Bonnet Syndrome Research Articles

Cerebellar Parieto-occipital Posterior Reversible Encephalopathy Syndrome and Cerebral Metamorphopsia Associated with Asymptomatic Atrial Septum Vegetation and Renal Disease: Case Report

Patient: Female, 25-year-oldFinal Diagnosis: Posterior reversible encephalopathy syndromeSymptoms: Visual disturbancesMedication: —Clinical Procedure: —Specialty: NeurologyObjective:Rare co-existance of disease or pathologyBackground:Posterior reversible encephalopathy syndrome (PRES) is a poorly characterized and enigmatic syndrome. Despite consistently presenting with nervous system vasogenic edema, this malady has been associated with variable triggers, neurological symptoms, and natural history.Case Report:The report presents a 25-year old African American female who presented with altered mental status and bilateral cortical blindness. Neuroimaging identified vasogenic edema in the cerebellum, parietal lobe, and occipital lobe. Her PRES was associated with a hypertensive emergency, renal failure, and an atrial septum vegetation (culture-negative endocarditis). All 3 contributing etiologies were addressed, upon which the patient began to recover. During recovery, the patient experienced cerebral metamorphopsia, visualizing her entire environment in the form of a cartoon. After 2 weeks of treatment she recovered to baseline state of heath, with vasogenic edema resolved on follow-up neuroimaging.Conclusions:This case presents a rarely catalogued phenomena during PRES recovery, cerebral metamorphopsia, along with a new potential association (culture negative atrial septum endocarditis). The report also highlights how PRES recovery patients (with cortical blindness) should be explicitly assessed for cerebral metamorphopsia and Charles Bonnet syndrome – which may distress patients. Lastly, the atypical presentation of cerebellar vasogenic edema in our patient validates existing literature that PRES does not have a uniform picture and is not well served by its current name or proposed diagnostic criteria. Therefore, renaming the disorder to reversible vasogenic edema syndrome and derestricting the diagnostic criteria, may prevent clinicians from being discouraged when faced with diagnosing PRES in the face of atypical findings.

Atypical Charles Bonnet Syndrome in a patient with hemianopia and cognitive impairment after right occipitotemporal stroke.

Atypical Charles Bonnet Syndrome in a patient with hemianopia and cognitive impairment after right occipitotemporal stroke.

This issue of acta

This issue of acta

A patient with nocturnal visual hallucinations.

Citation:Dholakia S. A patient with nocturnal visual hallucinations. J Clin Sleep Med. 2020;16(5):821–822.

Clinical Characteristics of the Charles Bonnet Syndrome in Patients with Neovascular Age-Related Macular Degeneration: The Importance of Early Detection

Purpose: We investigated the characteristics, prognosis, and clinical outcome of the Charles Bonnet syndrome (CBS) in patients with neovascular age-related macular degeneration (AMD). Methods: Five hundred psychiatrically healthy patients with neovascular AMD were screened for CBS. The individuals that fulfilled the inclusion criteria were systematically interviewed using a structured questionnaire that covered the impact, prognosis, risk factors, phenomenology, symptoms, and knowledge about the syndrome. A control group of 45 patients was used for comparison. Demographic data, current medication, and ocular risk factors were collected in all patients. Results: Forty-five patients with CBS were identified. The majority of patients reported images that consisted of colored (62%) animals (44%) or faces (42%) that lasted for seconds (53%). Most patients reported a self-limited disease with a median duration of symptoms between 9 and 11.5 months, with only 7% knowing about CBS at symptom onset. The degree of visual deficit did not predict the characteristics, complexity, frequency, duration, or impact of visual hallucinations. One-third of patients reported negative outcome, which was associated with shorter duration of CBS (p = 0.023), fear-inducing images (p < 0.001), and impact on daily activities (p = 0.015). Conclusion: The prevalence of CBS in neovascular AMD patients is high and clinically relevant. Patients with recent onset of visual hallucinations and describing fear-inducing images are at greater risk for negative outcome. Periodic screening may minimize the negative consequences of this disease.

Visual hallucinations in the works of Oliver Sacks

Hallucinations are one of the most bizarre experiences in several diseases. They appear in mental diseases as well as in physical illnesses and may be the consequence of the usage of drugs of abuse. However, a detailed analysis of how patients feel under hallucinations caused by different diseases is uncommon. This article analyses how visual hallucinations are considered in the works of the neurologist and writer Oliver Sacks, with special attention to his book Hallucinations. Hallucinations have been under consideration by culture, religion and arts, which has led to multiple interpretations. Sacks's interest in perception of sensations led him to work on the analysis of hallucinations, given the limited knowledge on the topic. References to hallucinations appeared in several of his books like Migraine, Awakenings and A leg to stand on. In Musicophilia Sacks approached the auditory hallucinations and in Hallucinations he considered them in depth. In the latter work, Sacks analyses especially those present in Charles Bonnet syndrome, in situations of sensory deprivation, in patients with epilesy, those present during treatment with levodopa and those caused by drug of abuse. Hallucinations is one of Sacks's books with greater neuroophthalmological content. The descriptions of the hallucinations of his patients or those experienced by himself, as well as the reflective analysis on the world of perception make this book one of the most fascinating works of Oliver Sacks.

AB032. Exploring the effects of a retinal prosthesis on visual hallucinations related to Charles Bonnet syndrome

Background: A first occurrence in the province of Quebec (Canada) of the use of a retinal prosthesis system (RPS) by a patient with a Charles Bonnet syndrome (CBS) provides a rare opportunity to explore the effects of this system on visual hallucinations (VH) related to CBS. Considering that the RPS artificially induces a visual perception and that VH related to CBS occur in cases of significant vision loss, this study aimed to explore whether the recovery of a visual perception could affect the nature and frequency of VH related to CBS.

Functional MRI study in a case of Charles Bonnet syndrome related to LHON

IntroductionCharles Bonnet syndrome is characterized by simple or complex visual hallucinations (VH) due to damage along the visual pathways. We report a functional MRI study of brain correlates of VH in the context of a severe optic atrophy in a patient with Leber’s Hereditary Optic Neuropathy (LHON).Case reportA 62-year-old man was diagnosed with LHON (11778/ND4 mtDNA mutation) after subacute visual loss in left eye (right eye was amblyopic). One month later, he experienced VH of a few seconds consisting in “moving red and blue miniature cartoons”. One year later VH content changed in colored mosaic (10–15 s duration), usually stress-related, and blue and white flashes (2–5 s), triggered by unexpected auditory stimuli. Audiometry revealed mild sensorineural hearing loss. Three block design functional MRI paradigms were administrated: 1) random “clap”, 2) “checkerboard” and 3) non-random “beep”. After random “claps” simple flashes were evoked with bilateral activation of primary and secondary visual cortex, cuneus, precuneus and insula. Neither hallucinations nor cortex activation were registered after “checkerboard” stimulation, due to the severe visual impairment. Primary and secondary auditory cortices were “beep”-activated, without eliciting VH by non-random “beep”.ConclusionsThe peculiarity of our case is that VH were triggered by random auditory stimuli, possibly due to a cross-modal plasticity between visual and auditory networks, likely influenced by the sensorineural deafness. Functional alterations of both networks in resting conditions have been demonstrated in LHON patients, even without an auditory deficit. Finally, the absence of VH triggered by expected stimuli is consistent with the “expectation suppression theory”, based on increased neural activations after unexpected but not by predicted events.

Evaluation of the Clinical Features, Management, and Prognoses of Patients With Charles Bonnet Syndrome.

Charles Bonnet syndrome (CBS) is characterized by visual hallucinations with preservation of cognitive abilities. The hallucinations consist mostly of vivid (realistic) objects and tend to reoccur. Here, we evaluate the etiologies, symptoms, treatments, and prognoses of 13 CBS cases. All patients had visual hallucinations but were normal on cognitive and psychiatric assessments. Patient demographic and clinical characteristics, treatment options, and 3-month follow-up data were retrospectively reviewed. The possible causes of CBS and what the patients perceived during their hallucinations were recorded. Antipsychotic agents, such as risperidone and quetiapine, and anticonvulsants, such as levetiracetam, may be effective in some cases.

Prevalence of Charles Bonnet syndrome in patients with age-related macular degeneration: systematic review and meta-analysis.

Age-related macular degeneration (AMD) is the most common cause of visual impairment in the developed world. A number of patients experience complex lifelike visual experiences-Charles Bonnet syndrome (CBS). In this systematic review, our aim was to provide an overview of the CBS literature in relation to AMD, to determine the prevalence of CBS in patients with AMD and to provide an overview of associated demographical and clinical aspects. We searched the literature databases PubMed/MEDLINE, EMBASE, Web of Science, the Cochrane Central, and PsycINFO on 22 March 2019 for studies evaluating the prevalence of CBS in patients with AMD. Two independent authors extracted the data and evaluated risk of bias. Studies were reviewed qualitatively in the text and quantitatively in a meta-analysis including subgroup analyses for differences between demographic and clinical factors. We identified 18 studies with data on >4303 patients with AMD. We found an overall prevalence of CBS of 15.8% (95% confidence interval: 11.0%-21.2%). When looking at consecutively recruited patients with neovascular AMD from the clinic, prevalence of CBS was 7.2% (95% confidence interval: 4.3%-10.6%). Among visitors to visual rehabilitation centres, prevalence of CBS was 31.6% (95% confidence interval: 21.7%-42.3%). Taken together, we find that CBS is rather common in patients with AMD.

The elephant in the room: understanding the pathogenesis of Charles Bonnet syndrome.

Charles Bonnet syndrome (CBS) is a syndrome characterised by complex visual hallucinations in individuals who are cognitively normal, though often elderly and visually impaired. Although first described over 250years ago, the condition remains poorly understood and difficult to treat. Our understanding of CBS pathogenesis has advanced little since it was first described, and much of the recent literature consists of case studies strikingly similar to the first published account of CBS. However, imaging studies have provided some indication as to the cortical areas implicated in the genesis of complex visual hallucinations, and the existence of similar hallucinatory syndromes in other sensory modalities suggests a common underlying mechanism. This review begins by describing what is currently known about CBS, focusing on epidemiology, clinical presentation and diagnosis. It then explores potential starting points for better understanding the pathogenesis of CBS, namely the existence of similar conditions in other sensory modalities and the reproduction of complex visual hallucinations in sensory deprivation scenarios. Finally, it discusses how CBS should be approached in clinical practice.

Charles Bonnet Syndrome Prevelance in Patients with Low Visual Acuity

Charles Bonnet Syndrome Prevelance in Patients with Low Visual Acuity

The Charles Bonnet Syndrome: a Systematic Review of Diagnostic Criteria.

To perform a systematic review of diagnostic criteria for the Charles Bonnet syndrome (CBS). Across 33 studies that specified diagnostic criteria for CBS, hallucinations and vision loss were a common requirement, but there was considerable heterogeneity regarding hallucination properties (i.e., formed vs. unformed) and the severity of vision loss. The exclusion of confounding neuropsychiatric disorders was also common, but specific disorders and their method of ascertainment were variable. There is considerable diagnostic heterogeneity for CBS in the literature. These differences have important implications for the results of observational and interventional studies of CBS and highlight the need for unified diagnostic criteria.

Giant Cell Arteritis and Charles Bonnet Syndrome. The Case of Saint Martin of Leon

Saint Martin of Leon was a monk who lived in the 12th century. The details of his life are known because they were described by his contemporary, Lucas de Tuy, in the Liber de Miraculis Sancti Isidori. Saint Martin of Leon was a venerable old man who suffered from severe headaches, considerable difficulties in raising or maintaining his arms raised, asthenia, and anorexia. In addition, he is represented in his codex Concordia with an unusual hyperpigmentation of the temples suggesting a therapeutic intervention on temporal arteries. These data lead us to think that this could be the first described case of giant cell arteritis with some clinical information. Moreover, he experienced a singular and curious appearance of Saint Isidore of Seville, who made him swallow a booklet, which might correspond to a complex visual hallucination or associated Charles Bonnet syndrome. Historical data on the disease are reviewed and discussed, as well as its evolution and treatment before giving steroids.

“Playthings of the Brain”: Phantom Visions in Charles Bonnet Syndrome

ABSTRACTCharles Bonnet Syndrome (CBS) is a condition of the visually impaired, in which hallucinations – or, more appropriately, visions – of geometric patterns, people, and objects appear within the visual field. Most people with CBS are older adults, a function of increased likelihood of visual deterioration in later life, but the condition can occur at any age. Diagnostic criteria vary among professions, but generally includes awareness that the visions are not real, the absence of other accompanying sensory hallucinations, and the absence of dementia or other neurological condition. The mechanism by which the phenomenon occurs is not well understood, although some speculation exists that it is akin to phantom limb syndrome. Although there has recently been increasing awareness about the condition, many in the fields of medicine, optometry, and social work have not heard of it. As front-line providers for clients, it is important that social workers be well informed about the condition to alleviate anxiety our clients may have about expressing their experiences with the condition.

People who saw dark, fat devils with sharp teeth and bulging eyes – Charles Bonnet syndrome: a case series

The Sri Lanka Journal of Psychiatry (SLJP) is a peer-reviewed, open access journal published bi annually by the Sri Lanka College of Psychiatrists.The Journal publishes original papers, brief reports including case reports and commentaries relevant to psychiatry and allied sciences. The Sri Lanka Journal of Psychiatry is committed to maintaining and conforming to the editorial and ethical standards recommended by the International Committee of Medical Journal Editors.Cover: Doorways: When one is ill, having somewhere to stay, where one is looked after, is usually a relief to the patient who is not well and his/her family. The picture shows the doorway to what remains of an ancient hospital in Mihintale, said to date back two millennia. Thilini Rajapakse, Department of Psychiatry, Faculty of Medicine, University of Peradeniya, Sri Lanka.

Glycerol Used for Controlling the Increased Intracranial Pressure-Related Charles Bonnet Syndrome

Glycerol Used for Controlling the Increased Intracranial Pressure-Related Charles Bonnet Syndrome

Charles Bonnet syndrome and periodic alternating nystagmus

To describe and discuss potential mechanisms for modulation of visual hallucinations by nystagmus. We present 2 patients with coexistent Charles Bonnet syndrome and periodic alternating nystagmus in the context of acquired visual loss. The combination has given rise to a rare phenomenon: visual hallucinations that move in a manner governed by the nystagmus, specifically by the direction and velocity of the slow phase. The perceived modulation of movement is selective for a surface in one case and a landscape in the other but not present for hallucinated individual objects and people separate from the hallucinated background visual scene. The collision of Charles Bonnet syndrome and periodic alternating nystagmus in these 2 patients has demonstrated that some visual hallucinations can be modulated by, or collaterally with, ocular movements. We propose 2 potential mechanisms based on ocular proprioceptive input from extraocular muscles projecting to either extrastriate processing of visual scene, or to higher-order visual cortical areas involved in analysis of motion signals across the whole visual field.

Screening for Charles Bonnet syndrome: Should the definition be reconsidered?

Purpose:Charles Bonnet syndrome (CBS) is a condition in which individuals with visual impairment (VI) and with no cognitive deficits experience visual hallucinations, typically with no other sensory hallucinations. Although few isolated case reports of CBS from India have been published, the prevalence for CBS in India is largely unknown. The primary aim of this study was to estimate CBS prevalence in patients with vision impairment visiting a tertiary eye care center.Methods:The study was conducted in two phases. In phase 1, patients with VI, age ≥40 years with presenting visual acuity worse than 20/63 were enrolled. In phase 2, patients with presenting visual acuity worse than 20/63 and/or with binocular visual field loss, age ≥18 years were recruited. A CBS survey was administered only to those who passed a screening test for cognition impairment.Results:A total of 218 patients were screened (phase 1 = 113 and phase 2 = 105). Two-hundred ten patients (mean age ± standard deviation = 49.2 ± 17.3 years, males = 139) were found eligible to complete the CBS survey. Fourteen patients were found to have visual hallucinations. In addition, three other patients had visual hallucinations with associated auditory input to the visual imagery. All patients had complete insight about their hallucinations.Conclusion:Depending on the inclusion criteria, we found the prevalence for CBS in patients with VI to vary between 6.7% to 8.1% (if including patients with auditory input). More investigation is needed to assess the associated role of other sensory inputs (e.g. auditory) with the visual imagery experienced in CBS.

Think sight loss, think Charles Bonnet syndrome.

Think sight loss, think Charles Bonnet syndrome.