A new smartphone app could diagnose people with a rare early-onset dementia suffered by Bruce Willis and Wendy Williams, years before symptoms appeared.
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 less than one in 30 cases of dementia and is mainly suffered by adults between the ages of 45 and 65.
It involves characteristic traits including behavioral and personality changes that can be easily confused with a psychiatric illness.
The app accurately distinguished between people with FTD and those without it 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 about the same as standard tests.
It was also able to identify any memory loss with 82 percent accuracy, about the same as standard diagnostic tests.
FTD is not well understood, and researchers hope the app they developed will pave the way for more studies on the disease and the 50,000 to 60,000 people in the United States who suffer from it.
The app is not expected to be available to the public, but to dementia research teams.
Frontotemporal dementia (FTD) presents differently from other types of dementia, typically starting with problems with memory and cognition.
People with FTD first show emotional and behavioral problems, such as stealing, swearing, inappropriate comments in public, impulsivity, and repetitive behaviors.
It can also manifest itself in the form of aphasia, which affects language, speaking, writing and comprehension skills. Both Bruce Willis and Wendy Williams are said to have been diagnosed with aphasia.
The family of actor Bruce Willis announced last year that he was diagnosed with frontotemporal dementia (FTD), less than a 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 took tests of executive function, such as planning and prioritizing, filtering out distractions, and impulse control.
Participants played through an app co-developed by software company Datacubed Health.
The matches were played over 11 days in three separate sessions, each lasting around half an hour.
A specific test was performed every six months to counteract any improvements that might arise with practice.
The average age of the players was 54 years old. Of those with data available, 59 percent had no symptoms, 20 percent were in the early stages of the disease and 21 percent had symptoms.
About 45 percent of participants who underwent genetic testing carried a genetic variant linked to the disease.
Users played games that collected information about their condition for researchers to read.
Some of the games included Ducks on a Pond, in which people were asked to identify which direction the central duck in a group of ducks is facing. Another game, Humi’s Bistro, asked users to learn food orders from various restaurant tables and deliver them to the correct tables.
Poor gaming performance on the app told researchers the extent of the shrinkage their brains have undergone. 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 problems making the correct sounds when saying a word, using words incorrectly, or mixing up the order of words in a sentence.
Wendy Williams, a 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 their struggles with alcohol and abuse, as well as her “cognitive issues.”
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: “Over time, the app can be used to monitor treatment effects, replacing many or most in-person visits to clinical trial 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.
Cognitive tests, such as the Montreal Cognitive Assessment (MoCA), were used as a benchmark for researchers to screen people for cognitive problems. They also used the finger tapping subscale of the Movement Disorders Society Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) to assess motor skills and dexterity.
Most patients with FTD and their families are diagnosed relatively late in their illness. The average age at which symptoms appear is 54 years, but many people with the disease may not be diagnosed until their mid-60s.
Dr Adam Boxer, lead author and neurologist at the University of California, San Francisco, said: “Most patients with FTD are diagnosed relatively late in the disease, because they are young and their symptoms are confused with psychiatric disorders.
“We’ve heard from families who often suspect their loved one has FTD long before the doctor agrees that that is the diagnosis.”
There are no treatments or cure for FTD, although doctors often manage 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.
The disease itself is not fatal, but it can cause other problems that are fatal. Over time, people experience worsening cognitive and behavioral symptoms as the brain deteriorates.
As motor skills decline, a person with FTD becomes much more susceptible to life-threatening falls and other accidents, as well as complications such as 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 Open JAMA Network.
