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

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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)

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

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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.

“How to Avoid Becoming Isolated as a Caregiver”

This is a good article in today’s Next Avenue (nextavenue.org) about the risks of becoming isolated as a caregiver:

Three caregivers’ stories, and advice on possible solutions
By George Lorenzo
November 21, 2019
Next Avenue

www.nextavenue.org/caregiver-avoid-isolation/

Copied below is an excerpt from the section on avoiding self-inflicted isolation.

Robin

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Excerpt from:

Three caregivers’ stories, and advice on possible solutions
By George Lorenzo
November 21, 2019
Next Avenue

Avoiding Self-Inflicted Isolation

Yet, despite all the services and support groups available, it is not uncommon for caregivers to avoid them and self-isolate. “Some of it [isolation and loneliness] might be self-imposed, because you do not want to impose on other people. I feel a huge responsibility for my mom and I don’t always trust people to take over for me,” Bagley says.

“The isolation is not like a desert island, but you can isolate yourself,” Danielson explains. “I have talked to people when I go to the day care center. Some of them are really distraught. Some of them don’t know what to do. It’s a difficult life, but I don’t want anybody to feel sorry for me. In many ways, it is a privilege to be able to take care of someone you love.”

Enter Peter Rosenberger, the passionate, highly empathetic and straight-shooting radio show host of Hope for the Caregiver, broadcast twice weekly nationwide on American Family Radio and Family Talk on Sirius XM-131.

Caregivers can call in to talk about their experiences and get advice from Rosenberger, who has been the primary caregiver for his wife, Gracie, for more than 30 years. Rosenberger is also author of a book of the same name as his radio show, and, most recently, a second book titled 7 Caregiver Landmines and How You Can Avoid Them.

For landmine No. 2, solitude, he offers several steps caregivers can take immediately: “Serving as a caregiver is simply too difficult to do alone,” he explains.

In addition to getting counseling from a mental health expert and participating in a support group, Rosenberger suggests that caregivers call three people they can trust and talk with them without going into a deep conversation. “Just ‘sip’ the friendship slowly,” he says.

Young-onset dementia: key pointers to diagnostic accuracy

This interesting medical journal article from 2019 is about the best practices in the United Kingdom in diagnosing “young onset dementia” (YOD), which is dementia diagnosed under 65 years. The authors indicate that YOD is poorly recognized and often misdiagnosed as psychiatric disorders, including depression.

Excerpt: Recent studies “indicate that the average time to diagnosis was 4.4 years in younger people for all-cause dementia compared with 2.2 years for late-onset disease of comparable severity. Increased time to diagnosis for younger people is more likely when the younger person receives a diagnosis of FTD [frontotemporal dementia], rather than other dementia types. … Given the significance of changes in empathy and disinhibition often associated with FTD, delay in diagnosis can mean that close relationships break down prior to diagnosis or that people take considerable risks. … This complexity often results to delays in diagnosis and additional stress, frustration and burden for families.”

Moderate improvement in latency to a PSP diagnosis over 20 years (Golbe)

I ran into Larry Golbe, MD, a PSP expert at a conference last week. He pointed out this interesting interesting article from late 2018. It’s about the reduced time to diagnosis in the New Jersey area for those with progressive supranuclear palsy (PSP). In the 1990s, the time from onset to diagnosis was about 44 months (just under 4 years). In the 2010s, the time from onset to diagnosis was about 29 months (about 2.5 years).

“Diagnosis of PSP earlier in the course may reduce its psychological and financial burden, permit earlier access to neuroprotective interventions, and avoid unnecessary diagnostic and therapeutic measures.”

Senior author Larry Golbe, MD and others speculate why there’s been a moderate improvement in latency to a PSP diagnosis. One thought is that there’s improved awareness among clinicians and the public about PSP due to the efforts of CurePSP, the advent of the web in the 1990s, and the public announcement by celebrity Dudley Moore of his diagnosis in 1998. (Moore died in 2002.)

Another thought is that clinicians have gotten better at recognizing those with PSP who don’t exhibit the “classic form.”

Several other guesses are made but you’ll need to read the full article, which you can find here: (available at no charge)

Is the Latency from Progressive Supranuclear Palsy Onset to Diagnosis Improving?
by Mansoureh Mamarabadi MD, Hadie Razjouyan MD, MPH, and Lawrence I. Golbe MD
Movement Disorders Clinical Practice
First published: 02 September 2018

onlinelibrary.wiley.com/doi/full/10.1002/mdc3.12678

What do you think is the reason that the latency to receiving a diagnosis has improved?

Robin