Background and Early Changes

Mr. P grew up in a small town. After leaving to serve in the armed forces, he returned to his wife Jane and baby girl Eva. He worked his way up to manager in a large local clothing store, and was active in the church. Mr. P was well liked and respected in town, and was known as a principled, quiet man who enjoyed long walks. He and his wife had a close marriage. His family had mostly moved away from the town by the time they’d settled there. Jane’s family, meanwhile, lived in Europe, where she was originally from.

When Mr. P was 46, he was laid off from his job. He told his wife the store was downsizing. She was concerned about his unemployment, but did not press him for details. He started working from home, turning his hobby of personal investing into a small business, with friends from around town and from church as his clients.

Jane started to notice changes in his behavior. For example, sometimes when she began to tell him about her day at work, he would just walk off. He started taking long, rambling walks, often to other towns, without telling Jane, who would be unable to reach him. The first time this happened, she called the police after he’d been missing for six hours, fearing something might have happened to him. He turned up after a day, seeming unconcerned that he had caused such worry. He was vague about where he had been and told her he “got a ride” back. He did not explain why he left without telling her, or why he never got in touch.

Jane grew concerned that her husband’s behavior might be due to depression over losing his job. He agreed to see a psychiatrist and was placed on an antidepressant. While Mr. P’s behavior still seemed different to her, he did not complain of feeling depressed, nor did he seem particularly sad. He continued to take long walks, however, and seemed uninterested in his family and his church. Jane assumed full childcare responsibilities while still working. She worried that he might be having an affair, leading her to become depressed and anxious, though she’d always been an upbeat and positive person. She felt alone in her husband’s hometown, so far from her own family.

One day, Jane overheard her husband take a business call from a client. She was horrified to hear Mr. P offering the client an “investment opportunity” that sounded to her like a scam—and even more horrified to hear that he himself had invested in this scam. She went to the bank to discover that their savings account had been cleared out. When she confronted him, he seemed indifferent to the fact that their life savings were now tied up in worthless land in another state. He also seemed not to care that he had done this without consulting his wife.

Depression or Something Else?

At this point Jane started worrying that something besides depression or another woman was affecting her husband; she wondered if he had a brain tumor. She was embarrassed about what people in their small town, especially his clients, might be thinking, and she prayed that they were not listening to his financial advice.

She asked her husband to see his doctor. Upon his return, Mr. P said he “checked out healthy.” Jane followed up by calling her husband’s doctor herself to report that he had been acting out of character, but the doctor assured her that everything seemed fine. Jane grew a little more frantic and questioned whether there was something wrong with her judgment. Nevertheless, she scheduled her husband to see a neurologist in the nearest city. She accompanied him on the two-hour journey but did not sit in on the appointment. The neurologist found Mr. P’s mental status testing and neurological examination to be normal. A brain scan revealed no strokes or tumors to explain his change. The neurologist suggested that he follow up with his psychiatrist for better treatment of his depression.

By age 49, Mr. P had stopped his financial-planning business; Jane had noticed that he began having trouble recognizing who he was talking to on the phone. He was becoming more withdrawn, and spent a lot of time picking up sticks in the yard. On his walks, which were now becoming more circumscribed, he started gathering feathers and broken glass; the house grew increasingly cluttered with these collections. His hygiene suffered. Eva, now in high school, stopped bringing friends over. She and her mother were both bewildered by his behavior, but Eva was also angry at her mother for “putting up with him,” and started rebelling.

A Diagnosis Without Explanation

Jane was getting more worried about leaving her husband alone while she was at work. At another visit to the doctor, Mr. P was referred for neuropsychological testing, also two hours away. Mr. P and Jane both attended that appointment. In most of the tests, he scored within normal range, except for a slightly lower-than-expected performance in attention and some executive function tasks.

On their own the test results were inconclusive. But, combined with the information Jane provided about her husband’s history, the results convinced the neuropsychologist that Mr. P should be re-evaluated by a neurologist. Mr. P complained about making another long trip for tests he did not think he needed, but at Jane’s urging, he agreed to go.

The neurologist reviewed his previous brain scan, and, without providing any information or counselling about the disease, diagnosed Mr. P with frontotemporal dementia (another name for behavioral variant FTD). Jane left with no guidance about what FTD entailed, what to expect or how to plan, and there was no follow-up appointment scheduled.

Jane asked friends and neighbors to stay with Mr. P while she worked, and sometimes she brought Mr. P with her to her job. Everyone was very kind, since they knew and remembered Mr. P, and they recognized that he now had a disease that changed his behavior.

At age 52, Mr. P started walking around the house naked, even in front of neighbors and his daughter. This behavior was particularly difficult for Jane to understand and to manage. While she knew he had FTD, she did not know if there were any medicines or treatments for his troubling behaviors.

Jane contacted neurologists at the hospital of a large regional medical center 90 minutes away for advice. The doctor confirmed Mr. P’s diagnosis, but did not offer any helpful treatments or advice. Not long after that appointment Jane made the difficult decision to place him in a nursing home, with financial help from Mr. P’s family and church congregants. Jane and Eva were able to start repairing their relationship, and visited Mr. P regularly. The nursing home staff knew Mr. P’s diagnosis but had not served anyone with FTD before.

His disease progressed to a severe stage; he could no longer communicate verbally. Nearly two years into Mr. P’s stay, a researcher visited the nursing home. While Mr. P could not participate in the study, Jane appreciated the opportunity to speak with someone with experience and an interest in FTD.

Two months later, Mr. P died suddenly. No autopsy was performed. Jane reported that his death left her with an empty loss that was worse than having him alive, even though he was no longer the person he had been.

Questions for Discussion

What changes in Mr. P caused Jane to feel this was different from depression?

It is not uncommon for unexplained symptoms to be diagnosed as a mood disorder, such as depression. But increasingly Jane became aware of uncharacteristic behavioral changes that made her question the initial diagnosis. Mr. P took frequent long walks without telling her where he was going. His judgment declined; he fell prey to a real estate scam and jeopardized their future finances. He showed a notable lack of concern for Jane’s emotional needs: specifically, her anxiety over his health and Eva’s rebellion. Once depression was established as the diagnosis, there was a tendency to ascribe all Mr. P’s unexplained changes to it, and to simply continue treating him as a depressed person. But Jane did not sense depression in Mr. P, and he never complained about being depressed himself.

What did Jane do that facilitated getting the diagnosis?

Her concerns persisted as Mr. P’s behavior continued changing and the ongoing treatments for depression did not help. After recognizing that he had fallen for a scam, she followed up with the bank and confirmed their savings were gone. She noted the increasingly odd collections of sticks, feathers and broken glass, and his poor hygiene. She was upset by Eva’s angry rebellion. Jane tried repeatedly to find other medical explanations, but the doctors saw no obvious issues in brain scans or neurological exams, and were unfamiliar with FTD. Finally, she attended a neuropsychological exam and shared with the doctor the history of Mr. P’s symptoms. She reflected on the past: Five years ago Mr. P was a manager of a store, and things had progressed to where he no longer took part in any goal-oriented activity and instead collected animal parts. Together these pieces prompted referral to a neurologist who recognized and diagnosed FTD, but who provided Jane with no education or information about the disease.

What particular evaluations helped to make the diagnosis of FTD?

Over the years, Mr. P had a variety of mental status evaluations and brain scans to look for stroke or tumor. These were all within normal limits. While the neuropsychological testing was within normal range in most areas, it detected a slightly lower-than-expected performance in attention and some executive function tasks. This finding, together with the history of Mr. P’s symptoms that Jane provided, prompted referral to a neurologist with more experience reading brain scans. He was able to detect subtle changes or atrophy in frontotemporal regions that indicated FTD.

How did the delay in getting a diagnosis affect Mr. P and his family?

Mr. P’s relationships with Jane and Eva were negatively affected by his unexplained loss of interest in them. Eva thought her mother was weak for “standing by her man” as his behavior changed. The progressive nature of the undiagnosed and incorrectly treated symptoms over time contributed to Jane experiencing depression and anxiety. His declining judgment compromised the family’s finances, which Jane could have kept safe had she known what to expect with FTD. There was no opportunity to plan for care needs outside of the home since Jane was not aware of how FTD’s progressive nature might present itself over time. While neighbors and friends came to understand that Mr. P was ill, the family never had support from anyone who was familiar with FTD and could have helped them understand and prepare for his changes.