Category Archives: FTD

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.

Robin

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:
http://forum.psp.org/viewtopic.php?t=8410

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:
http://forum.psp.org/viewtopic.php?t=8575

FTD Support Group Leaders’ Conference Call
Organizer: Association for Frontotemporal Dementias (ftd-picks.org)
12/20/10

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)
Chicago, IL
www.brain.northwestern.edu/ppa/

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.

Later Symptoms:
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:
www.nia.nih.gov/Alzheimers/Publications/FTLD/

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.

PSP and CBD folks –

A great new publication, Frontotemporal Disorders: Information for Patients, Families and Caregivers,has just been released by the National Institute on Aging and NIH.  It is free and available by download or in hardcopy via the following link:  [Editor’s note: link modified in 2017]

www.nia.nih.gov/alzheimers/publication/frontotemporal-disorders/introduction

Jennifer Merrilees, RN, from UCSF’s MAC, assisted in the creation of this booklet along with people from Northwestern University and the Association for Frontotemporal Dementias.

I’ve copied below some excerpts that are on terminology, CBS, and PSP.  I especially liked the terminology section (“FTD? FTLD? Understanding Terms”) and the section on symptoms (with a breakdown by type of symptom — behavioral, language, emotional, and movement).  Of course not all of the symptoms listed occur in each disorder nor in each person with a given disorder.

Robin

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www.nia.nih.gov/alzheimers/publication/frontotemporal-disorders/introduction

Excerpts from

Frontotemporal Disorders: Information for Patients, Families, and Caregivers
NIA and NIH
Published October 2010

FTD? FTLD? Understanding Terms
One of the challenges shared by patients, families, clinicians, and researchers is confusion about how to classify and label frontotemporal disorders. A diagnosis by one doctor may be called something else by a second, and the same condition or syndrome referred to by another name by a pathologist who examines the brain after death.

For many years, scientists and physicians used the term frontotemporal dementia (FTD) to describe this group of illnesses. After  further research, FTD is now understood to be just one of several possible variations and is more precisely called behavioral variant frontotemporal dementia, or bvFTD.

This booklet uses the term frontotemporal disorders to refer to changes in behavior and thinking that are caused by underlying brain diseases collectively called frontotemporal lobar degeneration (FTLD). FTLD is not a single brain disease but rather a family of neurodegenerative diseases, any one of which can cause a frontotemporal disorder (see “Causes,” page 11). Frontotemporal disorders are diagnosed by physicians and psychologists based on a person’s symptoms. FTLD can be identified definitively only by brain autopsy after death.

Types of Frontotemporal Disorders
Frontotemporal disorders can be grouped into three types, defined by the earliest symptoms physicians identify when they examine patients.

• Progressive behavior/personality decline—characterized by changes in personality, behavior, emotions, and judgment (e.g., behavioral variant frontotemporal dementia).

• Progressive language decline—marked by early changes in language ability, including speaking, understanding, reading, and writing (e.g., primary progressive aphasia).

• Progressive motor decline—characterized by various difficulties with physical movement, including shaking, difficulty walking, frequent falls, and poor coordination.

Types of Frontotemporal Disorders
Diagnostic Terms and Main Early Symptoms
Progressive Motor Decline

Corticobasal syndrome (CBS)
• Muscle rigidity
• Difficulty closing buttons, operating simple appliances
• Language or spatial orientation problems

Progressive supranuclear palsy (PSP)
• Progressive problems with balance and walking
• Slow movement, falling, body stiffness
• Restricted eye movements

FTD with parkinsonism
• Movement problems similar to Parkinson’s disease, such as slowed movement and stiffness
• Changes in behavior or language

FTD with amyotrophic lateral sclerosis (FTD-ALS)
• Combination of FTD and ALS (Lou Gehrig’s disease)
• Changes in behavior and/or language
• Muscle weakness, shrinkage, jerking

Movement Disorders
Two rare neurological disorders associated with FTLD, corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP), occur when the nerves attached to muscles malfunction and cause problems with movement. The disorders may affect thinking and language abilities, too.

CBS is caused by corticobasal degeneration—gradual atrophy and loss of nerve cells in specific parts of the brain. This degeneration causes progressive loss of movement and muscle rigidity, typically beginning around age 60. Symptoms may appear first on one side of the body, but eventually both sides are affected. Occasionally, a person with CBS first has language problems or trouble orienting objects in space and later develops movement symptoms.

PSP causes serious problems with balance and walking. People with the disorder typically move slowly, fall, and have body stiffness, especially in the neck and upper body—symptoms similar to those of Parkinson’s disease. A hallmark sign of PSP is trouble with eye movements, such as involuntary closing of the eyelids, difficulty opening and closing the eyes, trouble looking way up and way down, and limited blinking. These symptoms may give the face a fixed stare. Behavior problems can also develop.

Other movement-related frontotemporal disorders include frontotemporal dementia with parkinsonism and  frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS).

Frontotemporal dementia with parkinsonism is an inherited disease caused by a genetic mutation. Symptoms include movement problems similar to those of Parkinson’s disease, such as slowed movement, stiffness, and balance problems, and changes in behavior or language.

FTD-ALS is a combination of bvFTD and ALS, commonly called Lou Gehrig’s disease. Symptoms include the behavioral and/or language changes seen in bvFTD as well as the muscle weakness, shrinking, and jerking seen in ALS. Symptoms of either disease may appear first, with other symptoms developing over time. Mutations in certain genes have been found in some patients with FTD-ALS.

Confusing symptoms
Carol had a tingling sensation and numbness in her upper right arm. Then her arm became stiff. She had to change from cursive handwriting to printing. Carol, 61, told her doctor that she had trouble getting her thoughts out and  described her speech as “stumbling.” She had increasing trouble talking but could still understand others. Eventually, she was diagnosed with CBS.

Trouble with walking
For a year and a half, John had trouble walking and fell several times. He also had trouble concentrating. He couldn’t read because the words merged together on the page. John, 73, also seemed less interested in social activities and projects around the house. His wife noticed that he was more irritable than usual and sometimes said  uncharacteristically inappropriate things. John’s primary care doctor did several tests, then referred him to a  neurologist, who diagnosed PSP.

Common Symptoms
Symptoms of frontotemporal disorders vary from person to person and from one stage of the disease to the next as different parts of the frontal and temporal lobes are affected. In general, changes in the frontal lobe are associated with behavioral symptoms, while changes in the temporal lobe lead to language and emotional disorders.

Symptoms are often misunderstood. Family members and friends may think that a person is misbehaving, leading to anger and conflict. For example, a person with bvFTD may neglect personal hygiene or start shoplifting. It is important to understand that people with these disorders cannot control their behaviors and other symptoms.

Behavioral Symptoms
• Problems with executive functioning—Problems with planning and sequencing (thinking through which steps come first, second, third, and so on), prioritizing (doing more important activities first and less important activities last), multitasking (shifting from one activity to another as needed), and self-monitoring and correcting behavior.

• Perseveration—A tendency to repeat the same activity or to say the same word over and over, even when it no longer makes sense.

• Social disinhibition—Acting impulsively without considering how others perceive the behavior. For example, a person might hum at a business meeting or laugh at a funeral.

• Compulsive eating—Gorging on food, especially starchy foods like bread and cookies. People may take food from other people’s plates.

• Utilization behavior—Difficulty resisting impulses to use or touch objects that one can see and reach. For example, a person picks up the telephone receiver while walking past it when the phone is not ringing and the person does not intend to place a call.

Language Symptoms
• Aphasia—A language disorder in which the ability to use or understand words is impaired but the physical ability to speak properly is normal.

• Dysarthria—A language disorder in which the physical ability to speak properly is impaired (e.g., slurring) but the message is normal.

Emotional Symptoms
• Apathy—A lack of interest, drive, or initiative. Apathy is often confused with depression, but people with apathy may
not be sad. They often have trouble starting activities but can participate if others do the planning.

• Emotional changes—Emotions are flat, exaggerated, or improper. Emotions may seem completely disconnected from a situation or are expressed at the wrong times or in the wrong circumstances. For example, a person may laugh at sad news.

• Social-interpersonal changes—Difficulty “reading” social signals, such as facial expressions, and understanding personal relationships. People may lack empathy—the ability to understand how others are feeling—making them seem indifferent, uncaring, or selfish. For example, the person may show no emotional reaction to illnesses or accidents that occur to family members.

• Changes in insight—An inability to understand changes in one’s condition and how they affect others. This lack of awareness is hard for caregivers to deal with because the person may reject efforts to help.

Movement Symptoms
• Dystonia—Abnormal postures of the hands or feet. A limb may be bent stiffly or not used when performing activities that are normally done with two hands.

• Gait disorder—Abnormalities in walking, such as walking with a shuffle, sometimes with frequent falls.

• Tremor—Shakiness, usually of the hands.

• Clumsiness—Dropping of small objects or difficulty manipulating small items like buttons or screws.

Managing Movement Problems
No treatment can slow down or stop frontotemporal-related movement disorders, though medications and physical and occupational therapy may provide modest relief.

For people with corticobasal syndrome (CBS), movement difficulties are sometimes treated with medications for depression, Alzheimer’s disease, or Parkinson’s disease. But these medicines do not often lead to much  improvement. Physical and occupational therapy can help a person with CBS move more easily. Speech therapy may delay the worsening of language symptoms.

For people with progressive supranuclear palsy (PSP), sometimes Parkinson’s disease drugs provide temporary relief for slowness, stiffness, and balance problems. Although physical therapy generally does not help, exercises can keep the joints limber, and weighted walking aids—such as a walker with sandbags over the lower front rung—can help maintain balance. Speech, vision, and swallowing difficulties usually do not respond to any drug treatment. Antidepressants have shown modest success. For people with trouble looking up or down, bifocals or special glasses called prisms are sometimes prescribed. …

For any movement disorder caused by FTLD, a team of experts can help patients and their families address difficult medical and caregiving issues. Physicians, nurses, social workers, and physical, occupational, and speech therapists who are familiar with frontotemporal disorders can ensure that people with movement disorders get appropriate medical treatment and that their caregivers can help them live as well as possible.

Caring for a Person with a Frontotemporal Disorder
In addition to managing the medical and day-to-day care of people with frontotemporal disorders, caregivers can face a host of other challenges. These challenges may include changing family relationships, loss of work, poor health, decisions about long-term care, and end-of-life concerns.

Family Issues
People with frontotemporal disorders and their families often must cope with changing relationships, especially as symptoms get worse. …

Work Issues
Frontotemporal disorders disrupt basic work skills, such as organizing, planning, and following through on tasks. Activities that were easy before the illness began might take much longer or become impossible. People lose their jobs because they can no longer perform them. As a result, the caregiver might need to take a second job to make ends meet—or reduce hours or even quit work to provide care and run the household.

Caregiver Health and Support
Caring for someone with a frontotemporal disorder can be very hard, both physically and emotionally. To stay healthy, caregivers can do the following:

• Get regular health care.

• Ask family and friends for help with child care, errands, and other tasks.

• Spend time doing enjoyable activities, away from the demands of caregiving. Arrange for respite care—short-term caregiving services that give the regular caregiver a break—or take the person to an adult day care center, a safe, supervised environment for adults with dementia or other disabilities.

• Join a support group for caregivers of people with frontotemporal disorders. Such groups allow caregivers to learn coping strategies and share feelings with others in the same position.

The organizations listed in the Resources section of this book (starting on page 27) can help with information about caregiver services and support.

Long-Term Care
For many caregivers, there comes a point when they can no longer take care of the person with a frontotemporal disorder without help. The caregiving demands are simply too great, perhaps requiring around-the-clock care. As the disease progresses, caregivers may want to get home health care services or look for a residential care facility, such  as a group home, assisted living facility, or nursing home. The decision to move the person with a frontotemporal disorder to a care facility can be difficult, but it can also give caregivers peace of mind to know that the person is safe
and getting good care.

End-of-Life Concerns
People with frontotemporal disorders typically live 6 to 8 years with their conditions, sometimes longer, sometimes less. Most people die of problems related to advanced disease. For example, as movement skills decline, a person can have trouble swallowing, leading to aspiration pneumonia, in which food or fluid gets into the lungs and causes infection. People with balance problems may fall and seriously injure themselves.

It is difficult, but important, to plan for the end of life. Legal documents, such as a will and durable powers of attorney for health care and finances, should be created or updated as soon as possible after a diagnosis of bvFTD, PPA, or a related disorder. Early on, many people can understand and participate in legal decisions. But as their illness progresses, it becomes harder to make such decisions.

A physician who knows about frontotemporal disorders can help determine the person’s mental capacity. An attorney who specializes in elder law, disabilities, or estate planning can provide legal advice, prepare documents, and make financial arrangements for the caregiving spouse or partner and dependent children. If necessary, the person’s access to finances can be reduced or eliminated.

Resources

Federal Government

National Institute on Aging
www.nia.nih.gov/Frontotemporal

National Institute of Neurological Disorders and Stroke
www.ninds.nih.gov

MedlinePlus
www.medlineplus.gov

Organizations

Association for Frontotemporal Dementias
www.ftd-picks.org

ALS Association
www.alsa.org

CurePSP
www.curepsp.org

National Aphasia Association
www.aphasia.org

WE MOVE
www.wemove.org

Caregiver Services

Eldercare Locator
www.eldercare.gov

Family Caregiver Alliance
www.caregiver.org

National Academy of Elder Law Attorneys, Inc.
www.naela.org

Caregiver Support

FTD Support Forum
www.ftdsupportforum.com

PPA Support Group
www.groups.yahoo.com/group/PPA-support

Well Spouse Association
www.wellspouse.org

Diagnosis, Treatment, and Research Centers  [many are listed, including…]

University of California, San Francisco
Memory and Aging Center
San Francisco, CA
1-415-476-6880
www.memory.ucsf.edu/ftd