New NIH-funded smartphone app may be able to diagnose Bruce Willis and Wendy Williams’ aggressive dementia years in advance, study shows

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 have developed an app that uses memory and sorting games to detect signs of dementia with accuracy comparable to tests used by doctors.

The condition, frontotemporal dementia (FTD), accounts for fewer than one in thirty cases of dementia and mainly affects adults aged 45 to 65.

These are characteristic features, including behavioral and personality changes, that can easily be mistaken for mental disorders.

The app accurately distinguished between people with FTD and people without FTD in 93 percent of the cases.

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

FTD is not yet well understood and researchers hope that the app they have developed will pave the way for further research into the disease and the 50-60,000 people who suffer from it in the US.

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

Frontotemporal dementia (FTD) manifests differently than other forms of dementia, which normally start with memory and cognition problems.

People with FTD first exhibit emotional and behavioral problems, such as stealing, swearing, inappropriate comments in public, impulsiveness, and repetitive behavior.

It can also manifest as aphasia, which affects language skills, speaking, writing and comprehension. Both Bruce Willis and Wendy Williams are said to have been diagnosed with aphasia.

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

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

The games were played over eleven days in three separate sessions of approximately half an hour each.

One specific test was performed every six months to counteract any improvements that might occur through practice.

Of those with available data, 59 percent had no symptoms, 20 percent were in the early stages of the disease and 21 percent were symptomatic.

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

Among other things, Ducks in a Pond was played, where people were asked to indicate which direction the middle duck in a group of ducks is facing. Another game, Humi’s Bistro, asked users to learn the food orders from different restaurant tables and deliver them to the correct one.

Poor performance in games on the app would indicate the degree of brain shrinkage. In FTD, the part of the brain responsible for executive functioning, such as problem solving, shrinks as the disease progresses.

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

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

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

The app accurately distinguished between participants without symptoms and participants with dementia, with an accuracy rate of 93 percent. It also accurately distinguished between people with no symptoms and those in the early stages of the disease.

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

Most patients with FTD are diagnosed relatively late in their disease. The average age of onset of symptoms is 54 years, but many with the disease are not diagnosed until their 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 doctor agrees that it is the diagnosis.”

There are no treatments and no cure for FTD, although doctors will typically treat symptoms such as agitation with a psychiatric drug called Trazodone.

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

Although the disease itself is not fatal, it can cause other problems. 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 fatal falls and other accidents, as well as complications such as pneumonia.

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

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