There was a conference call today for support group leaders put on by the Association for Frontotemporal Dementias. (PSP and CBS/CBD fall into the “movement disorders” type of FTDs, which is how/why I’m invited to attend.) The topic of the call was Primary Progressive Aphasia, and the speaker was Sandy Weintraub, PhD, of Northwestern, one of the centers in the US studying PPA (and all FTDs).
My notes are below.
FYI – PPA has been addressed briefly in two webinars this year:
Boeve Webinar: Dr. Weintraub’s presentation is an expansion of the PPA topic within Dr. Brad Boeve’s recent webinar on cognitive and behavioral aspects in FTDs. Dr. Boeve said taht 60% of those with a clinical diagnosis of PPA end up being diagnosed with a tauopathy (such as PSP, CBD, or AD) upon brain autopsy.
I posted my notes on Dr. Boeve’s webinar back in April 2010:
Litvan Webinar: As we learned in Dr. Irene Litvan’s presentation, one of the three clinical presentations of CBS is PPA. In my notes on Dr. Litvan’s presentation, I had complained that she hadn’t covered the “progressive aphasia” presentation at all. Well, today’s conference call certainly supplied the missing pieces.
I posted my notes on Dr. Litvan’s webinar back in October 2010:
FTD Support Group Leaders’ Conference Call
Organizer: Association for Frontotemporal Dementias (ftd-picks.org)
Topic: Primary Progressive Aphasia: Understanding language presentations and approaches to care
Speaker: Sandra Weintraub, PhD
Northwestern University’s CNADC (Cognitive Neurology and Alzheimer’s Disease Center)
The FTD class of disorders is ever-widening.
Three main types of FTD disorders, depending on early symptoms:
* change in behavior, personality, and emotions: bvFTD
* decline in language (speaking, understanding, reading, writing): PPA
* change in motor function and movement: PSP, CBS, MND
“Early” = first 2 years. These years are critical for families. And critical for clinicians to understand the underlying pathology.
PSP, CBS, and MND often have cognitive symptoms associated with them as they progress.
All FTD disorders are progressive.
What is aphasia?
1. A disorder of language: inability to link words to thoughts for communication
2. Caused by brain damage: usually associated with a sudden loss of language function, caused by a stroke but in PPA is slowly progressive because the cause is neurodegenerative disease
3. Affects all aspects of language usage, not just speech output. A disorder of speech alone without language
impairment is “dysarthria” and it does not prevent the individual from communicating their thoughts since they can
still write normally. Asking the patient to write is one way to distinguish aphasia and dysarthria.
Early Symptoms of PPA (Primary Progressive Aphasia)
1. Gradual loss of language (aphasia): word-finding, speaking in full sentences, understanding conversation and/or written words, writing.
Subtypes include: Agrammatic, Logopenic, Semantic. Subtype depends on what the language disorder is.
Agrammatic: They say “water” rather than “I want water now.”
Logopenic: Slow output and groping for words.
Semantic: What is “salt”? Single-word comprehension deficit. (Called “Semantic Dementia”.)
2. Other cognitive functions are normal or relatively so. Hard to test someone’s memory if they can’t speak.
3. Daily living activities are affected mostly by aphasia in early stages. This is because short term memory and personality are intact.
4. Aware of symptoms and can become depressed due to this awareness.
5. Symptoms progress over time and other problems develop.
Boston Naming Test is used in diagnosis.
Example – Pictures of objects. The patient is asked to name the object.
Example – Test of single word comprehension – nouns and verbs. “Show me the picture where someone is laughing.” Someone with Semantic subtype cannot do.
Example – Color name comprehension. “Show me the color blue.”
Example – Body part name comprehension. “Show me the nose.”
“My sister like to have wheat bread. She will puts the mayo, cheese, turkey and lettuce. She will take a knife to cut the sandwich. The sandwich will put on the plate, My sister will have milk in a glass.”
–> This is agrammatic
“Get whatever type of bread you would like. Then add cheese, with different types. Then add liquid on the bread. Then leados, vegitables, chicken or turkey or other different ones. Then you have a good one!”
–> Fewer nouns here. The patient is having difficulty coming up with the right words. Could be agrammatic, logopenic or semantic! Lots of overlap! She thinks it is a logopenic patient. (I think “leados” is “lettuce.”)
MRI scan on the left. (Taken as if you were standing above the patient’s head.) PET scan on the right. The left side of the images are the right side of the brain.
1. Mutism. Usually this is a late symptom. In rare cases, someone can be mute for 3-4 years.
2. Severe difficulty understanding what others are saying even though hearing is normal
3. Personality changes
4. Memory loss
5. Daily living activities severely limited
Early PPA vs. bvFTD:
PPA patients have aphasia but no other symptoms.
bvFTD patients have personality change, concentration problem, and social-interpersonal problems.
Motor symptoms may be present in both.
Late PPA vs. bvFTD:
PPA patients have aphasia and concentration problem. They may or may not have memory loss, visual disorder, personality change, and social-interpersonal.
bvFTD patients have personality change, concentration problem, and social-interpersonal problems. They may or may not have aphasia, memory loss, and visual disorder.
Motor symptoms may be present in both.
Visual disorder = your brain can’t see.
bvFTD and PPA
* Psychosocial and treatment strategies differ vastly from memory loss dementia.
* Need specialized education, support services, community resources.
Northwestern’s PPA/FTD Program
* website: brain.northwestern.edu/ppa/
* semi-annual newsletters to NWU patients and families
* monthly caregiver support groups. They’ve been asked to split these into two — PPA and bvFTD.
* one conference per year (caregivers and patients)
In the new FTD booklet, there’s a section on PPA:
Strategies for families in managing symptoms of aphasia:
* Speak in simple sentences to patients — simpler words and simple forms of construction. Don’t use the word “or”!
* Construct a communication notebook for patients. Have a page devoted to “my favorite grocery items.” Take pictures and label them with words. iPhone application says words aloud.
* Devise a strategy for emergency situations bypassing the need to use the telephone
* Seek treatment from a speech-language pathologist
* Provide patient with identifying information, Example – MedicAlert bracelets.
* These are from: Weintraub S, Morhardt DJ: Treatment, education and resources for non Alzheimer dementia: One size does not fit all. Alzheimer’s Care Quarterly, July/September: 201-214, 2005. They are summarized in one of the handouts we received.
Question & Answer:
Q: What about the patient who lives alone?
A: Though some of these patients can’t speak, they may still be highly functional. Need to assess how this person can react to an emergency.
Q: What about depression?
A: This can be a serious problem. Medications can be very helpful. Families should be on the look-out for this.
Talking therapy is not always the best thing!
Comment by audience member: There can be profound loneliness in PPA.
Q: Any learnings from your patient support group?
A: Keep gatherings small. More like one-on-one or two-on-one. Any more people gets confusing for the patient.
Q: How does your patient support group work?
A: The patients demanded their own support group. Over time, the amount of talking has declined. Group leaders now insert activities when there isn’t much talking.
Q: What percentage of PPA patients end up having personality involvement?
A: We don’t know. All of the research has been with small series of patients.
Some studies show that the Semantic patients end up with more emotional/behavioral disturbances. This may be due to frontal lobe involvement.
Q: Is Namenda helpful in PPA?
A: Namenda didn’t help with PPA in a trial they did (3-6 months). There was no short-term benefit. So they assumed there was no long-term benefit.
Comment by audience member: Namenda helped his wife for several years.
Q: What portion of FTDs are PPA?
A: We don’t know. Northwestern sees a huge number of PPA patients.
Q: What do we know about genetic patterns with PPA?
A: There are only two PPA families we know of where it seems to be inherited.
In families, where someone has PPA, other family members (first degree relatives) may have early learning disorders (eg, dyslexia).
We haven’t followed those with early learning/language disorders to find out if they develop neurodegenerative diseases later.
Q: What are the pathological diagnoses for PPA?
A: We do have data on this:
60% of all PPA have some form of FTLD pathology (whether it’s tauopathy, FTDP43, CBD, PSP, etc).
40% have Alzheimer’s pathology.
If you have the logopenic form of PPA, you are more likely to have the Alzheimer’s form.
If you have the grammar form of PPA, you are more likely to have a tauopathy.