Summer 2017
Atypical Dementias

Frontotemporal Degeneration: Explaining the Importance of Differential Diagnosis

ABOUT THE AUTHORS

Brad Dickerson, MD
Associate Professor of Neurology, Harvard Medical School
Director, Frontotemporal Disorders Unit and Laboratory of Neuroimaging
Massachusetts General Hospital
149 13th Street, Suite 2691
Charlestown, MA 02129 USA
617-726-5571
brad.dickerson@mgh.harvard.edu
http://www.ftd-boston.org/

Brad Dickerson, MD is Associate Professor of Neurology at Harvard Medical School and Director of the Frontotemporal Disorders Unit at the Massachusetts General Hospital, an integrated multidisciplinary unit dedicated to the highest level of care of patients with these conditions. Dr. Dickerson is also on staff as a behavioral neurologist in the MGH Memory Disorders Unit. Dr. Dickerson is an active clinical consultant in many aspects of cognitive and behavioral neurology of neurodegenerative and related disorders, including frontotemporal dementia, primary progressive aphasia, Alzheimer’s, mild cognitive impairment, posterior cortical atrophy, and related conditions, and the use of neuroimaging and other diagnostic markers in neurodegenerative diseases.

Dr. Dickerson has published extensively in the field of neurodegenerative disease, neuroimaging, aging, and cognitive neuroscience. He is on the advisory board of the Massachusetts Alzheimer’s Association and the national Association for FTD. He is active in teaching, leading an annual course on Cognitive Neurology at the American Academy of Neurology and co-directing the annual Harvard Dementia Course. He is also an active mentor of trainees in neurology, psychiatry, and psychology, and of graduate and medical students, as well as undergraduate and high school students interested in this field.

Dr. Dickerson has published more than 70 manuscripts and book chapters and is in the process of editing 2 textbooks in the field of cognitive neurology and dementia, and runs a brain imaging laboratory affiliated with the Martinos Center for Biomedical Imaging.


Katie Brandt, MS
Caregiver Support Services Director

Frontotemporal Disorders Unit
Massachusetts General Hospital
149 13th Street, Suite 2691
Charlestown, MA 02129
(O) 617.643.2323
(M) 508.944.9610
Katherine.Brandt@mgh.harvard.edu
www.ftd-boston.org

Katie is a caregiver who knows firsthand how essential the support of community is for those affected by rare disease and dementia. Katie lost her husband Mike to the rare disease, Frontotemporal Degeneration (FTD), when he was 33 years old. Shortly thereafter, her father was diagnosed with Alzheimer’s at the age of 59. Today, Katie is an advocate, an educator, and a volunteer in the rare disease and dementia community. Katie’s story of love, loss, and resiliency is unique, but it has universal truths.

Katie has started a new campaign, Love Is Out There, to raise awareness for FTD, caregiving, and empowering families to take the power back from rare diseases. Through social media, community events, and educational forums, Katie and her network will raise awareness for families struggling with FTD and let them know that resources and support are out there. She has spoken at colleges, conferences, hospitals, the Massachusetts State House, and on Capitol Hill to share her experiences.

In the summer of 2012, she contacted The Association for Frontotemporal Degeneration and offered herself as an advocate and a volunteer. It was the beginning of a great relationship and a new career for Katie. Currently she serves as the Caregiver Support Services Director for the Frontotemporal Disorders Unit at Massachusetts General Hospital in Boston.


Jennifer Pilcher Warren, PhD, CMC
Senior Executive Director
Overlook CARE Management
99 Derby Street
Hingham, MA 02043
(781) 837-7444
(617) 605-4605
jpwarren@overlook-mass.org

Ms. Pilcher Warren, was awarded her doctoral degree in Gerontology from the University of Massachusetts in Boston. Her professional experience has focused primarily on care and housing arrangements for elders with Alzheimer’s disease and related dementias, including policy and fundraising work for the Alzheimer’s Association of Eastern Massachusetts, the Alzheimer’s Association of Utah, and the Massachusetts Association of Older Americans. She has held several positions on the board of the New England Chapter of the Aging Life Care Association and is currently the Editor in Chief for the Journal of Aging Life Care. For seven years, Ms. Pilcher Warren was affiliated with Hearthstone Alzheimer Care Assisted Living programs in Massachusetts and New York, where she held a number of management positions. For 8 years, Ms. Pilcher Warren worked both as a Geriatric Care Manager and as the Director of Operations for AZA Care Management of Boston. She currently holds the position of Senior Executive Director of Care Management for Overlook C.A.R.E., a non-profit Life Care Management practice with offices located in Hingham, Dedham, Plymouth, and Charlton, MA.

Brad Dickerson, MD, Jennifer Pilcher-Warren, PhD, CMC, Katie Brandt, MS

Frontotemporal Dementia (FTD) is the name given to a number of brain disorders that primarily affect the frontal and temporal lobes of the brain. They are usually slowly progressive and may affect various aspects of a person’s cognitive function, behavior, language, emotions or personality, and/or movement. FTD is estimated to affect 50,000 – 60,000 Americans and is the 3rd most common neurodegenerative dementia after Alzheimer’s disease and Dementia with Lewy Bodies. The onset for FTD is typically between the ages of 40-70, but onset as early as age 20 has been reported. FTD is the most common type of young onset dementia and is diagnosed most frequently in people between 50-60 years of age. Median life expectancy for people with FTD is 7-8 years, however, the rate of progression varies significantly among patients. Today it is estimated that in approximately 15-40% of all FTD cases, a genetic cause can be identified. Researchers are working in partnership with patients and families to learn more about the connections between family history, genetic mutations, and disease presentation.

The pathology of FTD is much more complex than that of Alzheimer’s disease. With FTD, the damage to the brain begins in the frontal and/or temporal lobe. The initial presentation of the disease can vary depending on whether the frontal or temporal lobe is affected first. When the initial pathology affects the frontal lobes, the main changes are in personality and behavior, known as Behavioral Variant FTD. Alternatively, when the temporal lobe is involved, patients present with loss of language skills, and may be diagnosed with the language variant, Primary Progressive Aphasia (PPA). Further, there are still other variants of FTD that involve movement disorders as well. To learn more about all variants of FTD, go to www.theaftd.org.

At the Massachusetts General Hospital Frontotemporal Disorders Unit, the staff there see many patients with atypical or early-onset cases of dementia with many patients having been diagnosed with either Behavioral Variant FTD (bvFTD), or Progressive Aphasia (PPA). The variants of FTD that involve movement disorders can be even more rare than either bvFTD or PPA. For that reason, this discussion will focus on those two diagnostic categories. For clarity, see the visual below that illustrates how we will divide these categories for the purpose of this discussion.

Behavioral Variant FTD (bvFTD)

Rather than changes in memory, as with Alzheimer’s disease, in the early stages of bvFTD patients typically present with changes in personality, ability to concentrate, social skills, motivation and reasoning. Identifying bvFTD can be challenging because symptoms can be subtle in early stages, and may combine features that are traditionally in the realm of psychiatry (e.g., personality changes, lack of empathy, compulsions). Patients are often first evaluated in general psychiatric settings and about 50% are initially diagnosed with a primary psychiatric illness. Families of those with bvFTD have often been noticing gradual changes in their loved one’s way of behaving and responding emotionally to other people. Memory, language, and visual perception are not usually impaired in the early stages, but will be as the disease progresses.

Behavioral Variant FTD affects “comportment”, i.e., the ability to recognize what behavior is appropriate in a certain social situation and adapt his/her behavior to the situation. However, patients with bvFTD have often lost the ability to see themselves as others see them. For this reason, they are often unable to recognize if they are acting in a socially unacceptable or unreasonable manner. Many patients with bvFTD also lack the ability to recognize their deficits or illness. These changes are often what is recognized early in the disease as “personality changes” by family members.

The most common features of bvFTD are as follows:

Disinhibition

  • Socially inappropriate behavior and/or impulsivity. For example, talking to strangers or displaying embarrassing behavior in public.

Apathy

  • Loss of interest, drive and/or motivation, which can lead to abandoning activities that were enjoyed previously and avoiding social contact.

Loss of sympathy/empathy

  • Diminished response to other’s feelings, diminished personal warmth/social connection, making patients appear selfish and unfeeling.

Repetitive/compulsive/ritualistic behavior

  • Often with no particular purpose, such as repeating patterns of movement, and behaviors like hand wringing, tapping or pacing.

Change in eating/drinking behavior

  • Changes in food preferences, often with a preference for sweet food, excessive intake, or restriction in food choices.

Decline in self care

  • Issues with hygiene or inability to perform ADLs.

Difficulty with reasoning, judgment, organization, and planning

  • Mental activities that promote decision making, being able to categorize information, and move from one perspective of a problem to another — such as managing financial or medical decisions.

Primary Progressive Aphasia (PPA)Primary Progressive Aphasia (PPA)

PPA is a form of FTD that involves a progressive loss of language function. The deterioration of the brain begins primarily in the temporal lobe, the part of the brain that is responsible for speech and language. Many patients initially experience difficulty thinking of common words while speaking or writing. PPA will worsen as the disease progresses to the point where verbal communication is very difficult. In the early stages of PPA, memory, reasoning, and visual perception are not affected, so many patients with this form of the disease are able to function reasonably well in routine daily activities despite their language difficulties. Adults of any age can develop this disease, but it is more common under the age of 65. In many cases, the person with PPA is the first to notice something is wrong and their complaints may be blamed on stress or anxiety.

People with PPA tend to have similar clusters of symptoms. Currently, researchers recognize three sub-types:

Semantic Variant (svPPA) – Word Understanding

  • Characterized by reduced ability to understand language
  • Difficulty understanding single words
  • Loss of word meaning

Non-fluent/Agrammatic Variant (naPPA) – Word Order and Word Production

  • Difficulty with word order and word production
  • Reduced ability to produce speech/language, slow and labored production of words, distortion of speech
  • Tendency to produce the wrong words
  • Grammatical errors
  • Apraxia of Speech –difficulty saying sounds, syllables and words due to inability of brain to control body parts needed for speech such as lips, jaw, and tongue.
  • Stammering or stuttering

Logopenic Variant (lvPPA) – Word Finding

  • Halting speech, difficulty repeating
  • Spelling errors common, naming of objects is impaired
  • Variable speech ability. Speech may be fluent during casual small talk but more challenging when a difficult or precise word needs to be used.
  • More apt to use fillers such as “the thing you use for it”, “you know what I mean” or “whatchamacallit”.

Patients with any of these variants experience difficulty with communication including:

  • Knowing what they want to say but the words won’t come out
  • Adjusting to a new communication style
  • Feeling rushed to get their thoughts out
  • Loss of previously enjoyed activities
  • Decreased comprehension of complex language
  • Feeling self-conscious and less useful
  • Use of vague/general words
  • Paraphasic substitutions of words or phonemes

In addition to a decline in language abilities, some non-language abilities such as memory, attention, judgment, or changes in behavior and personality can be affected. Disinhibited or inappropriate behaviors are more common in Semantic PPA while difficulty with problem solving, multi-tasking movement, and mobility is more common in naPPA.

Other Variants of FTD

There are also 4 other variants of FTD that involve issues with motor skills and movement. However, these diseases are very rare and diagnosed infrequently.

Corticobasal Syndrome

  • Cognitive and memory loss
  • Asymmetric rigidity
  • Apraxia (clumsiness of hand use)

Progressive Supranuclear Palsy (PSP)

  • Behavioral and cognitive loss
  • Parkinsonism
  • Eye movement disturbance

FTD-Motor Neuron Disease/ALS

  • Cognitive loss
  • Behavior impairment
  • Motor symptoms

ALS

  • Motor symptoms
  • Upper and/or lower motorneuron pathways

For all patients with FTD, the disease starts out distinctly as one of these variants, but will progress to involve other domains over time. Depending on the type and location of change in the brain, changes in movement may also occur for all variants of FTD, including: incoordination, slowing or stiffness, changes in walking or an increase in falls, changes in eye movements, and impaired swallowing. Survival for FTD patients is variable and can be anywhere from 2-20 years after the onset of symptoms. Average survival is between 7-8 years for most diagnosis, depending on how early the diagnosis was made.

Care Management & Differential Diagnosis

It is extremely important for Aging Life Care Managers to have as much information about the specific diagnosis of their clients and its symptomology in order to appropriately advise and guide their clients. If you have a client that has received a diagnosis of dementia, but the particular variation has not been identified, there are several reasons why doing so is important.

Practical

There are practical reasons that having a specific diagnosis can help your clients. FTD is a qualifying diagnosis for the “Compassionate Care Allowance” through social security. If a patient has this diagnosis, not only are they entitled to SSDI for income replacement, but  they are fast tracked and can start receiving benefits in as little as 4 weeks. This is significant as compared to other, less conclusive illnesses, such as  Fibromyalgia, where approval may take many months or even years (https://www.ssa.gov/compassionateallowances/ ).

Treatment

A differential diagnosis is important as it will inform treatment, both pharmaceutical and non-pharmacological. For example, for Logopenic PPA, a rare variant of FTD, drugs typically used for Alzheimer’s disease might be helpful. However, these same drugs would not be effective for someone with Behavioral Variant FTD. Non-pharmacologic interventions would also be different. For instance, if a patient with PPA was working with a Speech Therapist, the therapeutic approach and goals could be very different than they would be for treating other illnesses such as stroke or Alzheimer’s disease.

As demonstrated in the above vignette, the approach to Speech Therapy for a patient with PPA is vastly different than the approach that would be used with other diagnoses. If a differential diagnosis had not been made, it is likely the family would not know to seek out a therapist with specific knowledge of PPA. As a result, the patient and family could become increasingly frustrated and hopeless and feel as if the speech therapy was not effective. On the other hand, a Speech Therapist that was specifically trained in PPA, would know that the goals for working with a PPA patient would be very different. The therapist might work with the PPA patient and their caregiver to improve ways to express his/her needs when the patient could not find the right word. The therapist might teach the patient to describe the object that was desired. For example, the patient might describe wanting something “cold…white…I’m thirsty” and the caregiver would work on the skill of identifying that the patient was asking for milk.

Engagement Strategies

Different engagement strategies are needed for patients with FTD than those with Alzheimer’s disease or dementia. You can often engage a patient with Alzheimer’s disease by reminiscing over old family photos or listening to music they used to enjoy. In other words, you can rely on your past knowledge of the patient in order to engage them. With patients with FTD, on the other hand, because there are such strong personality and behavior changes, it is likely that the bvFTD patient will not enjoy the same things that they used to and engagement strategies will have to rely on engaging the patient in the current moment.

As you can see in the above example, the way we engage with patients with FTD must be distinctly different than the strategies we use to engage patients with other kinds of dementia. For many families of FTD patients, they feel as if the person sitting in front of them looks like the person they knew, but doesn’t act or feel at all like he/she once did. The daughter in the first example can revisit her relationship with her father and can refer to things they have had in common in the past. The wife in the second example, however, will have to totally redesign her relationship with her husband in order to engage him. (As it turns out, the wife and staff discovered later that Mike really enjoys musicals such as Annie and Grease and found many wonderful ways to engage him in that activity).

Behavioral Strategies

Differential diagnoses are vitally important for informing the strategies we will use to redirect and address difficult behavior. When memory loss is the primary symptom a patient is experiencing, utilizing “therapeutic fiblets” can often be effective. (A therapeutic fiblet is a term that refers to telling a version of the truth in order to avoid upsetting a person with cognitive impairment or to help him/her make a decision in their best interest). In working with a patient with FTD, however, this approach would only be effective if the patient had compromised executive function. Here is an example; we have an FTD patient at MGH who lives in an Assisted Living Community. His wife visits each week and each week she tells him he needs to stay as she is traveling for work. Because this patient is not good at assessing time, this approach works and allows the patient to happily live in the Assisted Living Community. However, if he did have a sense of time and a strong memory, this approach would not work.

Additionally, strategies for addressing risky behavior in FTD patients are different as well. Due to disinhibition, problems with executive function and compulsiveness, patients with FTD put themselves in danger more often than those with Alzheimer’s disease or memory loss. People with FTD may be more apt to make choices that are not good for their health, such as having multiple sexual partners or eating sugary foods even though they are diabetic. An FTD patient can be functional with finances, speech is intact, and they can get themselves to the bank. However, they are so impulsive they can ruin their finances by investing in risky situations or spending all their money.

Conversations about potential risks must be had earlier with patients and their families in order to avoid potential dangers. For example, while an FTD patient can often drive safely and functionally and will not get lost in the car, he/she may blow through a stop sign because they are so inner focused and have less impulse control. A person with PPA might have the memory to get where they are going, but if they get pulled over because a head light is out, the police might think their speech impairment is due to being intoxicated or high and will arrest them for driving under the influence. For these reasons, a patient with FTD may need to cease driving at an earlier time than a person with Alzheimer’s disease.

Caregiver Support

The Care Manager should also be aware of the additional strain these patient’s caregivers are experiencing. Burden and stress is higher among FTD caregivers than among caregivers of patients with Alzheimer’s disease or other dementias. This is most likely due to the fact that it is so much more difficult for caregivers of FTD patients to connect with their loved ones because of changes in personality and preferences. The young age of these patients is also very challenging for their caregivers, who are frequently spouses. Caregivers of these patients have often experienced major financial difficulties due to their spouse leaving the work force. Many of them are forced to continue working and leave their spouse at home. In addition, many of them are caring for the FTD patient as well as young children at the same time.

Moreover, there is a scarcity of resources for these caregivers. Many of the interventions for caregivers, such as support groups and educational programs, are typically tailored for patients with Alzheimer’s disease and are not suitable for FTD caregivers. If the caregiver is forced to place their loved one with FTD in a facility, they are faced with the reality that most nursing homes, assisted living facilities, and day centers are tailored to older clients. As a result, even when placement is achieved, staff at these facilities are frequently unable to cope with the symptoms of FTD. For all of these reasons, the support and guidance of an Aging Life Care Manager is essential for these caregivers to sustain and care for themselves as they continue to care for their loved one with FTD.

Resources for FTD

*Remind your families that the Alzheimer’s Association can support families with all kinds of dementia.  Some helpline staff have even received specialized training in the rare dementias.

REFERENCES


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