Charles Bonnet Syndrome Sufferers See Visions

Sight Seeing: A symptom of macular degeneration, Charles Bonnet Syndrome is a little-known condition in which the brain ‘fills in’ details for those whose vision is failing
People with CBS see “phantom visions.” Here are artistic renderings of what some of Dr. Mogk’s patients have seen.

Her visions started in the desert, a place known for mirages. But what Miriam Zeidman first saw was anything but a watery, undefined image in the distance. As she and her sister drove along a dusty Arizona road one evening, something came into sharp focus.

“I said to my sister, ‘My God! Look at all the apartment buildings!’ ” Zeidman recalls. “She said there were no apartment buildings, and I said there are, and she said no, there aren’t.”

The odd little exchange confused Zeidman, especially because the buildings looked so real. “I thought it was weird. I thought it was strange,” she says of the situation. Her sister just thought she was “totally crazy.” Still, Zeidman, who lives in Westland, was ready to write the whole thing off — until a few days later, when she began seeing faces, too.

Over the next year, Zeidman’s visions appeared to her daily, and in stark detail. One image appeared often, what Zeidman described as the face of a “gorgeous black woman” who wore an orange scarf around her neck. Like the apartment buildings, this face was so lifelike that Zeidman could have easily mistaken her for a real person had she not been aware for some time that these things she saw weren’t really there.

And yet Zeidman didn’t talk about any of this to anyone. She was afraid most people wouldn’t be as forgiving as her sister. Far from being “crazy,” Zeidman actually was suffering from Charles Bonnet Syndrome (CBS), a little-known affliction that is thought by experts to arise from the most common culprit of vision loss in aging adults.

A Swiss man named Charles Bonnet first noticed and recorded the symptoms in his grandfather in 1769, but it wasn’t until the mid 20th century that doctors formally identified CBS and started to recognize a curious body of symptoms — “phantom vision,” as some doctors still describe it today — in people who had varying degrees of vision loss. Patients would describe lifelike images that seemed to appear out of nowhere, and with no obvious context, like large wild animals in the middle of the living room, or vibrant flowers on a tree in winter.

But although CBS has come to be understood as a symptom of macular degeneration, it has remained relatively obscure and unknown, even in the medical community. This puzzles experts, including Dr. Mary Lou Jackson, director of vision rehabilitation at Massachusetts Eye and Ear and an assistant professor of ophthalmology at Harvard Medical School. Approximately 30 percent of people over the age of 75 show early evidence of macular degeneration. In her own practice, Jackson estimates that at least 25 percent of her patients with this common form of vision loss will also experience phantom visions caused by CBS, but in general won’t talk about it because they’re afraid it’s a sign of psychiatric issues or something much more serious, such as Alzheimer’s disease. Jackson thinks this is probably why CBS remains so mysterious and unknown. “For most people, it’s just an amusement,” Jackson says. “They realize that what they’re seeing isn’t right, and they don’t want other people to think they have mental illness.”

CBS is, in fact, innocuous, “like the brain just playing its own movie” to compensate for the type of vision loss created by macular degeneration, Jackson says. But that doesn’t mean doctors don’t need to be aware of it, especially in instances when patients have needlessly been referred to psychiatrists by those who’ve never heard of CBS.

“CBS is very underappreciated” in the medical community, “but not uncommon,” says Jackson, who’s enrolling patients in a study she hopes will shed more light on the curious condition.

The theory behind CBS is directly tied to what happens during macular degeneration. This form of vision loss affects the macula, the spot straight back from the front of the eye that allows us to process detail, such as tiny earrings on an earlobe, or street lights that turn green, or words on the page of a book. When the macula goes bad, however, you are no longer able to process these kinds of little details even though you can still technically “see.”

“It’s kind of like looking through frosted glass,” says Dr. Lylas Mogk, an ophthalmologist with Henry Ford Hospital and also a CBS expert.

CBS happens when the brain begins to “fill in” the lost details brought on by macular degeneration, sometimes in wild and fanciful ways. Mogk remembers one patient who constantly saw a Canadian Mountie in her living room. Another always saw little monkeys running around in the front yard. Many people who have macular degeneration will never experience CBS, but of those who do, the images will always be different and unique, Mogk explains. One common thread, though, is that the images people see are never personal to them. “So if you see a dog, it’s not your pet from childhood. If you see a person, it’s not your relative,” Mogk says. CBS might also linger for only a year and then go away, or it might remain with someone indefinitely, as in the case of Zeidman, who has seen images “constantly” for eight years. “It’s actually quite annoying,” says Zeidman, who tends to see buildings, faces, or “little girls running around.”

Despite these more mysterious aspects of CBS, experts are at least certain about two things: It’s not a sign that someone’s vision is getting worse, nor is it indicative of any kind of psychiatric issue. Unlike Zeidman, who admits she had fleeting moments when she thought she was crazy, Joan Lyon says she knew from the beginning that the visions she had were probably related to her macular degeneration.

“I looked out the kitchen window across the way where there were a bunch of maple trees, and they were covered with pink flowers, and I told my husband, ‘Look, they’re so pretty!’ ” Lyon says of the first time she experienced CBS two years ago. Lyon, who lives in Waterford Township, then started seeing different patterns on people’s clothing, recalling the time at a family wedding when she saw her daughter in a black-and-white outfit. About an hour later, Lyon wondered why her daughter had changed into something green.

“I asked her, why did you change? That was such a cute outfit you had on before!’ ” Lyon recalls. Of course, Lyon’s daughter had been wearing a green dress all along.

“I was never afraid to talk about it, but my regular doctor referred me to a psychiatrist, and I was quite offended,” Lyon says.

There’s no real “cure” for CBS, and experts are still trying to determine why the eyes seem to want to play tricks on those who are losing their vision. It’s a task that has taken on greater importance in recent years, Mogk says, as people have started to live to be old enough to develop macular degeneration, and therefore, CBS, in the first place. It takes decades for macular degeneration to develop and is due largely to exposure to environmental factors like smoking and sunlight that can cause inflammation in the eyes and damage the retinas over time.

“Macular degeneration was not diagnosed until about 20 years ago,” Mogk says. “It’s a new phenomenon, and in part it’s because people are living longer. A generation ago, you retired at 65 and you died at 68. You didn’t have time to get it.”

But as the huge population of Baby Boomers grows older, Jackson and Mogk predict macular degeneration will become more widespread, making CBS more common, as well. This generation will also be distinctly different from all those who have aged before them, Mogk says. They won’t consider themselves “old” in their late 70s or even their early 80s — and they will want to understand their health and take steps to preserve such critical functions as eyesight. Mogk says not smoking and avoiding sun exposure to the eyes are the top things people can do to protect their vision from macular degeneration.

Lyon, who’s in her 80s, is fairly certain that her years of sailing with her husband without wearing sunglasses is what brought on her own vision loss. And although there’s no cure for macular degeneration, Lyon’s experiences with CBS lasted only a year. She no longer sees the flowers or garish prints on people’s clothing.

“And yes, I was relieved when I found out about [CBS],” Lyon says. “It was a huge weight that was lifted off my shoulders.”

Six Telltale Signs of Charles Bonnet Syndrome:

1. Images occur when you’re fully awake and conscious.

2. You know the images aren’t real.

3. They occur in combination with normal perception.

4. They’re exclusively visual, with no accompanying sounds or other sensations.

5. They appear and disappear without obvious cause.

6. They’re amusing or annoying, but not grotesque.

Source: Macular Degeneration: The Complete Guide to Saving and Maximizing Your Sight, by Lylas G. Mogk, M.D., and Marja Mogk, Ph.D.