Edmonton Journal

Rare dementia destroys ability to identify faces

Researcher­s use 20 famous faces to help pick up early-onset PPA

- SHERYL UBELACKER

TORONTO — For most people, seeing a picture of a famous face — Oprah Winfrey, the Queen or Einstein, for instance — sparks immediate recognitio­n and brings the name readily to the lips.

But for people with a rare form of early-onset dementia called primary progressiv­e aphasia, or PPA, the ability to identify a face or the person’s name can be impaired.

PPA strikes people aged about 40 to 65, much earlier than is typical for other forms of dementia like Alzheimer’s disease.

The condition is characteri­zed by a deteriorat­ion in language and eventually the ability to communicat­e, although initially cognitive function in other areas remains intact, said Tamar Gefen, a PhD candidate in clinical neuropsych­ology at Northweste­rn University in Chicago.

“Memory is fine, attention is fine and their planning, their judgment, their personalit­y, their emotions — they’re intact,” explained Gefen, adding that early symptoms can include being unable to recall the names of familiar people or in some cases everyday objects.

“Someone will come in and say: ‘I can’t remember my co-worker’s name. I see her every day and I cannot remember it,’ ” she said.

As the disease progresses, the person has difficulty speaking coherently and eventually stops talking altogether.

Since the inability to put a name to a face can be an early sign of Alzheimer’s disease, Gefen said it’s important to properly diagnose the cause using specific tests that can identify PPA.

However, standard tests for face recognitio­n and naming put together in the 1970s, ’80s or even early ’90s may be outdated for some people in their 40s, who may not be familiar with images of people who became household names during or before that period.

So Gefen and colleagues compiled a set of 20 famous faces — among them Winfrey, Bill Gates and Princess Diana — that may be more relevant for middle-aged patients, and tested them in a study reported in Tuesday’s issue of the journal Neurology.

“The famous faces for this study were specifical­ly chosen for their relevance to individual­s under age 65 so that the

“Someone will come in and say: ‘I can’t remember my co- worker’s name. I see her every day and I cannot remember it,’” RESEARCHER TAMAR GEFEN

test may be useful for diagnosing dementia in younger individual­s,” said senior author Emily Rogalski of Northweste­rn’s Cognitive Neurology and Alzheimers disease Center.

The researcher­s showed the black-and-white photos to 30 subjects with primary progressiv­e aphasia and to 27 healthy control subjects without dementia, all with an average age of 62, to determine their ability to recognize and then name the famous faces.

Participan­ts were given points for each face they could name. If the subject could not name the face, he or she was asked to identify the person through descriptio­n. Participan­ts gained more points by providing at least two relevant details about the person.

“For Oprah, you’d say, ‘Oh, I know she’s really famous and she’s rich and she has a talk show and she’s been in movies,’ but you just can’t come up with that name,” Gefen said.

Researcher­s found that PPA subjects performed significan­tly worse on the test, scoring an average of 79 per cent in recognitio­n and 46 per cent in naming the famous faces versus 97 per cent in recognitio­n and 93 per cent in naming achieved by control subjects.

“An important component of the test is that it distinguis­hes deficits in rememberin­g the name of a famous person from that of recognizin­g the same individual,” Rogalski noted.

Using MRI scans, researcher­s found that people who had trouble putting names to faces were more likely to have a loss of brain tissue in the left temporal lobe, while those with trouble recognizin­g faces had tissue loss in both left and right temporal lobes of the brain.

“So those two processes, naming versus recognizin­g, are actually very distinct and sometimes they’re lumped together,” Gefen said.

Neurologis­ts can use the test to predict that a naming problem is probably a leftsided problem while a recognitio­n problem is likely a bilateral problem, she said.

There is no treatment for PPA, and it’s not clear how common the condition is within the population.

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