She is eight years old and was referred for neuropsychological assessment consequent to being in a car accident when she was only a few months old. By age eight, both sides of the lawsuit had waited a sufficient amount of time (according to the pediatric neurologist) to evaluate the effects of the accident.

Everything was going along as expected. She was completing the testing in various modalities, which would give us insight into her strengths and weaknesses in varied domains. These included: intelligence, perception, motor, language, learning, memory and achievement. All of a sudden, she said: "That purple light, and those little men are back again." I asked her to repeat what she had just said, as I wasn't sure I understood her. Again she noted, quite rationally, "The purple light and those little men are back again." This time she added: "Well I'm pretty sure you don't see them; nobody does, but I do."

Here was a seemingly perfectly normal child of eight, completing over an hour-and-a-half of testing with no "odd" behavior noted. There hadn't been any signs of emotional fragmentation, and now these delusions came up in the course of the evaluation. When asked about her visions and if this was the first time she experienced them, she answered no; she had seen them many times and had even told her mother about them. Her mother, she noted, had told her that many children had imaginary friends and fantasies and left it at that.

 

I checked and re-checked the subtests for signs of perceptual difficulties and found the results to be in the normal range.

 

Delusions, hallucinations, illusions - what is the difference? "An illusion is a perceptual disturbance, while a delusion is a belief disturbance" (David A. Gershaw, Ph.D., 1994).

People with a mental illness have trouble telling the difference between their fantasies and reality and will often come up with complicated explanations for the things they are seeing (sometimes called a delusion). An illusion is a misleading perception, usually visual. You see something, but you consistently misjudge its length, shape, motion or direction.

 

In their extreme form, delusions and hallucinations are symptoms of people who are psychotic; they cannot clearly distinguish what is real, from what is not. With psychotic disorders, hallucinations are most frequently auditory - like hearing voices.

I then began researching under what circumstances might someone have hallucinations, delusions or illusions without obvious psychosis, and found a fascinating disorder: Charles Bonnet Syndrome.

 

This syndrome generally affects older individuals with diminishing eyesight. However, the literature does note that it may occur in children. (J AAPOS. Oct. 1998;2(5):310-3). (Schwartz TL, Vahgei L. 26506-9193, USA) (BMJ 2004;328:1552-1554 (June 26), doi:10.1136/bmj.328.7455.1552.

 

What Is Charles Bonnet Syndrome?

A Swiss philosopher named Charles Bonnet first described this condition in the 1760 when he noticed his grandfather, who was blinded by cataracts, describing how he saw birds and buildings, which were not there. Although the condition was described very early, it is still largely unknown by ordinary doctors and nurses. This is partly because of a lack of knowledge about the syndrome, and partly because people experiencing it don't talk about their problems out of fear of being thought of as mental health difficulties.

At the moment, little is known about how the brain stores the information it gets from the eyes and how we use this information to help us create the pictures we see. There is some research that shows that all this constant seeing actually stops the brain from creating its own pictures.

People with Charles Bonnet Syndrome (or "Bonnet-people") are otherwise mentally sound. The "beings" appear when the Bonnet-people's vision deteriorates as a result of eye diseases such as age-related macular degeneration - or when patients have had both eyes removed. Though there have been reported cases in children, Charles Bonnet Syndrome is more common in older people with a high level of education.

Is There a Cure for Charles Bonnet Syndrome?

Unfortunately at the moment, there is no known cure or treatment for CBS. However, just knowing that it is poor vision and not mental illness that causes these problems often helps people come to terms with them. Generally, these experiences will disappear after about a year or 18 months, but of course, this will not happen for everyone with this problem.

Doctors are unfamiliar with the syndrome as a possible diagnosis. Near-misses have been reported, in which patients were almost confined to mental health institutions. Given the prevalence of partial visual impairment, the number of people in the community - especially elderly people, who do not report the symptoms for fear of being labeled as mentally unwell or demented - must be substantial.

Clinicians must, therefore, be aware and ask elderly people with visual impairment whether they have hallucinations. Firm reassurance that the syndrome is not related to mental illness is in itself a major relief to an elderly person already burdened with failing vision, social isolation and other medical problems.

I recommended that my patient be seen by a pediatric retinal specialist and a psychiatrist to help make a differential diagnosis. The specific outcome notwithstanding, I feel that this case and awareness of Charles Bonnet Syndrome may help many elderly people in our midst with failing sight, especially those who are having 'hallucinations' and are frightened that they may be thought of as mentally disturbed. We need to reassure them that they may be Bonnet/Bonnie people.

 

Originally published in the Jewish Press on November 22, 2006.

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