation is about “cognition in Parkinson’s Disease and Lewy body dementia.”

The slides I like the most are the two slides on the lefthand side of page three of the PDF. I’m constantly talking to caregivers about the balancing act between motor symptoms, cognitive symptoms, Parkinson’s medications, dementia medications, and psychosis medications.

http://med.stanford.edu/content/dam/sm/adrc/documents/2018-ADRC-kathleen-poston.pdf

Robin