Wednesday, October 30, 2013

A Different Kind of Clinical Study

In my professional life, I write about drug development. In my private life, I write about prosopagnosia. Those two worlds intersected last week when my husband, Chuck, completed a six-month clinical study about face blindness at the University of British Columbia/Vancouver General Hospital Eye Care Centre.

Although I'm accustomed to reporting on clinical trials of drug candidates, whether successes or failures, the study in which Chuck participated was very different, consisting of computer-based "face training" modules that he watched for 30 minutes, three times a week, over a three-month period. During a control period, he was exposed to a variety of faces, both new and repetitive, while watching 30-minute segments of a British television detective drama three times a week. At his post-testing, the researchers observed slight improvements in his ability to recognize faces -- a hopeful sign, though not an indication that he'll suddenly be able to enter a crowded party and pick out the faces of close friends.

Although the face training study did not involve medications -- not during this round, at least -- I was struck by the parallels between its design and the many drug trials conducted each year across a variety of indications. Talking with researchers throughout the trial period and during post-testing also hammered home the incredible wealth of knowledge that patients and their families bring to a study based on months or years of living with a given condition.

I shared those thoughts in a blog post for BioWorld Today. I invite you to read that post, entitled Patient Experience Still Undervalued in Regulating Drugs.

Tuesday, April 2, 2013

Are Some Children with Prosopagnosia Misdiagnosed as Autistic?

Is autism overdiagnosed? The question continues to generate debate, especially in light of a report from the National Center for Health Statistics indicating the prevalence of parent-reported autism spectrum disorder (ASD) among school-age children was 2.00% in 2011–2012, a 1.16% increase since 2007. The magnitude of the increase was greatest for boys and for adolescents aged 14 to 17, and researchers suggested that much of the increase in prevalence over the five-year period resulted from diagnoses of previously unrecognized ASD.

In the 20 years since I first learned of prosopagnosia, I've often wondered if some children diagnosed with ASD disorders -- particularly autism, Asperger syndrome and pervasive developmental disorder not otherwise specified -- have prosopagnosia, instead. Many characteristics associated with ASD also can occur in prosopagnosia, such as inappropriate social behavior and problems with non-verbal communication. For example, many individuals with Asperger syndrome and other ASD disorders avoid eye contact -- also a characteristic of those with prosopagnosia.

Consider this scenario: A child with prosopagnosia, or face blindness, is born into a family with "normal" face recognition. The child grows assuming that others cannot recognize faces, just as he or she cannot. Meanwhile, other family members assume the child "sees" faces just as they do. No one thinks to question the other's visual perception, even if the child sometimes acts slightly "strange," "eccentric" or even "lost" in his or her own environment.

While the child is young and primarily confined to the family circle, he or she learns to differentiate among family members by voices, gender, age and other mannerisms, assuming everyone else is navigating the world the same way. Family members may develop a protective instinct for the child, aware that he or she sometimes seems distracted, evasive around strangers or inclined to withdraw from social interaction -- characteristics that can easily apply to ASD.

It's not a stretch to imagine that concerned parents might eventually seek a professional assessment, with ASD the suspected diagnosis. In my view, it's equally likely that some children with prosopagnosia, exhibiting such characteristics, could be misdiagnosed with an ASD disorder, particularly since temporal lobe dysfunctions can be associated with both disorders.

In studying individuals with developmental, or congenital, prosopagnosia, researchers have learned that many were not diagnosed until they left home, for college or a first job, and realized for the first time that nearly everyone else they met could remember their face. As they grew, many of these children had compensated for face blindness by memorizing names, seating charts, sports jersey numbers and other routines that helped them navigate their environment at school and play, assuming everyone else was doing the same.

"People are more in tune with the possibility" that the characteristics of prosopagnosia might be mistaken for symptoms of ASD, said Jason Barton at the University of British Columbia's Human Vision and Eye Movement Lab. In fact, Barton's colleague, Brad Duchaine, whose Social Perception Lab at Dartmouth College works closely with developmental prosopagnosia, believes most of these individuals develop normal social interactions, even if their behaviors may sometimes appear a bit odd.

"There's no question that some people with developmental prosopagnosia also have social developmental issues," Barton said. "But it's not a given that if you have a problem recognizing faces you will necessarily fall into the autism spectrum. On the other hand, if a child has a diagnosis such as autism or Asperger syndrome, that person won't automatically have a problem recognizing faces."

But nearly two-thirds of those diagnosed with ASD do have problems recognizing faces, Barton acknowledged, even if they aren't completely face blind, like my husband, Chuck. Last month, researchers at Georgetown University Medical Center reported in the journal NeuroImage: Clinical that neurons in the fusiform part of the brain, which processes facial recognition, behaved abnormally in individuals with autism, causing difficulties with facial recognition. The scientists made the discovery using a form of functional magnetic resonance imaging, or fMRI, that's commonly used in prosopagnosia studies.

"One of the struggles is trying to figure out, if this is a part of the problem for some people with autism, can we retrain the way they see faces?" Barton asked. "And will that have any impact on their autism?"

The initial approach in working with such individuals is the same advice Chuck received as part of his "retraining": Look more closely at people's eyes.

"We might not be able to hang the entire fault for their social problems on the fact that individuals with autism don't look at the eyes," Barton said, "but if they do look at the eyes, will they pick up more social cues and, therefore, improve their social functioning?"

These are questions parents, and medical professionals, should ask if a school-age child is suspected of ASD -- particularly if the symptoms are mild. As Maximilian Riesenhuber, associate professor of neuroscience at Georgetown, said about the research on autism and facial recognition, huge variation exists, and some individuals with autism have no problem recognizing faces.

“But for those that do have this challenge, it can have substantial ramifications," he added. "Some researchers believe deficits in face processing are at the root of social dysfunction in autism.”

Thursday, March 7, 2013

Apperceptive vs. Associative: Will the Distinctions Make a Difference?

Apperceptive with elements of associative prosopagnosia. In plain English, that description essentially means Chuck cannot distinguish between faces he knows and those he doesn't, although he can recognize a face as such. Typically, this processing impairment results from damage to the right occipital temporal lobe -- the opposite side from Chuck's temporal lobectomy. In general, the associative aspect refers to his difficulty determining whether photos of two faces capture the same person or two different people, and then identifying someone from his or her photo. This memory "linking" impairment also is commonly associated with the brain's right anterior temporal region.

However, Chuck is left-handed -- or, more accurately, "mix-handed," which might have affected the location of face recognition functions in his brain. He's not exactly ambidextrous but he does perform a number of functions with his right hand. Did he develop that ability innately? Did his parents try to "re-train" him at a young age when he was performing tasks with his left hand? Did congenital damage to the right side of his brain prompt certain neurological functions to "migrate" to the left side? Any of these scenarios might have played a role in the development of his brain and the location of facial recognition centers. We'll never know the answer and, ultimately, it doesn't matter. The more important question is whether Chuck -- or potentially other prosopagnosia patients -- might benefit from the Face Training Program at the Human Vision and Eye Movement Lab in the University of British Columbia's Department of Ophthalmology and Visual Sciences.

This week's baseline visit to Jason Barton's lab offered some important clues. Over the course of four days, Chuck took a battery of tests.

Using the Cambridge Face Perception Test, he compared a "target" face to a range of similar faces, with one minute to rank the choices from farthest to closest to the target. 

The O'Toole Dynamic Face Recognition Test assessed his ability to identify short video clips of faces.

The "George" Component Test examined whether one of six facial features -- chin, forehead, eye size and distribution and mouth size and distribution -- was dissimilar in one face on a screen compared to two others.

The Cambridge Memory Test of Faces measured Chuck's ability to memorize faces shown in three orientations and then identify which of the faces he'd seen previously.

And the Warrington Memory Test of Faces and Words evaluated his ability to recognize previously seen faces and words.

Chuck also underwent a two-hour functional MRI, an electroencephalogram (EEG) and various ancillary studies, including face and voice matching, eye-tracking and word length effect.

Early results suggest Chuck performed below average in every test -- no surprise -- and no better than chance in some of them. In the components test, for instance, his performance was less accurate than random selection. Could the Face Training Program improve his score and, even more important, enhance his ability to identify the faces of family and friends in the real world? Only time will tell.

We'll be on our way home in the morning. The next step of Chuck's journey is just beginning.

Wednesday, March 6, 2013

Expanding the Boundaries of Face Blindness

Because prosopagnosia isn't life-threatening, research into the condition didn't exactly spread like wildfire. After Chuck was diagnosed with face blindness, we asked his medical team what resources were available to help him cope with the condition. For the most part, they could only refer to the textbook definition and wish him well. None of them had seen an actual case. They told us that cases of prosopagnosia largely affected patients who were critically ill with other conditions, such as disabling strokes that had bled into both sides of the brain or tumors that had spread into multiple brain regions. Chuck's doctors said that, for these patients, face blindness was the least of their problems -- hardly a heartening perspective for someone facing a life-changing diagnosis.

As a freelance writer who specialized in health care, I began searching the term "prosopagnosia" occasionally on medical websites, such as the National Institutes of Health. These attempts were pretty primitive; after all, this was in the days before search engines.

Although PubMed featured scholarly publications describing prosopagnosia, for years searches for clinical studies came up empty. Around 1997, I got a hit on the NIH website, but my excitement turned to disappointment when the prosopagnosia page was empty. (To this day, lists no studies for prosopagnosia or face blindness.)

Although the introduction of Google in 1998 made searching easier, it was 2005 before a search directed me to the website I literally ran to get Chuck, and we poured over the website, where he completed a questionnaire about face recognition difficulties. That response led us to Brad Duchaine, a researcher and lecturer at University College London who's now associate professor and head of the Social Perception Lab at Dartmouth College.

At the time, Duchaine and colleagues were focused on face recognition deficits in developmental disorders, but they were collecting names and contact information for individuals who might be interested in future research projects in prosopagnosia. Without hesitation, Chuck added his name to the list.

Several more years passed. In February 2008, we received an email from Duchaine, which read, in part:

"A group of researchers, including myself, have begun a large-scale study of acquired prosopagnosia, and we're interested in recruiting as many subjects as possible. Most of the testing will take place in Vancouver with a group of neurologists and psychologists coordinated by Jason Barton in the Human Vision/Eye Movement Center at the University of British Columbia.

"Our understanding of face processing and its dysfunction has benefited greatly from studies of people who have lost their ability to recognize faces due to brain damage experienced after maturity (acquired prosopagnosia). However, because the condition is relatively rare, few subjects have been studied by labs that specialize in face processing, so much remains to be learned about acquired prosopagnosia.

"Our study involves a large battery of behavioral tests measuring a wide range of abilities (face memory, face perception, face detection, expression recognition, object recognition, low-level visual abilities, etc.) and several scans that provide a detailed view of brain structure and activity in response to visual stimuli. Participants also have the option of enrolling in an experimental trial of perceptual training that we hope will improve face recognition ability."

In May 2010, Chuck's adventures in prosopagnosia research began with a visit to Barton's lab in Vancouver for "a functional MRI study of facial perception in normal subjects, prosopagnosic patients and patients with cerebral infarcts." Findings from that study -- for which I volunteered as a control -- concluded Chuck had a general diagnosis of intermediate prosopagnosia, including "significant problems with recognizing faces, which also extends to other object categories."

Fast forward to this week, when we returned to Barton's lab so Chuck could participate in a face training program. Following the first day of testing, which concluded with a functional MRI, Chuck received a more defined diagnosis of "apperceptive with elements of associative" prosopagnosia. In addition, we heard for the first time in 20 years that facial recognition may -- potentially -- be a skill that can be re-acquired or re-learned, thanks to neuroplasticity. Much work to do, but finally the prospect of "seeing" faces once again.

Sunday, March 3, 2013

And the Oscar Goes to...

Along with 40 million of our closest friends, Chuck and I watched the annual glamfest known as The Oscars. For Chuck, however, the awards show was more than a parade of gowns, speeches and bad jokes. It was also a sharp reminder of the limitations of movies, TV shows and theater for viewers with prosopagnosia.

In the old days before Chuck's face blindness, we could select a movie on a whim, simply because the story sounded compelling. Prosopagnosia has changed that. Trying to follow a complicated plot is hard work, not entertainment, for Chuck. As each character is introduced, he must quickly absorb something distinctive -- purple hair, a lisp, an eye patch, use of a wheelchair or cane -- since he can't recognize faces. When characters are introduced one at a time or in a small, diverse group -- like the eccentric family in Little Miss Sunshine -- he can differentiate them more easily. That's important, because as each new scene unfolds and the actors change costumes and sets, he must piece together their identities all over again. The faster he can do that, the more he can absorb of the story.

The challenge is greater with some movies than others. For instance, Chuck can keep up with James Bond, though he may be fuzzy on the minor characters. It's easier to follow a plot if he's read the book, like Marley & Me, which also featured a small cast -- and the lead character was a dog.  He has little difficulty identifying Meryl Streep in almost any movie, despite the fact -- or maybe because -- she lights up every scene. And biographies are favorites. Chuck thoroughly enjoyed Ray and The King's Speech -- of course, the films also featured a blind man and a monarch with a stutter, respectively. Different is better when it comes to picking a movie that someone with prosopagnosia can enjoy.

The opposite also is true. If a film begins with a rapid-fire montage -- think Saving Private Ryan or Gladiator -- it's so difficult to pick out the lead characters and learn something distinctive about them that it's an uphill battle to find them in ensuing scenes. Chuck watched both of these movies, but plot nuances were lost on him as he focused most of his energy on searching for the important characters in each scene.

While most people read reviews or talk with friends to decide whether or not to attend a given movie, we use a fairly unusual decision tree. War epic? Usually not, because a bunch of men wearing the same uniforms makes for a very difficult plot for Chuck. Ensemble cast? Not likely, unless they're strikingly different, such as the safe-cracking team of The Italian Job. Girl meets boy? Depends on how many girls and boys. Plenty of men protest about watching chick flicks -- even if they're classics like Pride and Prejudice or Sense and Sensibility -- but not for the same reason as Chuck. Musicals? Even if the plot is dense, songs can compensate. And let's face it, unless they're performing in A Chorus Line or TV's Glee, most singers and dancers are anonymous by design so Chuck doesn't have to look for them in each scene.

Of course, there are exceptions to these movie rules, and 2012 was full of them. Which brings me to the year's award-winning films. The first rule we broke was to watch Argo, which Chuck enjoyed but struggled to follow. Not surprising, considering the movie starts with hundreds of unnamed extras storming the U.S. embassy in Tehran. The upside: We lived through the Tehran crisis, which helped him to track the plot, even if some of the characters blended together.

Skyfall? Bond. Done.

Despite the spectacle, Chuck enjoyed Les Miserables. A musical, of course, and hard to top for the artistry of the sung performances. It helped that we'd seen the stage version and Chuck knew the general story.

Both Lincoln and Zero Dark Thirty were challenging. Although the main characters jumped off the screen, others in the large ensemble casts quickly melted together. During scenes without the leads, Chuck had to guess who was talking and how that dialogue factored into the plot. Fortunately, the context of both stories -- one from the history books and the other from recent history -- and the power of both films helped him to keep up.

We haven't yet seen Life of Pi, but a boy and a tiger? Sounds like a slam dunk. It's on our list.

But for Chuck, the most watchable movie of the awards season was Silver Linings Playbook, which featured four distinct and memorable characters. Even some of the cameo roles -- Chris Tucker as Pat's sad-luck friend and Anupam Kher as his court-ordered psychiatrist -- were sufficiently different that Chuck could spot them quickly as the story unfolded.

Filmmakers don't always load their movies with idiosyncratic characters, nor should they. But for someone with prosopagnosia, watching a small film with quirky personalities is the closest it gets to true entertainment. That said, movies do have one advantage over real life: they never change. The characters don't age, develop wrinkles and sags, gain or lose weight or cut and color their hair. The films Chuck watched before his surgery in 1993, when he could still recognize and form memories of a face, live forever. He can enjoy them as much today as he did 20 years ago.

Saturday, February 16, 2013

Traveling in a Vacuum

Although the inability to recognize faces is the most disabling aspect of prosopagnosia, the condition is multi-dimensional -- literally. Today, Chuck and I traveled to Austin, Texas, to spend a long weekend with our daughter, who is celebrating her 30th birthday. The trip brought to mind some of the spatial challenges he faces when navigating the world.

Chuck's sense of direction was deficient before he acquired prosopagnosia. From the time we were newlyweds 36 years ago, he relied on maps to track even familiar routes and had difficulty committing new directions to memory. However, Chuck's navigational skills deteriorated noticeably in the years following his brain surgery. Traveling is now disorienting and anxiety-inducing. He has difficulty processing the rush of information in a large airport or train station. And, similar to his inability to characterize individual faces, Chuck has difficulty spotting familiar landmarks that could point him toward his destination.

Thus, a trip through Hartsfield-Jackson Atlanta International Airport -- our local airport and also the world's busiest -- requires extra time and care to ensure we avoid a separation. Certainly, most people do this intuitively. Couples, families and business associates don't travel through airports and deliberately leave someone behind. But if I were to step away after passing through the security checkpoint or step off the underground train without ensuring that Chuck is one step behind, he would not be able to find me. Of course, he's an intelligent adult, so he could find an airport flight monitor and eventually locate the departure gate. But the process would be methodical, not a sprint to the gate.

We sometimes joke that I could have some fun with this if I wanted to hide in plain sight. And Chuck has a good sense of humor, able to laugh at himself when he makes a wrong turn on an airport concourse coming out of the restroom. But sometimes his spatial disorientation is no laughing matter. He's occasionally made a wrong turn walking the bike paths near our home and found himself in a neighborhood that looked totally unfamiliar. (Thank goodness for cell phones.) And there is no "divide and conquer" in our travel plans, where one goes for coffee, the other for a snack and we meet at the gate. From the time Chuck was diagnosed with prosopagnosia, whether on our own or with our children, family or friends, we have traveled in a pack.

In fact, our children were acutely aware, from an early age, that it was important to keep their father in sight in a crowd -- as much for his sake as for theirs. Even today, when we're together as a family, I sometimes notice them glance around instinctively to make sure they don't lose sight of their dad.

The degree of difficulty is higher when we travel through unfamiliar airports. And at Grand Central, when we visit New York? Although the station is an adventure for most visitors and a challenge for some, the frenetic activity and dizzying information displays are simply overwhelming for Chuck.

In short, plane and train trips are routine for most travelers, but not for those with prosopagnosia. Maybe some of the signs people hold up for arriving airport passengers are to welcome familiar faces, not to guide strangers to their limo.

Thursday, February 7, 2013

When Acquaintances Become Strangers

Today, we had to drop out of a group of couples from our church that meets every month because it's too stressful for Chuck to participate in these gatherings, even though it's a friendly crowd. Sometimes I think a "mixed" group of strangers and friends is more difficult for Chuck than a homogeneous bunch of strangers -- "Awesome! I don't know anyone!" -- or friends -- "OK, everyone here knows me so I'll have to mind my manners."

Over the past few years, this stepping back, or dropping out, socially has become more common. It's been nearly 20 years since Chuck's surgery, representing two decades of meeting people for whom he can't form a "picture" of each face in his mind. Although a few close friends greet him EVERY time by introducing themselves by full name, most people think, "Well, even though he can't recognize my face, he should know me by now." For the most part, that's completely wrong. He is blind.

In fact, Chuck has lost much of the context for the closest friends and family members he knew before prosopagnosia. They've aged, or moved, or died. For other acquaintances, he confronts a jumble of "Where did I meet them?" "How do I know them?" "What do we have in common?" when he encounters them. I sometimes see these questions play through his head as he struggles to use the available cues -- voice, gender, size, sometimes age -- to try to determine someone's identity. Doing this in a crowd, and then trying to remember what everyone is wearing so he can tell them apart after the initial greeting, is exhausting. And stressful, sometimes prompting a seizure. When that happens, the gathering is over for us. So, sometimes, it's easier not to attend in the first place.

Friday, January 25, 2013

Did I Do My Homework?

The decision to have the epilepsy surgery that resulted in prosopagnosia was not one that Chuck undertook lightly. He had been taking medication since he was 15 years old -- a spectrum of mono and combo therapies that included carbamazepine, phenobarbitalphenytoinvalproic acid and others -- but had never been seizure-free. At the time he was considering surgery, he was on high doses of two medications, which caused dizziness, hand tremors and occasional insomnia. He needed lab tests every three months to monitor his liver function.

When we arrived in Atlanta in 1979, Chuck had found a neurologist recommended for his expertise with epilepsy. The physician was young and energetic and served on the boards of several professional medical organizations. After experimenting over 10 years with multiple combinations of anti-epileptic drugs, however, the neurologist threw up his hands and told Chuck he had nothing else to offer. That's when Chuck decided to find another neurologist, who was the first to mention the surgical option.

In March 1993, Chuck was referred for testing to the Medical College of Georgia, now Georgia Regents University. That May, he was hospitalized for 10 days of EEG and video monitoring for seizure activity, using scalp and sphenoidal electrodes. The procedure, in which he was deliberately sleep- and exercise-deprived in an effort to trigger "typical" seizures, suggested his began in the left temporal lobe and migrated to the right anterior temporal area. When the prosopagnosia was discovered, that would become a significant finding.

Chuck also underwent baseline neuropsychological testing to assess memory and learning, general functioning, language, visual-spatial functioning, attention and concentration, cognitive flexibility and planning. Because he is "mix-handed," writing with his left hand but performing many functions with his right, he also underwent intracarotid amobarbital assessment, better known as the Wada test, which confirmed he was left cerebral language dominant but with a significant left-to-right crossflow.

In July, Chuck returned for a week of monitoring with left and right occipital temporal depth electrodes to try to pinpoint the focus of his seizures. The monitoring confirmed seizure activity starting in the left temporal lobe and sometimes spreading to the right side of the brain.

Still, the list of potential complications associated with the procedure -- in Chuck's case, a left posterior temporal-occipital multiple subpial transection and left posterior inferior temporal-occipital resection with electrocorticography and stimulation mapping -- was long and daunting, up to and including blindness, coma, stroke, paralysis or death.

In fact, Chuck's neurosurgeon counseled Chuck, then 40, that epilepsy surgery posed greater risks for him than for the average candidate because he was "a complicated patient" and more "highly functioning" than most surgical candidates, with a white-collar job in inventory management, a stable family, a home and a large circle of friends.

But living with seizures that occurred as often as two or three a week and without warning -- at home, at work, at church, at meals, awake, asleep -- was increasingly difficult and even risky for him. One of the scariest incidents occurred when we were picking up a few items at the local hardware store on a Saturday afternoon. While our children followed me on a quest to find the right paint color for an indoor project, Chuck went to the garden section to retrieve some weed killer. I found him several minutes later in the altered state of consciousness typical following a seizure. Some minutes later, Chuck complained his mouth tasted bitter and he needed a drink of water. Instinctively, I looked at the bottle of weed killer in his hands and inspected the cap. Somehow, he had managed to open it following the seizure and swallow several ounces. Fortunately, the weed poison wasn't fatal to humans -- only nauseating -- but a concentrated form of insect repellent could have hospitalized or killed him.

Chuck and I reflected on incidents like these frequently during the months he was evaluated for surgery. We considered ourselves blessed to have two beautiful children, good jobs and a home in metropolitan Atlanta -- the middle class America dream. Were we playing with fire? Asking the right questions? Seeking a perfect world that did not exist? Could the surgery somehow leave Chuck more incapacitated than the seizures?

In early December 1993, Chuck agreed to have his name placed on the surgery schedule. "If I don't do it now, I'll be calling to get back on the schedule the next time I have a seizure," he said at the time.

Saturday, December 29, 2012

A Puzzling Diagnosis

Imagine watching your child kicking a soccer ball with friends or playing pick-up basketball but not knowing which child is yours. Imagine walking into a party in your neighborhood and not seeing a familiar face. Imagine attending a family wedding but not recognizing a soul. Since 1993, this has been Chuck's new normal. In his world, every face belongs to a stranger, even if the person he passes silently on the sidewalk or church pew is a co-worker or close friend.

Although Chuck looked exactly the same after epilepsy surgery -- once his hair grew back, at least -- no one looked the same to him. He was surrounded by the same familiar family members, co-workers, friends and neighbors but could "recognize" the people in his life only by their voices, gait, scent, mannerisms or distinctive hairstyle.

That's how Chuck discovered prosopagnosia in the first place. When he was discharged from the Medical College of Georgia 10 days after surgery, his visual perception seemed altered but the changes were subtle. Colors were the same, but the hues were different. If our dark blue Chevy Lumina was parked next to a similar model of the same color, Chuck sometimes went to the wrong car. He had trouble finding a certain can of soup in the pantry or locating a particular tool in the garage. For the first month or so following surgery, we thought these idiosyncrasies were related to the trauma to his brain and the gradual healing process. After all, Chuck had temporarily lost the ability to speak when his brain swelled in the days following surgery, and he was still undergoing speech therapy to help overcome difficulties with word finding and retrieval.

But when Chuck returned to work six weeks after surgery, co-workers also looked different. Since he couldn't explain the changes and seemed to reengage with colleagues during that first day back on the job, he chalked up the experience to sensory overload. But day two at the office was a repeat of the first. His co-workers were strangers once again.

Now, we knew something was wrong, though we didn't have a name or explanation for the problem. Imagine passing coworkers in the hall, sitting across from them in meetings or standing in line with them in the cafeteria and not recognizing a single face. And the problem extended beyond the workplace. Chuck couldn't pick out our two children among a group of classmates or teammates. If he lost sight of me in a crowd -- at church or in a grocery store, for instance -- he had to focus on remembering what I was wearing while searching for me.

When Chuck returned to MCG for a 10-week post-op assessment and testing, he mentioned the strange difficulty in distinguishing faces. Without a word, his neurosurgeon began pulling films from Chuck's chart while keying information into his computer. He examined the MRI film and notes from Chuck's pre-op testing. A few minutes later, he told us he strongly suspected that Chuck had acquired prosopagnosia. We now had a diagnosis, but we had no idea what the term meant or where it would lead us.

Friday, March 30, 2012

A Little Background Is in Order...

So what is prosopagnosia, you may ask? That's a legitimate question, because most physicians have never seen a case of the condition, if they've even heard of it.

The term is literally from the Greek -- "proson" for face, and "agnosia," for not knowing. When my husband, Chuck, developed the condition as a complication of epilepsy surgery 19 years ago, his neurologist and neurosurgeon accurately interpreted his symptoms but could offer little additional help. In 300 epilepsy surgeries performed at the center, they had never seen a case of prosopagnosia, and studies on the condition were few and far between. Members of the team conducted follow-up neuropsychological testing, confirming Chuck's face blindness, and handed us a photocopy of the first chapter from The Man Who Mistook His Wife for a Hat and Other Clinical Tales, the book of classic case studies from renowned neurologist Oliver Sacks. Awesome, I remember thinking. Will Chuck now see me as a hat?

Well, it turns out Chuck still viewed my face as a face -- albeit a confusing collection of features. That's what people with prosopagnosia see: eyes, nose, cheekbones, brow, mouth, hair, and ears, but with no way to assemble those features into the single, distinctive image of a face they can recognize and store in memory.

That's one of the major losses Chuck has suffered since the day of his surgery. Since he can't recognize faces, he's been unable to snap a mental photograph of the faces of people he's met since December 28, 1993 -- friends, colleagues, neighbors. He has a memory of my face and those of siblings and close friends as adults, but our children were just eight and ten when Chuck developed prosopagnosia so he cannot "see" their faces as adults. That's caused practical challenges, as he sometimes has trouble discriminating between me and our daughter, Kathleen, since we're both tall and slender. Fortunately, her hair is longer and darker than mine, but most differences -- except for age -- end there.

And age is not a terrific differentiator for people with prosopagnosia. Neither is skin tone. Chuck has trouble distinguishing between a light-skinned African American and a dark-skinned Caucasian. He can identify a child from a short adult, but beyond that he struggles to categorize a person's age. He tends to focus on other attributes: voice, gait, a distinctive perfume, and the like. Unlike the unique image of a person's face, those characteristics aren't terribly reliable, which has caused more than one uncomfortable situation. More on that to come.