Category Archives: FTD

Problems families dealing with dementia may face

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I recently stumbled across the website, patient.info, which is a resource for UK physicians about various disorders.  The website contains a helpful page about caregiving for someone with dementia.  Though it is written with physicians in mind (ie, how physicians can be supportive of families), I think the page provides a good summary for laypeople as well.

In addition, I think much of this article applies to caregivers who are NOT dealing with dementia.  Much of the information and advice is generic.

Here’s a link to the full article:

patient.info/doctor/supporting-the-family-of-people-with-dementia

Supporting the Family of People with Dementia
Patient, a UK organization
Last reviewed May 2014

Note that in the UK “caregivers” are referred to as “carers.”  (It’s a superior term in my mind.)

Robin

“Shining A Light”: Actress Kimberly Williams-Paisley on Caregiving

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“Shining A Light”: Actress Kimberly Williams-Paisley on Caregiving

In the October/November 2016 issue of Neurology Now magazine, actress Kimberly Williams-Paisley shares her experience about caring for her mother with primary progressive aphasia, a type of frontotemporal degeneration.  (PSP and CBD are also types of frontotemporal degeneration.)  The actress wants other families to avoid the mistakes she made.  Though Brain Support Network does not include PPA among our four disorders, the article has some good advice for our group members as well.

Ms. Williams-Paisley has written a book titled  Where the Light Gets In, described as “unflinching” by the Neurology Now article.  If someone reads it, let me know if you’d recommend it and if it’s relevant to one of the disorders in our group.

Here are a few caregiver-related excerpts from the magazine article:

* The “family shouldered the burden for far too long.”

* The family allowed the mother to drive for far too long, even after several accidents.

* “[Driven by guilt, indecision, and uncertainty, the family…stumbled on. It was a situation Kim thought could have been avoided if the family had had a conversation with Linda before the disease robbed her of insight and judgment.”

* Family members should “not try to do all the caregiving on their own. It’s important for caregivers to reach out and ask for help,” Kim says.

* “One of the most profound lessons…learned…was this: Put your wishes in writing before you get sick.  The fact that her mother, Linda, did not put her wishes for long-term care in writing caused Kim and her family a great deal of guilt and uncertainty.”

Here are some relevant suggestions from the physician quoted in the article:

* Become educated about the disease.  Find resources.

* Teach patients how to communicate anger.

* Help patients cope with aphasia through music therapy or other expressive arts.

* Help patients focus on abilities the patient still has to make him or her feel better.

* Encourage patients and their families to participate in clinical trials.

* Speak out about the disease.

* Know that we are all imperfect.

Here’s a link to the full article:

journals.lww.com/neurologynow/Fulltext/2016/12050/Shining_A_Light__Actress_Kimberly_Williams_Paisley.18.aspx

Neurology Now
October/November 2016
Volume 12 – Issue 5, p 24–27
 
Shining A Light
Actress Kimberly Williams-Paisley wants to help other families dealing with primary progressive aphasia avoid the mistakes she and her family made in caring for her mother.
by Mary Bolster and Gina Roberts-Grey

Be sure not to miss the “web extra”!

Robin

“Fighting Loneliness When Dementia Steals a Spouse”

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This is a very personal essay from November 2016 by children’s book author and illustrator Nancy Carlson. She describes the loneliness she feels when dementia has “stolen” her spouse. At this time, her husband Barry, with a frontotemporal dementia (FTD) diagnosis, was living in a care home.

Many spouses in our local support group have expressed similar feelings of loneliness at our support group meetings. One describes it as being widowed yet her husband is still alive.

I’m not sure that dementia is a requirement for the caregiver to feel this kind of loneliness. Having your spouse in a care facility or having a spouse unable to communicate in any way are certainly lonely circumstances.

Here’s a link to the full essay:

womensvoicesforchange.org/my-journey.htm

Emotional Health
Fighting Loneliness When Dementia Steals a Spouse
By Nancy Carlson
Women’s Voices for Change
November 14, 2016

Robin

 

“Atypical multiple system atrophy is a new subtype of frontotemporal lobar degeneration”

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This is an interesting paper out of the Mayo Clinic Jacksonville about “four patients…with clinical features consistent with frontotemporal dementia (FTD), including two with corticobasal syndrome, one with progressive non-fluent aphasia, and one with behavioral variant FTD.  None had autonomic dysfunction.”

Curiously, these four patients met the pathological criteria for multiple system atrophy (MSA).  These were all atypical MSA cases because “[all] had frontotemporal atrophy and severe limbic α-synuclein neuronal pathology.”

The Mayo researchers note a previous case report of a woman with progressive dementia, psychosis, and muscular rigidity “with neuropathological features of both MSA and LBD” (Lewy body dementia).  This could be a fifth case of atypical MSA with features of FTD.

Researchers also note that six other atypical MSA cases have been reported around the world.

They conclude:

“Atypical MSA does not fit into any of the current categories [of FTLD – frontotemporal lobar degeneration]. We propose a new category of FTLD for atypical MSA—FTLD-synuclein. Alternatively, atypical MSA could be considered a subtype of MSA in addition to MSA-P and MSA-C, namely MSA-FTD. From a clinical perspective, this is a more challenging diagnosis, since none of our patients carried an antemortem diagnosis of MSA; rather, the clinical picture was that of FTD.”

The abstract is copied below.

The things you learn via brain donation!

Robin

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Acta Neuropathologica. 2015 Jul;130(1):93-105. Epub 2015 May 12.

Atypical multiple system atrophy is a new subtype of frontotemporal lobar degeneration: frontotemporal lobar degeneration associated with α-synuclein.

Aoki N, Boyer PJ, Lund C, Lin WL, Koga S, Ross OA, Weiner M, Lipton A, Powers JM, White CL 3rd, Dickson DW.

Abstract
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease clinically characterized by cerebellar signs, parkinsonism, and autonomic dysfunction. Pathologically, MSA is an α-synucleinopathy affecting striatonigral and olivopontocerebellar systems, while neocortical and limbic involvement is usually minimal. In this study, we describe four patients with atypical MSA with clinical features consistent with frontotemporal dementia (FTD), including two with corticobasal syndrome, one with progressive non-fluent aphasia, and one with behavioral variant FTD. None had autonomic dysfunction. All had frontotemporal atrophy and severe limbic α-synuclein neuronal pathology. The neuronal inclusions were heterogeneous, but included Pick body-like inclusions. The latter were strongly associated with neuronal loss in the hippocampus and amygdala. Unlike typical Pick bodies, the neuronal inclusions were positive on Gallyas silver stain and negative on tau immunohistochemistry. In comparison to 34 typical MSA cases, atypical MSA had significantly more neuronal inclusions in anteromedial temporal lobe and limbic structures. While uncommon, our findings suggest that MSA may present clinically and pathologically as a frontotemporal lobar degeneration (FTLD). We suggest that this may represent a novel subtype of FTLD associated with α-synuclein (FTLD-synuclein).

PMID: 25962793  (see pubmed.gov for the abstract only)

“Diagnosing and Treating Rapidly Progressive Dementias” (dementia within one year)

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Rapidly progressive dementias (RPDs) “include prion diseases such as CJD [Creutzfeldt-Jakob Disease], autoimmune dementias, paraneoplastic conditions, infections that can mimic prion diseases, and fungal infections as well as some viral and toxic metabolic conditions, such as Wernicke’s encephalopathy (a condition that stems from thiamine deficiency often due to malnutrition) and even conditions stemming from complications from an overdose of Pepto-Bismol.”

Here’s a short overview of RPDs. I don’t think it’s necessary to read the entire article, though it is short. For our purposes, here are the key excerpts:

* “Dementia may result from as many as 40 different diseases and conditions ranging from dietary deficiencies to inherited diseases, according to the Encyclopedia of Mental Disorders. With that, the definition of dementia has broadened over time from a focus on memory loss to a focus on impairment in one or more cognitive domains — particularly memory, language, frontal executive function, organizing, planning, and multitasking — that is severe enough to interfere with a person’s daily function. Typically, chronic degenerative dementias are characterized by damage or wasting away of brain tissue usually progressing over seven to 10 years. These include Alzheimer’s disease, frontal lobe dementia, and Pick’s disease. Dementias associated with progression of other diseases or conditions include Huntington’s disease, Lewy body dementia, and other parkinsonian dementias, such as corticobasal degeneration.”

* “Michael Geschwind, MD, PhD, associate professor of neurology at the Memory and Aging Center at the University of California, San Francisco said that although there is no defined time frame for rapidly progressing dementias (RPDs), he uses the term to describe patients that go from normal cognition to dementia within a year or less. However, he has seen some patients with Creutzfeldt-Jakob Disease (CJD) take as long as two years to develop dementia. Although there can be overlap with some typical degenerative dementias presenting in a rapid fashion, in general, patients who may have an RPD need clinicians to consider a different set of disorders, because these conditions can be both treatable and quickly fatal in some cases, Geschwind explained. … In a 2007 monograph on RPD published in Neurology Clinic, Geschwind’s team observed that 15% to 20% of the 825 patients referred to UCSF with rapidly progressing dementia presumed to be CJD turned out to have other non-prion conditions.”

* “Steven Vernino, MD, professor of neurology and neurotherapeutics at the University of Texas Southwestern Medical Center in Dallas, specializes in autoimmune encephalopathies. As a referral center, he said they see a fair number of treatable autoimmune RPDs each year. These include anti-N-methyl D-aspartate (anti-NMDA) receptor antibody encephalitis, paraneoplastic limbic encephalitis (PLE), and autoimmune limbic encephalitis associated with potassium channel-related antibodies, such as leucine-rich, glioma-inactivated 1 (LGI-1) antibody.”

Here’s a link to the full article for those who want to know more:

www.neurologyadvisor.com/neurodegenerative-diseases/diagnosing-and-treating-rapidly-progressive-dementias/article/382447/

Diagnosing and Treating Rapidly Progressive Dementias
Michael O’Leary
Neurology Advisor
November 10, 2014

Robin