New NIH-funded smartphone app may be able to diagnose aggressive dementia suffered by Bruce Willis and Wendy Williams years in advance, trial indicates

A new smartphone app could diagnose people with a rare early-onset dementia suffered by Bruce Willis and Wendy Williams, years before symptoms manifest.

Researchers at the University of California developed an app that uses memory and sorting games to detect signs of dementia with similar accuracy to tests used by doctors.

The condition, frontotemporal dementia (FTD), accounts for fewer than one in 30 dementia cases and is primarily suffered by adults ages 45 to 65.

It involves characteristic features including behavioral and personality changes that can be easily mistaken for psychiatic illness.

The app accurately distinguished between people with FTD and those without 93 percent of the time,

The smartphone app was able to differentiate between people with FTD and those without it with 93 percent accuracy, which is roughly the same proficiency as standard tests.

It was also able to identify any memory loss with 82 percent accuracy, roughly the same as standard diagnostic tests. 

FTD is not well understood, and researchers hope that the app they have developed will lead the way for further studies into the disease and the 50-60,000 people suffering from it in the US.

The app is not expected to be made available to the public, but rather to dementia research teams.  

Frontotemporal dementia (FTD) presents differently from other types of dementia, which normally starts with memory and cognition problems.

People with FTD first show emotional and behavioral problems, such as stealing, swearing, inappropriate comments in public, impulsivity, and repetitive behaviors.

It can also show itself in the form of aphasia, which affects language skills, speaking, writing, and comprehension. Both Bruce Willis and Wendy Williams are said to have been diagnosed with aphasia.

Actor Bruce Willis' family announced last year that he was diagnosed with frontotemporal dementia (FTD) - less than one year after he retired from acting due to his battle with aphasia, a speech and language disorder

In the study, 360 people who were genetically predisposed to the disease but had not yet developed symptoms performed tests of executive function, such as planning and prioritizing, filtering distractions, and controlling impulses.

The participants, aged 54 on average, played the games via an app co-developed by software company Datacubed Health.

The games were played over 11 days in three separate sessions, each lasting around half an hour.

One specific test was conducted every six months to counter any improvement that may come with practice.

Out of those with available data, 59 percent were without symptoms, 20 percent were in the early stages of the disease, and 21 percent were symptomatic.

About 45 percent of the participants who underwent genetic testing carried a gene variant linked to the disease.

Games played included Ducks in a Pond, which asked people to identify which direction the center duck in a group of ducks is facing. Another game, Humi’s Bistro, asked users to learn the food orders of several restaurant tables and deliver them to the right ones.

Poor performance with games on the app was said to signal the degree of brain shrinkage. In FTD, the part of the brain responsible for executive functioning, like problem-solving, shrinks as the disease progresses.

The app also recorded speech changes and body movements. Patients with FTD often have slower speech and trouble making the right sounds when saying a word, using words incorrectly, or mixing up the order of words in a sentence.

Wendy Williams, former talk show host, has also been diagnosed with FTD. She announced the diagnosis in February. Her diagnosis comes as she and her family have opened up about her struggles with alcohol and abuse, as well as 'cognitive issues'

Some people with FTD also have irregular body movements, including tremors, muscle spasms, rigidity, and poor balance.

Dr Adam Staffaroni, a neuroscientist and co-author of the study, said: ‘Eventually, the app may be used to monitor treatment effects, replacing many or most in-person visits to clinical trials’ sites.’

The app accurately distinguished between participants without symptoms and those with dementia, with an accuracy rate of 93 percent. It also accurately differentiated between people without symptoms and those in the early stages of the disease.

Bruce Willis' double diagnosis: How aphasia can lead to dementia 

Hollywood legend Bruce Willis has been diagnosed with frontotemporal dementia (FTD) less than a year after he was diagnosed with aphasia in spring 2022, after suffering a loss of ability to understand or express speech. 

Cognitive tests, such as the Montreal Cognitive Assessment (MoCA), were used as a reference point for researchers to screen people for cognitive issues. They also used the the Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS) Finger Tapping Subscale to assess motor skills and dexterity.

Most patients with FTD receive the diagnosis relatively late in their disease. The average age at which symptoms appear is 54, but many with the disease may not be diagnosed until the early- to mid-60s.

Dr Adam Boxer, lead author and neurologist at UC San Francisco, said: ‘Most FTD patients are diagnosed relatively late in the disease, because they are young, and their symptoms are mistaken for psychiatric disorders.

‘We’ve heard from families that they often suspect their loved one has FTD long before a physician agrees that is the diagnosis.’

There are no treatments and no cure for FTD, though doctors will typically manage symptoms like agitation with a psychiatric drug called Trazodone.

Because FTD first manifests as behavior and mood problems, many people with the condition are diagnosed early on with mood problems, such as depression and bipolar disorder.

The disease itself if not fatal, but it can cause other issues that are. Over time, individuals experience worsening behavioral and cognitive symptoms as the brain deteriorates.

As motor skills decline, a person with FTD becomes much more susceptible to potentially deadly falls and other accidents, as well as complications like pneumonia.

People may also develop difficulty swallowing, which can lead to dehydration, malnutrition, or pneumonia. They may also be more likely to develop other medical conditions, such as heart disease or diabetes, due to changes in daily habits as well as poor blood pressure regulation, and loss of control over heart rate.

The researchers, who received funding from the National Institutes of Health, published their findings in the journal JAMA Network Open.