PSP and CBS excerpts from curriculum on dementia for healthcare professionals

Someone in our local support group recently sent me this link to US Dept. of Health and Human Services’s curriculum for physicians (especially primary care physicians) and healthcare professionals (social workers, psychologists, pharmacists, emergency department staffs, dentists, etc.) on dementia. Though the web address includes the term “Alzheimer’s,” frontotemporal dementia is also mentioned in this curriculum:

Training Curriculum: Alzheimer’s Disease and Related Dementias
Health Resources and Services Administration (part of Dept of HHS)
bhw.hrsa.gov/grants/geriatrics/alzheimers-curriculum

One of the types of frontotemporal dementia is the “motor type,” which include corticobasal syndrome and progressive supranuclear palsy.

Here are some excerpts on frontotemporal dementia.

Robin

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Overview of Mild Cognitive Impairment and Dementia for an Interprofessional Team (Module 1)

Frontotemporal Dementia Types
* There are at least 3 distinctive clinical syndromes, each with heterogeneous neuropathology.
– Progressive behavior/personality decline: behavioral variant FTD (bvFTD)
– Progressive language decline: Primary progressive aphasia (PPA)
– Progressive motor decline: corticobasal syndrome, amyotrophic lateral sclerosis, or [progressive] supranuclear palsy. FTD with progressive motor decline is rare. FTD with progressive motor decline can involve movement problems/slowed movement, muscle rigidity (Parkinsonian symptoms), body stiffness, and changes in behavior or language.
* Behavioral variant FTD (bvFTD) is the most common variant. It is characterized by marked personality changes and changes in social conduct.


Understanding Early-Stage Dementia for an Interprofessional Team (Module 5)

Early-Stage Frontotemporal Degeneration (FTD): Overview
* FTD is a heterogeneous group of diseases with overlapping clinical symptoms but different causative genes and differing underlying pathologies.
* FTD is caused by damage to frontal and/or temporal lobes. Impairments generally progress quickly but memory often remains intact.
* Persons with FTD demonstrate changes in behavior and personality, language problems, and motor problems.
( Memory impairment is minimal in early stages.


Palliative and End-of-Life Care for Persons Living with Dementia (Module 12)

When to Consider Hospice Care in Persons with End-Stage FTD
* Persons with end-stage FTD are generally younger and healthier than persons with other types of end-stage dementia.
* As with other dementias, FTD is often not recognized as a terminal diagnosis.
* End-stage FTD may “look different” than other advanced dementias.

 

Apathy – description and treatment

Brain Support Network volunteer Denise Dagan came across this article in a recent Parkinson’s Disease (PD) organization’s newsletter about apathy in PD.  Certainly apathy occurs in many of the disorders in the Brain Support Network community as well — especially progressive supranuclear palsy (PSP).  That’s why I’m sharing the article within our network.

These statements in the article caught Denise’s eye:

“Persons with apathy generally do not recognize the symptoms, so caregivers will need to bring it to medical attention. … It is important to assess for apathy because those with apathy are 2.5 times more likely to report poor quality of life in comparison to those without apathy. Apathy is also associated with more severe motor impairment. PD patients with apathy are less physically active and may not adhere to medical recommendations. Relationships may suffer as well since caregivers often experience more frustration and stress.”

The author of the article is Rosa Chuang, MD.  She may be familiar to some in our multiple system atrophy (MSA) group.  She used to practice at Stanford but is now in Seattle.

The article is copied below.

Robin

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www.apdaparkinson.org/community/northwest/about/newsletters/

Apathy in Parkinson’s Disease
Parkinson’s Pathfinder (Newsletter by APDA Northwest)
Summer 2017
By Dr. Rosalind Chuang

Apathy is a common non-motor symptom of Parkinson’s disease but often times not recognized or commonly mistaken for depression. Some studies show that 30-40% of PD patients have apathy, but the frequency can range from 20-70%, depending on how patients are asked. It can occur at any stage of PD and can even occur before motor symptoms develop. It is important to assess for apathy because those with apathy are 2.5 times more likely to report poor quality of life in comparison to those without apathy. Apathy is also associated with more severe motor impairment. PD patients with apathy are less physically active and may not adhere to medical recommendations. Relationships may suffer as well since caregivers often experience more frustration and stress.

WHAT IS APATHY?

Apathy is defined as:
• Loss of motivation or lack of initiative
• Loss of pleasure
• Decreased goal directed behaviors
• Decreased goal directed cognitive activity
• Decreased interests and emotions (reduced display of emotions)

WHAT TO LOOK FOR IF YOU ARE CONCERNED ABOUT APATHY

A common complaint from family and friends is that the PD patient just “sits around” or “doesn’t seem to care about anything.” Nothing gets done and a person often declines social activities if given a choice. This can be misinterpreted as fatigue, laziness, or lack of empathy/ uncaring.

Persons with apathy generally do not recognize the symptoms, so caregivers will need to bring it to medical attention. Medical providers may ask specific questions from the Starkstein apathy scale to determine apathy. Some questions on the scale include:

• Any interest in learning new things?
• Does anything interest you?
• Do you look for things to do?
• Are you concerned about your condition? Or unconcerned about many things?
• Does someone have to tell you what to do each day? Do you need a push to get started on things?
• Are you neither happy nor sad, just in between?

As you can see, these questions are similar to those to assess for depression, so sometimes it can be difficult to separate apathy from depression. Often times, patients can have both depression and apathy, but in ~10- 28% of time, patients can have apathy alone.

WHY IS IT NOT DEPRESSION?

In both depression and apathy, a person may no longer enjoy things. However, someone with depression may endorse feeling “blue” or sad. Other “negative” symptoms of depression include inappropriate guilt, loss of appetite, loss of sleep, or thoughts of death. An apathetic person does not cry frequently or have suicidal thoughts.

TREATMENT

It is important to evaluate if the symptoms are from apathy alone because it can affect treatment. If apathy is associated with depression or anxiety, treatment of co-morbid conditions can help reduce apathy. Sometimes isolated apathy can also respond to the SSRIs used to treat depression, but generally studies don’t show good response. Dopamine medications (levodopa or dopamine agonists) may also improve apathy. (In some patient who have undergone deep brain stimulation for PD, rapid withdrawal of their PD medications resulted in apathy.) In one trial, PD apathy responded to rivastigmine, a medication used for dementia, even though the patients did not actually have dementia.

For isolated apathy, I generally recommend non-pharmacologic treatment. These include:

• Write down at least 3 daily goals and 3 weekly goals. These goals can be physical, social, or thinking activities.
• Daily goals should be specific and can be reasonably achieved.
• Create a schedule: be specific when each task will should be accomplished.
• Review the written list at breakfast, lunch and dinner to remind yourself of the next goal.
• Cross off each task as you complete them.
• Say “yes” to at least one thing every day even if you don’t feel like it.
• Maintain routine: continue to do things you used to do, even if you don’t feel like it.
• Recall an activity that you used to enjoy and try to restart that activity.
• Exercise even if you don’t feel like it.
• Must leave the house at least once a day

Even though apathy is not as easily treated as the motor symptoms of PD or other non-motor symptoms such as depression, simply recognizing and understanding apathy is an important part of overall management of Parkinson’s disease.

Save the Date! Saturday, October 28, PSP/CBD Research Update and Family Conference

Save the date!

Brain Support Network will host the:

PSP/CBD Research Update and Family Conference
Saturday, October 28, 2017
Crowne Plaza Foster City (San Francisco Bay Area)

This conference is for families coping with progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD).   Professionals and anyone in the community are also welcome to attend.

BSN’s planning partner is Dr. Adam Boxer and the team at the UCSF Memory & Aging Center. UCSF is the lead institution for PSP and CBD clinical trials. We are lucky to have them in our backyard!

The conference will be run from 9am to 5pm. The morning will feature international researchers in town for a major conference on PSP, CBD, and tau. The afternoon will feature Bay Area clinicians (from UCSF and Stanford), healthcare professionals, and families.

We anticipate registration will open in early September. Join our PSP or CBD email lists and we’ll send you an update when registration opens. Alternatively, check back at our website in September to register. Our meeting facilities are planned to accommodate 150 participants.

Stay tuned for more details!

Robin

 

Inheritability of PSP

This is an interesting, short question-and-answer on the Genetic and Rare Diseases Information Center (part of NIH) about the inheritability of progressive supranuclear palsy (PSP), from September 2012.

Robin

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rarediseases.info.nih.gov/diseases/7471/progressive-supranuclear-palsy/cases/36421

Progressive supranuclear palsy
Genetics and Rare Diseases Information Center (part of NIH)
Last updated 9/12/2012

Question: My identical twin sister has been diagnosed with progressive supranuclear palsy (PSP). As it seems this disease may have a genetic cause, what is the chance that I would also develop PSP? And within what timeframe?

 

Answer: The following information may help to address your question:

What is the chance of developing progressive supranuclear palsy if a relative is affected?

Researchers do not yet completely understand the causes of progressive supranuclear palsy (PSP), so it is not possible to predict the chance of a relative developing the condition if one family member has been diagnosed. Most of the time, only one person in a family develops PSP. It is not very common to have multiple members of a family develop PSP. One study found that 7% of individuals with PSP had a family history of potentially related conditions (including dementia or parkinsonism), which suggests there may be a shared genetic risk factor.[1][2]

A few genetic risk factors (particularly a gene called MAPT) are known to play a role in the development of PSP.[3][4] However, these genetic risk factors have only been found in a few families. Researchers suspect that there are other genetic factors that haven’t been found yet that also contribute to progressive supranuclear palsy.

 

If a family member has a known genetic risk factor for progressive supranuclear palsy, what is the chance a relative has inherited it?

Close relatives such as parents, siblings, and children of an affected individual have a greater chance of sharing a genetic predisposition than do aunts, uncles, grandparents or cousins. Identical twins share their entire DNA, so they should have the same genetic risk factors.

 

If an individual inherited a known risk factor, what is the chance of developing progressive supranuclear palsy?

Having a genetic risk factor increases the chance of developing progressive supranuclear palsy (PSP); it does not guarantee that an individual will develop this disease at some point in their lifetime. The exact risk of developing PSP in an individual with a genetic predisposition is currently unknown.[5]

 

What is the average age at diagnosis for progressive supranuclear palsy?

Individuals with progressive supranuclear palsy usually experience the first symptoms of this disease while in their 60s.[1]

 

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

 

References
1. Progressive supranuclear palsy. Genetics Home Reference. March 2011; http://ghr.nlm.nih.gov/condition/progressive-supranuclear-palsy. Accessed 9/7/2012.

2. Donker Kaat L, Boon AJ, Azmani A, Kamphorst W, Breteler MM, Anar B, Heutink P, van Swieten JC. Familial aggregation of parkinsonism in progressive supranuclear palsy. Neurology. 2009; 73:98-105. http://www.ncbi.nlm.nih.gov/pubmed/19458322. Accessed 9/12/2012.

3. Rojo A, Pernaute RS, Fontán A, Ruíz PG, Honnorat J, Lynch T, Chin S, Gonzalo I, Rábano A, Martínez A, Daniel S, Pramstaller P, Morris H, Wood N, Lees A, Tabernero C, Nyggard T, Jackson AC, Hanson A, de Yébenes JG. Clinical genetics of familial progressive supranuclear palsy. Brain. 1999; 122:1233-1245. http://www.ncbi.nlm.nih.gov/pubmed/10388790. Accessed 9/6/2012.

4. Dickson DW, Rademakers R, Hutton ML. Progressive supranuclear palsy: pathology and genetics. Brain Pathology. 2007; 17:74-82. http://www.ncbi.nlm.nih.gov/pubmed/17493041. Accessed 9/6/2012.

5. Litvan I. Update on epidemiological aspects of progressive supranuclear palsy. Movement Disorders. 2003; 18:S43-S50. http://www.ncbi.nlm.nih.gov/pubmed/14502655. Accessed 9/6/2012.

 

“Square patches of light remember you” – reflections on grandmother with PSP

This beautiful remembrance, “Square patches of light remember you,” was published in the June 13, 2017 issue of Neurology journal.  These reflections are by Bronte Nicole Ficek, whose grandmother Lola had progressive supranuclear palsy (PSP).  See:

www.neurology.org/content/88/24/2333.full