Everything Is Just Fine: Anosognosia in Frontotemporal Degeneration
Partners in FTD Care, Winter 2019
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Caring for people with FTD presents challenges no matter what. Anosognosia can make it even more difficult. Anosognosia is the inability to recognize or perceive one’s illness and its associated limitations. Also referred to as “lack of insight,” anosognosia is a hallmark symptom of FTD, especially in behavioral variant FTD. People who present with anosognosia display a profound lack of emotional concern about their disease and its impact on their family members. Even those who acknowledge they have a brain disorder may deny they are upset or worried about their condition. Anosognosia is one of the main reasons people with FTD refuse medication and medical/personal care. People with anosognosia may be able to function normally in some areas of their lives, yet demonstrate risky behavior elsewhere because they do not recognize their limitations.
The Case of Roger A.
Roger A. is a 60-year-old farmer from Ohio. He lived on his family’s Century Farm his entire life, raising seed corn, wheat and cattle. After college, he spent several years in the Navy, then spent the rest of his adult years working the farm.
At 23, he married his high school sweetheart, Alma, and raised two sons and a daughter; all three work in the family business and are married with children. Roger was president of his local Rotary and was a deacon in his church. A strapping man with a booming voice, he was usually assertive about getting his way.
Roger began to change in his early 40s. He became irritable and stopped paying attention to farm prices. He stopped attending church regularly and began to collect muscle cars. A lifelong hunter, he began to add pistols and semi-automatic weapons to his gun collection. Believing the guns weren’t loaded, Alma allowed this—until the day their oldest grandson picked up a gun and fired a bullet though the living room floor. Roger was outraged that the child had touched his gun but still refused to use his gun safe. For their own protection, the grandchildren were told not to be around Roger unless supervised by another adult.
On his 44th birthday, Roger quit his civic positions following rumors that he had made sexual advances toward women at church. Alma did not believe the rumors but threatened divorce if they proved true.
Shortly thereafter, Roger said he wanted to move to Sun City, Arizona. He and Alma purchased a second home there when he was 45—but a year later, he sold the house, saying he did not like his “female neighbors.” Instead, the couple bought an apartment in a posh golf community in Arizona and planned to spend their winters there.
Back in Ohio, Roger started openly spending time with Heather, a divorced beautician. Mortified, Alma tried to get him to give up the affair. He refused and began taking Heather to Arizona with him for the winter. Increasingly stressed by Roger’s odd behavior, Alma was almost relieved to stay in Ohio and spend time with their grandchildren.
One day while driving to work, Roger’s daughter got a call from her father. He demanded she pull over, remove her shirt, take a cell phone picture of her naked breasts and send it to him immediately. This shocking request prompted a full-blown family crisis. Alma knew she had to act: She insisted Roger see their primary care physician for help.
Roger went to the appointment alone and told the doctor the only thing wrong was Alma: She was “frigid,” he said. The doctor suggested the couple was experiencing marital discord. Alma arranged for counseling, hoping it would ease the tension in the family. But counseling proved frustrating and unsuccessful, as Roger became more self-centered and inflexible. He continued seeing Heather, unable to comprehend Alma’s unease with the situation. He insisted that there was nothing wrong with him, even telling the therapist that he wanted a divorce so that he could pursue younger women.
Roger’s spending habits grew more reckless. He bought two antique tractors, as well as a new combine and tractor every spring for three years. He ordered hunting gear and began to haunt farm sales, collecting old equipment. The family continued running the business, but Alma was concerned that Roger’s erratic behavior would put their livelihoods in jeopardy. Neither had named a durable power of attorney for financial or healthcare decision-making. On the advice of a doctor, Alma and her eldest son began to seek a diagnosis so they could apply for guardianship of Roger. But he kept insisting that nothing was wrong, that he was still running the business well and that his marriage was “better than ever.”
Roger’s family nevertheless convinced him to see a series of specialists. The fourth specialist diagnosed him with bipolar disorder. Treatment efforts failed, however, and Roger said he was done seeing doctors. After an essentially normal neuropsychological battery, a magistrate denied Alma’s request for guardianship.
In the midst of this trying ordeal, Alma began researching a condition one of the specialists had mentioned in passing: FTD. Roger’s symptoms—disinhibition, lack of awareness, changes in mood—seemed to match those common in behavioral variant FTD (bvFTD). Notably, memory loss was not listed as a primary symptom. Most standard cognitive evaluation tools focus largely on memory and do not adequately assess the frontal executive skills or behavioral changes caused by FTD. Alma recalled that Roger’s evaluations had primarily assessed memory. Her research highlighted the importance of finding a doctor with experience diagnosing and treating FTD. At the recommendation of the elder law attorney helping with guardianship, Roger’s family insisted he see a behavioral neurologist, who diagnosed him with bvFTD at age 47.
Despite a measure of relief that came with finally getting a diagnosis, Alma had reached her limit and told her children she would be pursuing a divorce. She was increasingly frightened of Roger and upset about his relationship with Heather—he had even tried to convince Alma to let her move in with them at the farmhouse. When Alma told her husband she wanted a divorce and would be moving into an apartment by herself, he did not understand, saying, “There’s nothing wrong with me.”
Roger became angrier and more aggressive. Both Alma and Roger’s doctor agreed he had become a danger to himself and others. He spent two weeks in a geriatric psychiatric unit, the safest inpatient option due to his dementia, and started medications to address his agitation. Staff recommended a more structured supportive living situation, but he refused. After being discharged, Roger relocated to Arizona but could not keep his apartment clean or independently manage his daily needs. He refused to take his medications, and the stress of his rapidly changing life made him agitated and argumentative. His children quickly relocated him to an assisted living center in Ohio, but he required more supervision than the program could provide. He was discharged after repeatedly fondling women. He did better after moving into a more structured assisted living facility, although he regularly resisted directions from the predominantly female staff. He denied his illness the entire time, asserting that he was perfectly healthy.
Meanwhile, Alma put the divorce on hold while she got her family’s affairs in order. To save the farm and plan for the costs of Roger’s care, Alma sold it to her children. Knowing her husband would not understand why she agreed to the sale—after all, he still did not recognize he had FTD—Alma followed the advice of Roger’s doctor and told a white lie. She told Roger the farm’s property taxes were overdue and the farm was bankrupt. Believing Alma’s story, Roger consented to the sale.
With the matter resolved, Alma reinitiated divorce proceedings. Afraid of what the community would think, she limited her social outings to church and meetings of her local FTD support group. Heather remained involved with Roger and assisted with his care.
For several years, Roger would stay in assisted living for most of the year and spend winters in Arizona with Heather. Eventually, Alma and the children offered to pay Heather a modest salary to care for him full-time. Heather accepted and moved Roger into her home in Ohio for a trial run.
As his FTD progressed, Roger needed greater assistance with activities of daily living, particularly following a mild stroke at age 57. Nevertheless, he continued to insist that he was completely normal, and actually grew more resistant to Heather’s attempts to help. Exhausted from these constant conflicts, Heather began to burn out. She learned that contradicting or correcting Roger only made him angry; he began to threaten her physically during confrontations. His doctor prescribed quetiapine (50 mg at bedtime) for aggression.
Roger began to develop aphasia. He could no longer read or produce words, and began having difficulty eating. He would stuff his mouth until swallowing was physically impossible. Heather took a caregiver training course and learned to feed him slowly; she eventually switched him to a milkshake diet to prevent choking.
At age 58, Roger refused to allow even Heather to provide personal care. He occasionally experienced incontinence and would hide his soiled clothing in the drawer with his clean underwear. Each time Heather corrected him for doing so, he became belligerent. Frustrated, Heather spoke to a dementia care manager who recommended incontinence garments. She also bought pet stain remover to clean rugs and furniture, and to prevent upholstery from rotting.
Roger’s FTD kept worsening. He began to eat compulsively and gained weight, causing mobility problems; Heather resorted to hiding food in a locked fridge. He refused all bathing and grooming. Roger’s doctor prescribed additional medication to manage his angry outbursts.
Realizing she could no longer manage Roger on her own, Heather tried to enroll him in an afternoon adult day respite program, but he refused to go. Then, with the help of Roger’s children, she found a specialized memory care facility for him. On the second day, he escaped; on the third, he tried to fondle two women and was asked to leave. Roger was then accepted to a Veterans Affairs Nursing Home with mostly male residents and care staff. He was placed on a secured unit. The staff initiated a meeting with his family, who agreed to try medication to address his aggressive behavior. Roger’s issues decreased due to medication, less contact with women and positive behavioral interventions.
Advanced disease and hospice
Now 60, Roger has seen a decline in his health over the last several months. He is dependent in all activities of daily living except walking, although he falls approximately twice per week. Care staff redirects him to a chair without foot pedals for easier mobility. He has become nonverbal but will have periodic spontaneous vocalizations. His resistance to care and prior aggressive behaviors have faded. Doctors reduced his medication to only those necessary to manage his restlessness. He cannot eat without choking and is on a full thick-liquid diet.
After Roger went through two bouts of aspiration pneumonia and a urinary tract infection, the staff spoke with Alma and Heather about the benefits of hospice care. Both women recognized how fragile his health had become and agreed to focus fully on his comfort. He entered hospice and is now in a quiet room, spending time with occasional visitors. Staff read and sing to him. He no longer takes food or fluids. Alma and Heather spend time by his side daily.
Questions for discussion:
Was Roger in denial of his illness?
No. There is an important difference between “denial” and “anosognosia.” Denial is a psychological defense mechanism or way of coping with an unpleasant or painful situation. When that situation is resolved, the denial response is no longer needed. Anosognosia is the result of damage to the brain’s right parietal lobe and/or frontal lobe, the anterior cingulate, and the orbitofrontal cortex, which is irreversible. Whereas denial is generally temporary, anosognosia will only worsen as the disease process continues.
Did the consequences of Roger’s behavior— including Alma’s response to that behavior— help him to recognize his disease and adjust to his limitations? Did medication help?
Nothing Alma, Heather or professionals said or did changed Roger’s lack of insight into his difficulties, nor his lack of concern about how his FTD impacted himself and his family. Attempting to reason with someone with anosognosia is generally ineffective, as are activities designed to help them understand they have an illness. Although you might get the person to verbally acknowledge their disease, the accompanying emotional indifference prevents them from caring about, or changing, their aberrant behaviors. Despite efforts from Alma and Heather, Roger kept saying he felt fine; he resisted efforts to help with activities of daily living and became aggressive when questioned about his actions.
There are no medications that can reverse or treat anosognosia. Carefully prescribed medications may help to lessen agitation or aggressive behavior, but they will not change the lack of self-awareness.
How could Roger develop reckless and inappropriate behavior and still be able to function independently in other areas?
The location of disease in the brain determines the earliest symptoms and functional impairment. Research has suggested that anosognosia in bvFTD is associated with atrophy in parts of the right temporal lobe that have been linked to a person’s ability to evaluate how others respond to their behavior. For a time, Roger retained the complex cognitive skills involved in driving a car, but was so behaviorally impaired that he demanded nude photos of his daughter. This disparity is a source of great confusion for many family members and loved ones, but it can be explained by the fact that FTD is a progressive disease, gradually impairing different parts of the brain—and therefore different brain functions—as it worsens.
How could Roger’s behavior and relationships change so much, yet he continued to think nothing was wrong?
Due to damage in his frontal lobe, Roger was not able to recognize how much his personality had changed and how different his actions and decisions were from before. For many people, behavioral variant FTD (bvFTD) irreversibly damages the brain circuits involved in recognizing and maintaining our own self-image. Roger therefore could not understand how his new behaviors conflicted with the self-image he maintained: Essentially, he thought he was the same person he had always been. Despite the obvious changes in his personality, judgment and behavior, as well as the changes in his relationships, Roger believed that he was still running his business well and that his marriage was better than ever.