Visual Release Hallucinations
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Visual release hallucinations, also known as Charles Bonnet syndrome or CBS, are a type of psychophysical visual disturbance in which a person with partial or severe
blindness Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as correctable eyewear, assistive devices, and medical treatment†...
experiences visual
hallucination A hallucination is a perception in the absence of an external stimulus that has the qualities of a real perception. Hallucinations are vivid, substantial, and are perceived to be located in external objective space. Hallucination is a combinati ...
s. First described by
Charles Bonnet Charles Bonnet (; 13 March 1720 – 20 May 1793) was a Genevan naturalist and philosophical writer. He is responsible for coining the term ''phyllotaxis'' to describe the arrangement of leaves on a plant. He was among the first to notice parthe ...
in 1760, the term ''Charles Bonnet syndrome'' was first introduced into English-speaking
psychiatry Psychiatry is the specialty (medicine), medical specialty devoted to the diagnosis, prevention, and treatment of mental disorders. These include various maladaptations related to mood, behaviour, cognition, and perceptions. See glossary of psych ...
in 1982. A related type of hallucination that also occurs with lack of visual input is the
closed-eye hallucination Closed-eye hallucinations and closed-eye visualizations (CEV) are hallucinations that occur when one's eyes are closed or when one is in a darkened room. They can be a form of '' phosphene''. Some people report CEV under the influence of psyched ...
.


Signs and symptoms

People with significant
vision loss Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as correctable eyewear, assistive devices, and medical treatment†...
may have vivid recurrent visual hallucinations (fictive visual percepts). One characteristic of these hallucinations is that they usually are " lilliputian" (hallucinations in which the characters or objects are smaller than normal). Depending on the content, visual hallucinations can be classified as either simple or complex. Simple visual hallucinations are commonly characterized by shapes,
photopsias Photopsia is the presence of perceived flashes of light in the field of vision. It is most commonly associated with: * posterior vitreous detachment * migraine aura ( ocular migraine / retinal migraine) * migraine aura without headache * scint ...
, and grid-like patterns. Complex visual hallucinations consist of highly detailed representations of people and objects. The most common hallucination is of faces or cartoons. Sufferers understand that the hallucinations are not real, and the hallucinations are only visual, that is, they do not occur in any other senses (such as hearing, smell or taste). Visual hallucinations generally appear when the eyes are open, fading once the visual gaze shifts. It is widely claimed that sensory deprivation is instrumental in the progression of CBS. During episodes of inactivity, hallucinations are more likely to occur. The majority of those suffering from CBS describe the duration of hallucinations to continue for up to a few minutes, multiple times a day or week. Even though people of all ages may be afflicted by Charles Bonnet syndrome, those within the age range of 70 to 80 are primarily affected. Among older adults (> 65 years) with significant vision loss, the prevalence of Charles Bonnet syndrome has been reported to be between 10% and 40%; a 2008 Australian study found the prevalence to be 17.5%. Two Asian studies, however, report a much lower prevalence. The high incidence of underreporting this disorder is the greatest hindrance to determining the exact prevalence. Underreporting is thought to be a result of sufferers being afraid to discuss the symptoms out of fear that they will be labeled of unsound mind.


Pathophysiology

There is no general consensus on the definition of CBS. Predominant factors correlated with CBS are a decrease of visual acuity, visual field loss, and elderly age. While characteristic features of visual hallucinations are not specifically linked to the anatomical site of the ocular injury, they usually match to the location of visual loss. The most commonly accepted theory for Charles Bonnet Syndrome proposes that extreme visual impairment promotes sensory deafferentation, leading to disinhibition, thus resulting in sudden neural firings of the visual cortical regions. A few studies record that visual hallucinations are likely to be concentrated in the blind regions. Functional magnetic resonance imaging (fMRI) of Charles Bonnet Syndrome patients displays a relationship between visual hallucinations and activity in the ventral occipital lobe. A connection between
age-related macular degeneration Macular degeneration, also known as age-related macular degeneration (AMD or ARMD), is a medical condition which may result in blurred or no vision in the center of the visual field. Early on there are often no symptoms. Over time, however, som ...
(AMD) and colored visual hallucinations has been presented. Color vision signals travel through the parvocellular layers of the
lateral geniculate nucleus In neuroanatomy, the lateral geniculate nucleus (LGN; also called the lateral geniculate body or lateral geniculate complex) is a structure in the thalamus and a key component of the mammalian visual pathway. It is a small, ovoid, ventral projec ...
(LGN), later transmitting down the color regions of the ventral visual pathway. Due to cone photoreceptor damage located in the macula, there is a significant reduction of visual input to the visual association cortex, stirring endogenous activation in the color areas and thus leading to colored hallucinations. Patients with CBS alongside macular degeneration exhibit hyperactivity in the color areas of the visual association cortex (as shown in fMRIs). Those who have significant ocular disease yet maintain visual acuity may still be susceptible to CBS. The Deep
Boltzmann Machine A Boltzmann machine (also called Sherrington–Kirkpatrick model with external field or stochastic Ising–Lenz–Little model) is a stochastic spin-glass model with an external field, i.e., a Sherrington–Kirkpatrick model, that is a stochastic ...
(DBM) is a way of utilizing an undirected probabilistic process in a neural framework. Researchers argue that the DBM has the ability to model features of cortical learning, perception, and the visual cortex (the locus of visual hallucinations). Compelling evidence details the role homeostatic operations in the cortex play in regards to stabilizing neuronal activity. By using the DBM, researchers show that when sensory input is absent, neuron excitability is influenced, thus potentially triggering complex hallucinations. A short-term change in the levels of feedforward and feedback flows of information may intensely affect the presence of hallucinations. In periods of drowsiness, CBS related hallucinations are more prone to arise. Disrupting cortical homeostatic processes after vision has been lost may prevent or setback the emergence of hallucinations. At varying stages of the cortical grading, acetylcholine (ACh) may impact the balance of thalamic and intracortical inputs as well as the balance in between bottom-up and top-down. Particularly in CBS, a shortage of acetylcholine at cortical locations should correspond to the onset of hallucinations. The syndrome can also develop after bilateral optic nerve damage due to methyl alcohol poisoning.


Diagnosis

A variety of disciplines including optometry, ophthalmology, geriatric medicine, psychiatry, and neurology play a part in securing the diagnosis of CBS. Since CBS is not commonly recognized by all clinicians, it oftentimes goes misdiagnosed and identified as
psychosis Psychosis is a condition of the mind that results in difficulties determining what is real and what is not real. Symptoms may include delusions and hallucinations, among other features. Additional symptoms are incoherent speech and behavior ...
, delirium, or
dementia Dementia is a disorder which manifests as a set of related symptoms, which usually surfaces when the brain is damaged by injury or disease. The symptoms involve progressive impairments in memory, thinking, and behavior, which negatively affe ...
. As a result of this, it is estimated that almost 60% of CBS patients hesitate to notify their physicians. By focusing on the specific type of visual hallucination, one may find an accurate diagnosis. If a patient presents symptoms indicative of Charles Bonnet syndrome, basic laboratory examinations like metabolic panel and blood count tests, as well as neuroimaging, may aid in an accurate diagnosis.


Prognosis

There is no treatment of proven effectiveness for CBS. For those experiencing CBS, knowing that they are suffering from this syndrome and not a mental illness seems to be the most comforting treatment so far, as it improves their ability to cope with the hallucinations. As time passes from the initial onset of visual hallucinations, studies show that around 60% of those living with CBS feel that visual hallucinations have no effect on their lives, 33% of people feel that the hallucinations are disruptive to their lives, and 7% of people even find pleasure in the hallucinations. A large proportion of those suffering from CBS develop the visual hallucinations as vision begins to deteriorate and stop hallucinating once vision is entirely gone. Complex hallucinations may progress over time if the primary loss of vision is due to damage of the early cortical areas. If activation of the early cortical areas is suppressed when CBS symptoms have already been exhibited, hallucinations may temporarily terminate. Also, interrupting vision for a short time by closing the eyes or blinking may be helpful. It is possible for a stressful life event to alter the disposition of hallucinatory experiences as well as the emotional experiences (from unconcerning to concerning) in CBS. As expressed in some patients, an interplay between CBS and an acute or post-traumatic stress disorder may exist. The role that trauma plays in CBS may affect how and when a hallucinatory episode is triggered.


History

The disease was first noted by the Swiss naturalist
Charles Bonnet Charles Bonnet (; 13 March 1720 – 20 May 1793) was a Genevan naturalist and philosophical writer. He is responsible for coining the term ''phyllotaxis'' to describe the arrangement of leaves on a plant. He was among the first to notice parthe ...
, who described the condition in 1760. He documented it in his 90-year-old grandfather who was nearly blind from
cataract A cataract is a cloudy area in the lens of the eye that leads to a decrease in vision. Cataracts often develop slowly and can affect one or both eyes. Symptoms may include faded colors, blurry or double vision, halos around light, trouble ...
s in both eyes. After Bonnet’s grandfather received bilateral cataract surgery, his vision evolved from slightly better to complete deterioration over time. It was around this period that his visual hallucinations started. His hallucinations consisted of perceptions of men, women, birds, carriages, buildings, tapestries, physically impossible circumstances and scaffolding patterns. Even though his health was in good shape and he had an absence of any psychiatric disorders, the source of the hallucinations remained unknown. At forty years old, Charles Bonnet himself suffered from an unrevealed cause of severe vision loss and experienced the hallucinations. In 1936, Jean Lhermitte and Julian de Ajuriaguerra, concluded that visual hallucinations consist of thalamic lesions as well as ocular pathology. In 1967, French-Swiss neurologist, Georges de Morsier, coined the term ''Charles Bonnet Syndrome'' in Bonnet's honor. De Morsier’s description of CBS implies a concentrated neurodegeneration, usually occurring in the elderly with typical cognition. In psychiatric literature, the most commonly accepted interpretation of CBS is that of Gold and Rabins’. In 1989, they detailed that the hallucinations associated with CBS are not affecting other sensory modalities. They believed that the visual hallucinations are oftentimes stereotyped, persistent, and/or repetitive in nature.


Society and culture

The syndrome is discussed in: * Vilayanur S. Ramachandran's book ''
Phantoms in the Brain ''Phantoms in the Brain: Probing the Mysteries of the Human Mind'' (also published as ''Phantoms in the Brain: Human Nature and the Architecture of the Mind'') is a 1998 popular science book by neuroscientist V.S. Ramachandran and ''New York Time ...
''. Ramachandran suggests that
James Thurber James Grover Thurber (December 8, 1894 – November 2, 1961) was an American cartoonist, writer, humorist, journalist and playwright. He was best known for his cartoons and short stories, published mainly in ''The New Yorker'' and collected ...
, who was blinded in one eye as a child, may have derived his extraordinary imagination from the syndrome. * Vikram Chandra's book '' Sacred Games'' (2006) *
David Eagleman David Eagleman (born April 25, 1971) is an American neuroscientist, author, and science communicator. He teaches neuroscience at Stanford University and is CEO and co-founder of Neosensory, a company that develops devices for sensory substitut ...
's book '' Incognito: The Secret Lives of the Brain'' *
Oliver Sacks Oliver Wolf Sacks, (9 July 1933 – 30 August 2015) was a British neurologist, naturalist, historian of science, and writer. Born in Britain, Sacks received his medical degree in 1958 from The Queen's College, Oxford, before moving to the Uni ...
' 2012 book '' Hallucinations'' * The Indian movie ''
Jawan of Vellimala ''Jawan of Vellimala'' () is a 2012 Malayalam film written by James Albert and directed by debutant Anoop Kannan. It stars Mammootty, Sreenivasan, Mamta Mohandas and Asif Ali. Cast * Mammootty as Gopikrishnan,retired soldier, Indian Army ...
'', released in 2012, in which Mammootty is a victim of the syndrome * ''The Black Canvas'' (2014), a chamber opera by the Greek composer Spyros Syrmos, is about a celebrated painter whose visions are caused by CBS. * Margaret Atwood's short story "Torching the Dusties" *
Deborah Lawrenson Deborah Lawrenson (born November 1960 in London, England) is a British novelist and journalist. She was educated at Blackheath High School, The British School of Brussels and Eastbourne College, and read English at Trinity College, Cambridge. Sh ...
's novel ''The Lantern'' (2011) * Gareth Brookes' graphic novel ''A Thousand Coloured Castles'' (2017) * '' Dealt'', 2017 documentary about notable card mechanic Richard Turner * The 2019
Netflix Netflix, Inc. is an American subscription video on-demand over-the-top streaming service and production company based in Los Gatos, California. Founded in 1997 by Reed Hastings and Marc Randolph in Scotts Valley, California, it offers a fi ...
film '' Velvet Buzzsaw''


See also

* * * * *


References


External links


Information on Charles Bonnet syndrome from RNIB

National Public Radio article with an audio segment about Charles Bonnet syndrome


Ted Talk, Feb 2009.


'Damn Interesting' article on Charles Bonnet syndrome
* {{Medical resources , DiseasesDB = , ICD10 = , ICD9 = , ICDO = , OMIM = , MedlinePlus = , MeSH = , GeneReviewsNBK = , GeneReviewsName = Hallucinations Symptoms and signs of mental disorders Psychopathological syndromes