We were recently approached by a student called Abbie Cohen from the University of Sheffield who is doing a journalism course and wanted to write an article about Charles Bonnet Syndrome. This is the vey interesting story that she put together:  Phantom visions: the mystery of Charles Bonnet Syndrome  To this day, Charles Bonnet Syndrome remains unknown not only to the general public, but also to members of the medical profession. We look into the condition, addressing the causes and treatments available for patients as well as the need to raise awareness about it.

Timothy Lee*, a 40-something-year-old visually impaired man, sits quietly at a table in the lounge area of Sheffield Royal Society for the Blind. He raises one arm, making grasping movements with his hand, then does the same with his other hand, and repeats these movements several times.

'I'm catching my butterfly,' he tells Bessie Goddard, a support group leader at the Society.

Although what he sees is unique to him, Mr Lee is not the only one who experiences visions as part of his everyday life; others like him will see giant caterpillars in their gardens, people sitting on their windowsills, or rows of buses in the street that aren't really there. These people have Charles Bonnet Syndrome, a condition whereby visually impaired individuals are known to experience visual hallucinations.

In the United Kingdom, around two million people have been diagnosed with having visual hallucinations of various causes. Amongst these, according to a report published in 2013 by the Royal College of Ophthalmologists and the Royal National Institute of Blind People, more than 100 000 are believed to have Charles Bonnet Syndrome. Why has this condition remained such a mystery, both to the general public and the medical profession? More importantly, what still needs to be done in order to help the patients who, often unknowingly, have this condition? 

Charles Bonnet Syndrome in overview

Dr Dominic Ffytche, expert in visual perceptual disorders and a Clinical Senior Lecturer in Old Age Psychiatry at King's College London, says that Charles Bonnet Syndrome is not associated with mental illness; rather, it develops as the brain rewires itself in response to having an eye disease that causes substantial sight loss.

'Not everyone with a visual impairment has this condition. You do not need to be completely blind to have Charles Bonnet Syndrome, but you do need to lose a certain amount of vision in order to be at risk,' says Dr Ffytche.

According to Manoj Kulshrestha, General Ophthalmologist and Consultant Ophthalmic Surgeon at Hywel Dda University Health Board in Wales, several of these eye diseases include retinal detachment, choroidal haemorrhage, glaucoma, cataract and macular degeneration, the latter of which is the leading cause of blindness in adults in the UK and the Western world.

'Young visually impaired people with severe hereditary macular dystrophies may develop the condition, but not with the same frequency as elderly people, unless the underlying condition is particularly severe,' says Mr Kulshrestha.

'If the dystrophy is progressive causing more severe visual loss, it may occur in later life.'

Mrs Goddard, 89, now head of the Charles Bonnet Syndrome support group at the Sheffield Royal Society for the Blind, also suffers from the condition. She says her that own experiences with it arose as a byproduct of her age-related macular degeneration in her 70s.

'When I found out I had it, at the time, I wore reading glasses and did not realise my vision was going,' says Mrs Goddard.

'I see patterns like Christmas streamers on walls, and arches in the background of my television screen. I see little creatures or people as tall as the ceiling moving towards me. When I look away, they're gone.'

'It is very hard to explain to someone who knows nothing about it, especially as no two people with the condition are the same. It isn't a condition that just happens; it gradually creeps up on you, and you're never always sure whether it's a vision.'

These visual hallucinations, rather than associated with the patients' memories, are believed to be constructed by the brain. The visions fall into distinct categories, the most common of which include small dots, geometrical patterns, disembodied faces described as grotesque and deformed, and figures that are often seen wearing a type of headgear. Patients who also see text and letters as a part of their visions are, according to Dr Ffytche, incapable of actually reading the words.

'These categories all reflect different parts of our brain, which are specialised in different aspects of our day-to-day vision. It is when these bits of the brain become activated when they shouldn't be, that we perceive figures, faces or colours when we aren't supposed to,' adds Dr Ffytche. 

Furthering research and raising awareness

Charles Bonnet Syndrome is still very much a mystery to most, including professionals. According to Tim Goddard, Public Relations Officer at the Macular Society, research into Charles Bonnet Syndrome is on the rise yet remains insufficient, as larger scales of study are still required in order to better understand this condition.

'We need to find ways of controlling the hallucinations, in order to prevent them or to generally aid our understanding of them,' says Mr Goddard.

'Charles Bonnet Syndrome is something that is often not taken as seriously as we think it should be by the medical profession, and not enough research has been done.'

Because of the lack of research carried out in the area over the years, doctors are also beginning to find errors and misconceptions in past research. Over the last six months, recent studies have demonstrated that the visual hallucinations caused by Charles Bonnet Syndrome are likely to persist for much longer than the time frame that was initially thought.

'For the last 20 or 30 years or so, we've been telling people that Charles Bonnet Syndrome gets better with time and goes away within 18 months, but we've just done a study that shows that this probably isn't correct,' says Dr Ffytche.

'People have hallucinations for much longer than that; they can become less intense but still, they won't go away for many, many years.'

Steve Hambleton, General Manager at the Sheffield Royal Society for the Blind, believes that even though a lack of awareness exists amongst the general public, more could be done to help doctors understand the condition:

'Unfortunately, despite Charles Bonnet Syndrome having first been recognised in the 19th century, there are still many people in the medical profession who are not aware of the condition,' says Mr Hambleton.

'Certainly more could be done to raise awareness amongst the medical profession and the Society does try to do this in a limited way, through the work experience placements we offer to medical students and the visual impairment awareness training we deliver to medical students at the University of Sheffield.'

Raising the right kind of awareness

Crucially, increased awareness amongst the general public about the very existence of this disease will help prevent patients who have the condition, yet who are unaware that it exists, from experiencing what is known as Negative Outcome Charles Bonnet Syndrome, whereby they are frightened by their visions and prevented from carrying out basic everyday activities.

'It is important that people know about it before they experience it. More does need to be done about this, as not enough people know about Charles Bonnet Syndrome,' says Mr Goddard.

'If you know about the possibility of your having it, then you're less likely to worry about it; on the other hand, if people don't know anything about the condition, then they often link it to dementia or mental illness and may experience negative effects.'

Dr Ffytche agrees, and also believes that increased awareness will help provide accurate information about the number of people affected by the condition in the UK.

'It is hard to know how many people have Charles Bonnet Syndrome, because many people who experience visual hallucinations think they have a mental illness and don't tell anyone about them,' says Dr Ffytche.

A further issue that has also risen is that of inaccuracy in media coverage of this condition – an issue that must be addressed, if we are to properly understand and inform the public about it in the long run.

'This is one challenge that we're facing; even if we want to raise awareness, we need to make sure we're raising awareness in the right way, and that people fully understand Charles Bonnet Syndrome before they spread the word about it,' says Mr Goddard.

What forms of treatment can be given to patients suffering from this condition, when research is still underway in order to be able to merely understand it? According to Mr Hambleton, there are no specific treatments available for Charles Bonnet Syndrome as of yet. From the patient's point of view, however, Bessie Goddard insists it is important for people with the condition to not only become aware of it, but to also share their experiences with relatives and friends.

'Everyone with Charles Bonnet Syndrome struggles with it at first, because they don't know what it is,' she says.

'They don't know where to go or who to go to. You need to learn to just laugh about it, and it's involving your family and talking to people that truly helps,' says Bessie Goddard.

Where speaking to family and friends can be difficult, for people like Sheffield-based Timothy Lee whose butterfly visions affect him on a day-to-day basis, societies such as the Sheffield Royal Society for the Blind provide support through counselling and group sessions. These have helped some patients cope by simply talking to other patients with the condition, helping them begin to see Charles Bonnet Syndrome in a more positive light.

'Back in 2002, we recognised that our clients who have Charles Bonnet Syndrome found this condition very distressing, and were uncomfortable talking about it with family and friends,' says Mr Hambleton.

'This prompted us to start the support group so that they could talk about it amongst people that were experiencing the same problem. The group helped them to gain confidence in dealing with the problem and explaining it to family and friends.'

*Name has been changed for the purposes of this article