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FEATURE: Primary Progressive Aphasia  - A New Normal

 

Editor's Note:   This article is written by Justine Leshikar, an SLP and daughter of  a man diagnosed with Primary Progressive Aphasia. Questions or comments can be emailed to Justine.  


 


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This is Justine, with her father Jim, on her wedding day..


 

When I was a junior in high school, I decided that I wanted to quit the cheerleading squad in the middle of football season. I informed my father of this decision and he said, "Absolutely NOT. Tripp's (our last name) are NOT quitters." That statement was informally coined as our family motto and has remained our theme over the last 5 years as we have navigated through my father's speech and language difficulties and what we now know is a diagnosis of Primary Progressive Aphasia- Logopenic subtype.

 

My father is a retired Commander in the United States Navy Reserve, a retired healthcare consultant who specialized in strategic planning, a devout Catholic, faithful husband of 39 years to my mother, proud father of 2 sons and 2 daughters and a REALLY proud Papa of 8 grandchildren. He was always a very well spoken man and was the guy you wanted proofreading your resume or essay. 


FEATURE:  We have an exciting announcement! 


 

Have you been just diagnosed with Primary Progressive Aphasia, a form of dementia?

- Were you told that there's nothing that can be done to help you?

Announcing the PPAToolbox program. A lifestyle, comprehensive program for PPA available nowhere else. 
 
  
Our research  covering FTD  and PPA centers show that many of these  centers have  programs and clinicians that will help you receive a diagnosis; and often medical advice and counseling but, they usually don't  offer much else.  So - you have a diagnosis; what do you do next

 

To fill the void concerning the lack of suitable rehabilitation and on-going options for people with Primary Progressive Aphasia, AphasiaToolbox has created an evidence-based program  called:

The PPAToolbox Lifestyle Program

Our program is  an intensive program, designed to not only help you work toward maintaining and using your speech, word retrieval, reading  & writing, and cognition but to provide on-going support and socialization.

Our program is a group program that is coach led and SLP supported:

Following an initial intake and consultation, you will have a 2-week trial of the program.

Each week, you will be able to select up to 3 coach-led sessions covering writing, word retrieval/semantics, cognitive exercises, and up to 2 conversational cafes.

Once a month, you will have a private session with a Speech-Language Pathologist to determine formative assessment

You will have the options of additional private coaching, consultation and/or speech therapy.

Our program is accessible:

By using secure video conferencing technology, you may participate easily from the comfort of your home in this innovative program.

> Our program is simple:

The program is a one-price program with two goals:

We help you maintain your language skills.

We help you to use new tools and other ways to communicate. 

> The cost of the program  includes a professional evaluation and up to 5 sessions weekly. 

For information on the PPA Toolbox Lifestyle Program, please contact Bill Connors at 724-494-2534 or information@aphasiatoolbox.com.

Sources on PPA Treatment:


3. A Thief That Robs the Brain of Language, The New York Times, By JANE E. BRODY, Published: May 2, 2011
 
Mesulam Awarded Potamkin Prize

 

 


 

Doctor and Researcher M. Marsel Mesulam, MD, director of the Northwestern University Feinberg School of Medicine at The Cognitive Neurology and Alzheimer's Disease Center and the first to identify primary progressive aphasia as an independent form of dementia,  has been awarded the 2014 Potamkin Prize.  

 

The award is given annually to a scientist for their work in helping advance the understanding of neurological disorders including Pick's disease and Alzheimer's. The $100,000 prize has become an internationally recognized tribute for advancing dementia research.   
Telecare in Dementia Care

 

Editor's Note:

As we were were getting ready to complete this editon of the newsletter, we found an interesting article discussing the use of smart technology specifically relating to care of Alzheimer's Disease and related forms of dementia.  This was of special interest to aphasiatoolbox as we have been providing rehabilitation and support to people in their homes with aphasia and dementia using technology since 2005.  


The article discusses telecare and how people with Dementia can still live independently using smart technology.  

 

The name 'telecare' refers to sensor-based monitoring; these are devices that continuously, automatically and remotely monitor real-time emergencies and lifestyle changes over time to manage the risks associated with living alone. Sensors around the home can be linked via a telephone line. The system monitors a person's activities and, if a problem occurs, triggers an alarm to a relative, a designated caregiver or call center. 


Some of the companies mentioned in this article include: 

 

SmartThings

Lively

BeClose 

GrandCare Systems

 

Sources:   

 1.  

Telecare helping Alzheimer's patients live in the 'connected home', Telehealth and Telecare Aware,  August  28, 2014


2.   Rajiv Siotia and Chris Simpson,

Applying telecare in dementia: what psychiatrists need to know

, Advances in Psychiatric Treatment (2008),  14: 382-388


 

 3.  Chou HK1, Yan SH, Lin IC, Tsai MT, Chen CC, Woung LC.   A pilot study of the telecare medical support system as an intervention in dementia care: the views and experiences  of primary caregivers.,  J Nurs Res. 2012 Sep;20(3):169-80

 

4.  SmartThings sensors let Alzheimer's patients stay at home, safely, Aug 26, 2014

 

FEATURE:   Diagnostic Criteria for PPA


Diagnostic Criteria for Primary Progressive Aphasia  (Mesulam, 2003, NEJM)

 

1.  There is an insidious onset and gradual progressive impairment of word finding, object naming, grammar, or word comprehension manifested during conversation or assessed with the use of standard neuropsychological tests of language.  Isolated motor speech disorders (such as dysarthria, apraxia of speech, aphemia) are not by themselves sufficient for making the diagnosis.  


 

2.  All major limitations in activities of daily living can be attributed to the language impairment for at least two years after onset.  

 

3. Premorbid language function (except for developmental dyslexia) is known to be intact.  


 

4. Prominent apathy, disinhibition, loss of memory of recent events, visuospatial impairment, visual-recognition deficits, are absent during the initial phase of illness, as indicated by the history, evaluation of activities of daily living or neuropsychological testing. 


 

5. Acalculia (inability to perform simple mathematical calculations) and ideomotor apraxia (inability to pantomime movement as instructed by an examiner) can be present even in the first two years of illness.  


 

6. Other cognitive functions may be affected after the first two years of illness, but language remains the most impaired function throughout the course of the illness and deteriorated faster than other affected functions. 


 

7.  Specific causes of aphasia, such as stroke or tumor, as ascertained by neuroimaging, are absent.

 

Source: 

 http://ppaconnection.org/index/diagnosticcriteria


 

FTD and PPA  - For more  information


 

> National Institute on Aging,  

Frontotemporal Disorders: A Resource List

This site  offers a complete bibliography covering FTD  and PPA.


>Assn for Frontotemporal Degeneration

This is the homepage for the Association for Frontotemporal Degeneration, which includes information on PPA.


 > National Aphasia Association

This is the homepage for the National Aphasia Association which offers good resources on PPA.

 

IMPPACT

The International PPA Connection site offers  resources and support for patients and family members.  This site is also a central site for support and dissemination of international collaborative research on PPA.  

Quote of the Month 


 

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Diagnosed with PPA?  Not ready to quit? Contact aphasiatoolbox  for information on our  
PPAToolbox Program.  
  

August 31, 2014
SPECIAL EDITION - Primary Progressive Aphasia
 
 
This is Sharon Rennhack, the  chief  editor for the aphasiatoolbox newsletter.  
 
This month we are offering a special focus on one form of dementia  - specifically,  Primary Progressive Aphasia. 

>  See two personal accounts :   one is an article from Justine Leshikar discussing her father's PPA, and  the second is a video interview with  actress Kimberly William-Paisley discussing her mother's  PPA.   They both discuss  a new "normal" way of living.  
 
>  I  discuss the basics of Primary Progressive Aphasia.

>  In his video, Master Clinician Bill Connors discusses what clients who have a diagnosis  of PPA can do.

>   We present another first for aphasiatoolbox.com.   The PPAToolbox program is the first, online comprehensive support and practice program for people with PPA and their caregivers.  
 
We believe that people with PPA can and should work on their language skills as Justine mentions in her article. For that reason,  we are creating a new treatment group called the PPA Lifestyle Treatment group.

Please contact us if you want information on this group,  and please share this edition with a person with Primary Progressive Aphasia.

 

Sharon Rennhack

Chief Editor 
Aphasiatoolbox 
VIDEO:  Actress Kimberly  Williams-Paisley discusses  

her mother's  PPA


 


 



 

In this February 2014 video  interview and in  the March 2014 issue of Redbook Magazine,  actress Kimberly Wiliams-Paisley talks  about her mother's diagnosis of  dementia, specifically Primary Progressive Aphasia.  Kimberly is the husband of singer Brad Paisley.
 

See the video.

 

VIDEO:  Master Clinician Bill Connors discusses Primary Progressive Aphasia

 

"This is the finest, most useful collection of up-to-date information and resources on PPA that I have seen."   

Bill Connors

 

 

Bill Connors discusses Primary Progressive Aphasia
Bill Connors discusses Primary Progressive Aphasia

 

If you have questions about this video , please contact us or call  us at 724-494-2534.  


FEATURE:  
What is Primary Progressive Aphasia? 


 

Primary progressive aphasia, or PPA, is a type  of Frontotemporal Dementia that affects speech and language. Unlike Alzheimer's  Disease, cognitive functioning 
remains intact in early PPA. [1]

PPA is a progressive impairment of language function that involves changes in the ability to speak, read, write and understand what others are saying. It is associated with a disease process that causes atrophy in the frontal and temporal areas of the brain, and is distinct from aphasia resulting from a stroke. 




> The frontal lobe, located at the front of the brain, is associated with reasoning, motor skills, higher level cognition, and expressive language.  At the back of the frontal lobe, near the central sulcus, lies the motor cortex. This area of the brain receives information from various lobes of the brain and utilizes this information to carry out body movements.  [2]

> The temporal lobe, located on the bottom section of the brain, is also the location of the  primary auditory cortex, which is important for interpreting sounds and the language we hear. The hippocampus is also located in the temporal lobe, which is why this portion of the brain is also heavily associated with the formation of memories. Damage to the temporal lobe can lead to problems with memory, speech perception, and language skills. [2]

Progressive language difficulties in word-finding, word usage, word order, word comprehension or word spelling lead to a diagnosis of PPA. Each individual with PPA has a different pattern of impairment, leading to the classification of PPA into subtypes or variants. 

What are some initial features of PPA? 

The initial features can vary from one person to another. [3]

In many instances, the patient may be the first to note that something is wrong and the complaints may initially be attributed to stress or anxiety. One or more of the following can emerge as the initial symptom:

* Slowed or halting speech
* Word-finding hesitations
* Sentences with abnormal word-order in speech or e-mails
* Substitution of words (e.g., "table" instead of "chair)
* Using words that are mispronounced or incomprehensible
*Difficulty understanding conversation despite normal hearing
* Sudden lapse in understanding simple words
* Forgetting the names of familiar objects
* Inability to think of names of people, even though the person is recognized
* New impairments in spelling

What are the subtypes or variants of PPA?

In 2011, criteria were adopted for the classification of PPA into three clinical subtypes   [4]:

 

PPA-G (Agrammatic/Nonfluent Subtype): This subtype has a problem with word-order and word-production.

Speech is effortful and reduced in quantity. Sentences become gradually shorter and word-finding hesitations become more frequent, occasionally giving the impression of stammering or stuttering. Pronouns, conjunctions and articles are lost first. 

PPA-S (Semantic Subtype): This subtype features a problem with word-understanding.

The principal feature is a loss of word meaning, even of common words. Speech has less nouns and is therefore somewhat empty of meaning. This is the one subtype where changes of personality and behavior are frequent. There may be agitation, display of excessive friendliness to strings, change of dietary habits, etc.  

PPA-L (Logopenic Subtype): This subtype features a problem with word-finding. 

In contrast to PPA-G, speech is fluent during causal small talk but breaks into mispronunciations and word-finding pauses when a more difficult or precise word needs to be used.  
Spelling errors are common. The naming of objects becomes impaired.  While this is a description of most common features, remember that each person is different which may result in some differences in how the symptoms present and unfold.

Sources:




4. Classification of primary progressive aphasia and its variants, Gorno-Tempini, M.L., Hillis, A.E., Weintraub, S, et.al. Neurology; March, 2011


FEATURE:     

Pharmacological Interventions  - Medicines in Use with PPA     

 

Editor's Notes:

 

The ice bucket challenge has resulted in a real surge of interest in the progressive disease Amyotropic Lateral Sclerosis (ALS) including famous celebrities celebrity videos .   ALS has really no effective medicine or pill despite years of research and development.   The same may be said for PPA.

 

Northwestern University tells  us -  "there are no pills yet for PPA."  [ 1 ]  And,  in their presentation at the FTD/PPA Family Caregiver and Professional Education and Support Conference in 2012, Michelle Sanfilippo and Tory Eitz [2] also state that there are currently no FDA-approved medications to treat FTD or PPA.  

Most pharmalogical interventions for PPA have been borrowed from among those currently available for Alzheimer's Disease or Parkinson's Disease, including Cholinesterase inhibitors, NMDA receptor antagonists and Dopamine Receptor Agonists. 

AphasiaToolbox has reviewed the research concerning potential medications to treat primary progressive aphasia. There is limited evidence of the efficacy of pharmacological treatments in FTD/PPA.

Despite the dearth of evidence,  we  are presenting our research concerning medication and PPA, as we believe that caregivers and clients will want to know about all possible treatments relating to PPA.   Leave no stone unturned is our policy!  

> CHOLINERTERASE INHIBITORS 

Cholinesterase inhibitors are a class of medications that work to increase the amount of acetyl choline in the brain, where they increase memory performance and mental function.

These drugs bind to cholinesterase resulting in increased acetylcholine in the synapses, causing increased parasympathetic activity i.e. vasodilatation, constriction of pupils in the eyes, increased secretion of sweat, saliva and tears, slow heart rate, mucus secretion in the respiratory tract and constriction of bronchioles etc.

NMDA RECEPTOR ANTAGONISTS 

Although it has been used in Europe for many years, NMDA receptor antagonists such as Namenda (Trade Name: Memantine) have only been made available more recently in Canada and the United States for Alzheimer's Disease.  This drug blocks the transmission of a brain chemical called glutamate, a brain neurotransmitter. Too much glutamate is felt to cause calcium overload in brain cells. 

Namenda (Trade Name: Memantine) is prescribed to improve memory, attention, reason, language and the ability to perform simple tasks. It can be used alone or with other Alzheimer's Disease treatments. There is some evidence that individuals with moderate to severe Alzheimer's who are taking a cholinesterase inhibitor might benefit by also taking memantine. Donepezil (Aricept) is the only cholinesterase inhibitor approved to treat all stages of Alzheimer's Disease, including moderate to severe.

Reportedly, Namenda helps by delaying the worsening of symptoms for some people temporarily. Many experts consider its benefits similar to those of cholinesterase inhibitors. It can cause side effects, including headache, constipation, confusion and dizziness.

> DOPAMINE RECEPTOR AGONISTS

These medicines constitute a class of drugs used to treat Parkinson's disease symptoms that mimic the action of naturally occurring dopamine. They stimulate dopamine receptors directly without being metabolized to another compound as is the case with levodopa. Although this class of medication is less potent than levodopa, they can be very beneficial in treating symptoms for long periods of time.

Bromocriptine (Trade names: Parlodel, Cycloset, Brotin) is a dopamine Receptor antagonist. 

Sources:   



Clinical Trials covering FTD/PPA

1. 
Witt K1, Deuschl G, Bartsch T., Frontotemporal dementias, Nervenarzt. 2013 Jan;84(1):20-32. In German

2. 
Freedman M., Frontotemporal dementia: recommendations for therapeutic studies, designs, and approaches. Can J Neurol Sci. 2007 Mar;34 Suppl 1:S118-24.

3. 
Johnson NA, et al, Pilot Trial of Memantine in Primary Progressive Aphasia, Alzheimer Dis Assoc Disord. 2010 Jul-Sep; 24(3): 308.
4. 
Reed DA, et a,  A clinical trial of bromocriptine for treatment of primary progressive aphasia, Ann Neurol 2004; 56:750.

5. 
Kertesz A et al,  Galantamine in frontotemporal dementia and primary progressive aphasia,   Dement Geriatr Cogn Disord. 2008;25(2):178-85. 

In addition to this report on "Pharmacology for Primary Progressive Aphasia",  see also our recently-published report  - "Pharmacology for Aphasia Treatment and Recovery".
The Aphasia Center of Innovative Treatment, Inc | | bill@aphasiatoolbox.com | http://www.aphasiatoolbox.com
800 Vinial Street, B408
Pittsburgh, PA 15212

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