FDG-PET Scanning Shows Distributed Changes in Cortical Activity Associated with Visual Hallucinations in Eye Disease.

Charles Bonnet syndrome (CBS) is characterized by recurrent or persistent complex visual hallucinations that occur in visually impaired individuals with intact cognition and no evidence of psychiatric illness. Patients usually retain insight into the unreal nature of their hallucinations.3,4 CBS is often misdiagnosed, and predominantly affects elderly patients with vision changes (e.g., age-related macular degeneration, glaucoma, and cataract). While many require only the assurance of the benign nature of the hallucinations, nonpharmacological and pharmacological interventions have been reported to be useful in the treatment of CBS. This case involves an 83-year-old female, with a two-year history of CBS, who presented to the clinic with worsening visual hallucinations over the past few months. She was starting to lose insight into her hallucinations secondary to her new diagnosis of dementia. Several pharmacological agents were explored to determine the most appropriate choice for our patient. Ultimately, this patient was started on donepezil (reported to be successful in a CBS case report), which helped improve her cognitive function. At future follow-up visits, her hallucinations improved and her cognitive function stabilized. Pharmacists should be aware of CBS and its treatment options to properly assist physicians in the medication-selection process to alleviate distress experienced by patients with CBS. In patients who may benefit from pharmacological treatment, physicians should weigh the risks and benefits of the different treatment options. Donepezil can be a favorable option in CBS patients with Alzheimer's type dementia.

Charles Bonnet, His Life, and His Syndrome

Background and objectivesIn Charles Bonnet Syndrome (CBS), visual hallucinations (VH) are experienced by people with sight loss due to eye disease or lesional damage to early visual pathways. The aim of this cross-sectional study was to investigate structural brain changes using magnetic resonance imaging (MRI) in CBS. MethodsSixteen CBS patients, 17 with eye disease but no VH, and 19 normally sighted people took part. Participants were imaged on a 3T scanner, with 1 mm resolution T1 weighted structural imaging, and diffusion tensor imaging with 64 diffusion directions. ResultsThe three groups were well matched for age, sex and cognitive scores (MMSE). The two eye disease groups were matched on visual acuity. Compared to the sighted controls, we found reduced grey matter in the occipital cortex in both eye disease groups. We also found reductions of fractional anisotropy and increased diffusivity in widespread areas, including occipital tracts, the corpus callosum, and the anterior thalamic radiation. We did not find any significant differences between the eye disease participants with VH versus without VH, but did observe a negative association between hippocampal volume and VH severity in the CBS group. DiscussionOur findings suggest that although there are cortical and subcortical effects associated with sight loss, structural changes do not explain the occurrence of VHs. CBS may relate instead to connectivity or excitability changes in brain networks linked to vision.

Back to table of contents Previous article Next article LettersFull AccessComplex Visual Hallucinations, Presumably Due to Subarachnoid Hemorrhage, Treated Successfully With RisperidoneDavid R. Spiegel, M.D., and Tim Petersen, M.D.David R. SpiegelSearch for more papers by this author, M.D., and Tim PetersenSearch for more papers by this author, M.D.Published Online:1 Jul 2011AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Visual hallucinations have several known nonpsychiatric etiologies and may be treated accordingly. We present, with interest, a case of a 56-year-old woman who developed complex visual hallucinations (CVH) status post-cerebrovascular accident (CVA) in her right middle cerebral artery (MCA) distribution. The hallucinations appear to have been successfully treated with risperidone.Case ReportOur patient is a 56-year-old, right-handed woman who presented with a large right-MCA aneurysm and several small left-MCA aneurysms. Her right-MCA aneurysm was successfully surgically repaired; no operative intervention was required for the left aneurysms. In addition to transient sensory and motor deficits, her son noted that after the surgery she would report seeing a woman and a black cat. She had no previous visual dysfunction, although, interestingly, her insight into the CVH improved even before medication treatment. Our patient returned 3 weeks later with new hemorrhages in her right-MCA area, with ischemia. No new surgeries were performed, and her condition stabilized after 1 week with appropriate medical intervention. During this admission, her CVHs persisted, and the authors were consulted because of her “behavioral disturbances.” There was no apparent association between the hallucinations and sleep or time of day. There were no reports of complex partial or generalized seizures. Per her son's report, she had no previous history of psychiatric illness, visual hallucinations, or auditory hallucinations. She had no history of substance abuse. She did not have fluctuating awareness or alertness; she had a Richmond Agitation And Sedation Scale score of 0 and 26/30 on the Mini-Mental State Exam. Risperidone 1.5 mg/day was prescribed in an attempt to treat her CVHs, which resolved within 7 days of initiating treatment.DiscussionAlthough the etiologies for CVHs have been reviewed elsewhere,1 our patient's phenomenology merits a discussion of delineating between Charles-Bonnet syndrome secondary to cortical stroke. With respect to the latter, CVHs may develop secondary to activated occipital cortical regions adjacent to stroke-damaged areas with associated visual field deficits,2 whereas stroke is generally regarded as a condition in which decreased afferent input that yields CVHs of a particular quality (straight ahead, colored, people, insight), although it also occurs in patients with visual-field defects and normal central acuity.3 The theories proposed to explain the etiology of both conditions fall into two categories. The “deafferentation” theory proposes that the lack of visual sensory input into the cortex causes spontaneous neuronal discharge leading to abnormal visual perceptions not unlike the phantom-limb syndrome. The second, “perceptual release” theory, is based on the premise that a normal sensory input inhibits irrelevant impulses from the conscious perception of images. Where there is a reduction in sensory input, the threshold to suppress irrelevant images cannot be achieved, and previously subconscious perceptions are “released” into consciousness, resulting in CVHs.4Risperidone has potent serotonin type-2 receptor antagonism and has been suggested by some authors to have efficacy in treatment of CVH through the blockade of such receptors in the visual cortex.5EVMS Dept. Of Psychiatry Norfolk, VA e-mail: [email protected]edu1. Manford M , Andermann F : Complex visual hallucinations: clinical and neurobiological insights. Brain 1998; 121:1819–1840Crossref, Medline, Google Scholar2. De Haan EH , Nys GM , van Zandvoort MJ , et al.: The physiological basis of visual hallucinations after damage to the primary visual cortex. NeuroReport 2007; 18:1177–1180Crossref, Medline, Google Scholar3. Schadlu AP , Schadlu R , Shepherd JB: Charles Bonnet syndrome: a review. Curr Opin Ophthalmol 2009; 20:219–222Crossref, Medline, Google Scholar4. Ashwin PT , Tsaloumas MD : Complex visual hallucinations (Charles Bonnet syndrome) in the hemianopic visual field following occipital infarction. J Neurol Sci 2007; 263(1–2):184–186Crossref, Medline, Google Scholar5. Howard RJ : Risperidone for visual hallucinations in the elderly. Eur Neuropsychopharmacol 1996; 6:28Crossref, Google Scholar FiguresReferencesCited byDetailsCited ByComplex Visual Hallucinations Associated With Parietal InfarctLokesh Shahani, M.D.1 January 2013 | The Journal of Neuropsychiatry and Clinical Neurosciences, Vol. 25, No. 1 Volume 23Issue 3 Summer 2011Pages E44-E44 Metrics PDF download History Published online 1 July 2011 Published in print 1 July 2011

Visual release hallucinations, also known as Charles Bonnet Syndrome, is a fairly common condition that goes unrecognized by more than 50% of all physicians. The syndrome is characterized by complex visual hallucinations in patients who have experienced partial or complete loss of vision. The hallucinations are exclusively visual in nature, usually temporary, and unrelated to mental and behavioral disorders. To make the diagnosis of CBS, patients must maintain some degree of insight into the unreal nature of their visual experiences, with an absence of delusions and hallucinations in other sensory modalities. This case report details a 93-year-old male with a history of mild cognitive impairment and bilateral cataracts who was found to have visual release hallucinations on hospital admission. The purpose of this case report is to increase awareness for Charles Bonnet Syndrome as a potential cause of visual hallucinations, particularly in elderly patients with a history of visual impairment.

P03-91 Charles Bonnet Syndrome and vitamin B12 deficiency: A case report

To the Editor: Complex visual hallucinations are usually a sign of acute psychopathology or gross cognitive impairment: they have been reported in patients with senile dementia, late paraphrenia, epilepsy, malignant hypertension, tumors, and adverse drug reactions.1 However, they may also occur in people with an ophthalmologic disorder: the Charles Bonnet Syndrome is a condition in which otherwise healthy individuals, i.e., without demonstrable psychopathology, experience vivid visual hallucinations.2–4 An analysis of the 64 cases reviewed by Schultz and Melzack reveals that the syndrome can occur at any age though it is more common in elderly people.5 During the last year, three very old patients, two nonagenarians and an 87-year-old woman, have come to our observation for “pleasant” complex visual hallucinations. A male, 93-years-old, with a university education, is in general good health and suffers from a mild degenerative joint disease, predominantly affecting his left hip; there is no history of major diseases. The content of his hallucinations is the representation of flowers (cyclamens’ fields, floods of wistarias and lilacs), Renaissance paintings, Chinese tapestries, and monumental squares. Hallucinatory symptoms have occurred with a frequency of once/twice a week during the last year. This patient is unimpaired in basic and instrumental activities of daily living. His mental status is good, with a MMSE score of 29/30. His Geriatric Depression Score is 7/30. There is no evidence of psychopathology either anamnestic or present. There is no family history of mental diseases. Neither epileptiform activity nor pathological slow activity was observed during basal registration or hyperventilation on EEG. A female, 92-years-old is a noblewoman with a secondary school education. She is only moderately hypertensive and is taking Enalapril 10 mg daily. She is in very good health and has no history of major somatic disease. The content of her visual hallucinations is characterized by flowers (chrysanthemums and orchids) and by tropical plants and trees, paintings (such as Botticelli's Spring), golden frames, and silk curtains floating in the wind. Hallucinatory symptoms have occurred three/four times a month during the last 3 months. Her MMSE score is 30/30. She does not suffer from depressive symptoms (GDS = 3/30). She is completely self-sufficient in both ADL and IADL functions. There is no evidence of psychopathology, either anamnestic or present. There is no family history of mental diseases. Neither epileptiform activity, nor pathological slow activity was observed during basal registration or hyperventilation on EEG. This is an 87-year-old female with primary education background. She suffers from a recent hip fracture caused by an accidental fall. Her MMSE is 25/30, and her GDS score is 10/30, indicating a mild degree of depression. Before the fracture she was completely self-sufficient in both ADL and IADL functions. The content of her hallucinations is poorer than the previous two cases, but equally pleasant: little geometrical and colorful flowers, floral tapestries, mountain brooks, clouds moving fast in the sky, and waves crushing on the shore. Hallucinatory symptoms occurred once/twice a week during the last 3 years. In this old lady, as well, there was no evidence of psychopathology, anamnestic or present, nor was there a family history of mental diseases. EEG showed a normal posterior alpha rhythm at 10 Hz, bilateral and synchronous, with short periods of theta rhythm (7 Hz), especially during hyperventilation. No epileptiform nor pathological slow activity was detected. The only common trait among the patients was the presence of ocular pathology. The 93-year-old man (Case 1) suffers from bilateral cataract and from the sequelae of a previous occlusion of the central retinal vein in his left eye. Visual acuity is 20/30 in the right eye, and the left eye is completely blind. The 92-year-old woman (Case 2) suffers from a cataract reducing her visual acuity to 18/30 in the right eye and from an opacity of the lens outside the left visual axis; presently, there is no indication for surgery. The visual evoked potential revealed a normal bilateral N75 response and a slightly delayed bilateral P100 response: right eye 113 msec, left eye 112 msec (laboratory range = 91–110 msec). Wave amplitude was normal in the left and reduced in the right eye. The second woman (Case 3) suffers from bilateral cataracts, reducing visual acuity in her left eye to 1.5/30 and in her right eye to 15/30, for which there is an indication for surgery extraction. The visual evoked potential showed a bilaterally delayed P100 (right eye 126 msec, left eye 125 msec) with a normal amplitude in the left and reduced in the right eye. In all three cases, the onset of the hallucinations was not associated with a detectable change of vision; hallucinations are stopped by focusing attention on a specific object. Results are consistent with earlier reports suggesting that these hallucinations are not due to psychopathology or compromised cognitive functioning. Our patients do not suffer from mental disorders, and their insight is good. Because there are not symptoms of mental diseases, it could be proposed that complex visual hallucinations represent ongoing neural activity in the visual system following eye damage. Sensory deprivation might produce patterns of nerve impulses that give rise to visual experiences.5 Complexity of hallucinations is probably attributable to the degree of schooling and culture of the patients: the richer the cultural background, the greater the complexity of psychic symptoms. Occurrence of visual hallucinations in old age is frequently described, but, in general, they are negative and unpleasant symptoms of mental diseases or the phenomenon of adverse drugs effects. In our three very old patients, emotional reactions to the visual hallucinations were positive and perceived as pleasant; the 92-year-old woman was only worried that the symptoms were prodromes of impending death (is it Heaven?). Literature observations suggest that the Charles Bonnet Syndrome is frequently overlooked or misdiagnosed by both ophthalmologists and psychiatrists. This may be true also for geriatricians.

Objective: Charles Bonnet Syndrome (CBS) consists of visual hallucinations occurring in patients with visual impairment. CBS is commonly overlooked by medical professionals and underreported by patients due to the underlying stigma attached to the presence of hallucinations. Lack of awareness regarding CBS among medical professionals may lead to failures in providing forewarning and education to patients with visual impairment. In patients who are not familiar with CBS, this might lead to a higher risk of distress, misattribution to cultural belief or mental illness, and the potential of developing psychosis, especially in elderly patients with other modalities of sensory deprivation. This case report aims to increase awareness and knowledge about CBS among clinicians for the proper management of patients.Method: This case illustrated a patient with worsening visual impairment who presented with typical CBS and later progressed to visual hallucination and persecutory delusionResults: Providing information on CBS and antipsychotics resulted in less distress and improved the patient’s visual hallucination and delusionConclusion: Education and reassurance play a role in the management of CBS. It is important for medical professionals to be aware of CBS and provide clear information for patients who are at risk to lower their distress.

The rarity of Charles Bonnet syndrome

<p>Charles Bonnet syndrome (CBS) was first described by Swiss philosopher Charles Bonnet in 1760. The defining characteristics of the syndrome include visual impairment, complex visual hallucinations, preservation of mental competence, and insight. The syndrome is an infrequently diagnosed but rather common cause of complex visual hallucinations. Its prevalence in patients with visual impairment varies from 10% to 15%, and it occurs most commonly in elderly people. The common conditions leading to the syndrome are age-related macular degeneration, glaucoma, and cataract.</p> <h4>ABOUT THE AUTHORS</h4> <p>Nahla Mahgoub, MD, is Staff Psychiatrist, Weill Cornell University-Westchester Division, New York. Michael Serby, MD, is Professor of Clinical Psychiatry, Beth Israel Medical Center, Albert Einstein College of Medicine, New York.</p> <p>Address correspondence to Dr. Mahgoub via e-mail at: <a href="mailto:NAMD06@aol.com">NAMD06@aol.com</a>.</p> <p>Dr. Mahgoub and Dr. Serby have disclosed no relevant financial relationships.</p> <h4>EDUCATIONAL OBJECTIVES</h4> <ol> <li>Identify the common clinical features of Charles Bonnet syndrome.</li> <li>Discuss potential treatments for Charles Bonnet syndrome.</li> <li>Name the type of disorder of which Charles Bonnet syndrome is comprised.</li> </ol>

The Charles Bonnet syndrome (CBS) is characterized by distinct visual hallucinations and ocularpathology causing visual impairment in patients with insight and the absence of psychiatric comorbidity. The number of reported cases of CBS is expanding as the population ages and the prevalence of vision disorders increases. Antipsychotic medications are often prescribed. However, their efficacy in CBS has been based on sketchy evidence. The use of serotonin selective reuptake inhibitor (SSRI) for CBS was anecdotally reported. We herein describe effectiveness of escitalopram in a series of patients suffering from CBS who were unresponsive to antipsychotic treatment. Eight consecutive patients suffering from CBS who did not respond to standard antipsychotic treatment were switched to escitalopram. CBS severity prior to escitalopram treatment was quantified using the Clinical Global Impression (CGI) scale and again after eight weeks of treatment. All had undergone brain CT and cognitive assessment. Brain CT imaging was normal except for an incidental finding of a small frontal meningioma in one patient. All had Mini-Mental Status Examination scores of ≥ 27/30. There were four men and four women, with a mean age of 81.7 ± 7.3 years. Previous antipsychotic treatment was mostly with risperidone, 1.0 to 3.0 mg/daily. Mean CGI-severity upon switching to escitalopram treatment was 5.7. This was significantly reduced to 1.8 (p < 0.001) after eight weeks of escitalopram treatment (mean dose: 11.8 mg/daily). There were no side effects, nor any adverse events were reported. This is the first case-series to show that SSRI is an effective and well-tolerated treatment for visual hallucinations associated with vision impairment such as in CBS.

Charles Bonnet Syndrome (CBS) is a rare clinical condition which is characterized by complex hallucinations in visually impaired patients. The pathophysiology of this disorder remains largely unknown, and there is still no proven treatment for this disease. In our study, we aimed to investigate the neural activity through Electroencephalography (EEG) power and evaluate the effect of rivastigmine in combination with alpha-lipoic acid on hallucination in two CBS patients with diabetic retinopathy. EEG data was recorded with standard routine EEG protocols for both patients in our electrophysiological research laboratory (REMER Clinical Electrophysiology and Neuromodulation Research and Application Laboratory) with Brain Vision Recorder (Brainproduct, Munich, Germany). All spectral analyses were processed by BrainVision Analyzer 2 (Brainproduct, Munich, Germany, 2.0.4 Version) in 128 Hz sample rates and the EEG recording and analysis was performed before the administration of rivastigmine (4.5 mg/daily and five patch daily for the first and second patients, respectively) in combination with alpha-lipoic acid (600 mg/daily) for both patients while they were not hallucinated during the time period recordings. Based on our measurement protocol, we have compared the patients in the study group with the three control subjects who were found to be normal except of visual disturbances secondary to significant diabetic retinopathy. Highest theta power values were found in right occipital and left temporo-parietal regions for first and second CBS patients, respectively. Additionally, power spectra were lower in two cases as compared to their control groups in the alpha band for all electrodes. We have also shown that acid rivastigmine in combination with alpha-lipoic exerted significant anti-hallucinatory efficiency. Our present findings could support the hypothesis that increased activation of specific areas in the source monitoring system plays an important role in the pathogenesis of CBS. In addition, rivastigmine in combination with alpha-lipoic acid could be a new valuable option for CBS patients.

Charles Bonnet syndrome is characterized by formed visual hallucinations in individuals with vision loss. It is reported that one in five older adults with vision loss suffer from Charles Bonnet syndrome and the suspected lack of awareness amongst family physicians may lead to misdiagnosis and inappropriate treatment. To assess Canadian family physicians' awareness of Charles Bonnet syndrome. We conducted a national perception and practices survey of family physicians across Canada to assess (i) the level of awareness of Charles Bonnet syndrome amongst family physicians; (ii) the frequency of family physicians' encounters with patients with visual hallucinations and (iii) management strategies and referral patterns for patients with Charles Bonnet syndrome presenting to family physicians. Four hundred and ninety-nine family physicians across Canada answered at least one question on the survey. 54.7% indicated they were not at all aware and 19.7% indicated they were slightly aware of Charles Bonnet syndrome. 72.8% of physicians had patients present with visual hallucinations once a year or less often. The frequency of patients seen in practice with visual hallucinations is significantly associated with awareness by physicians of Charles Bonnet syndrome (β = 0.501, t(388) = 5.59, P < 0.001). Of those who encountered patients with visual hallucinations, the majority of respondents (77.4%) provided counselling or education to the patient. There is a great lack of knowledge about Charles Bonnet syndrome among family physicians. Awareness of Charles Bonnet syndrome is critical to appropriate diagnosis, assessment and treatment of this condition and to reassure patients that they are not suffering from a mental disorder.

Charles Bonnet syndrome (CBS) refers to visual hallucinations that occur in individuals with preserved cognitive functions associated with visual impairment. This article reviews occurence of visual hallucinations in subjects with CBS by journals published in English in the Pubmed database in the period 1992-2018. Criteria for selection of appropriate papers were sufficient information and perspicuous view on pathogenesis, epidemiology, clinical presentation and treatment possibilities of CBS. Most commonly, visual hallucinations in patients with CBS are complex, repetitive and stereotyped. Such individuals have preserved insight that those percepts are not real, and there is an absence of secondary explanatory delusions and hallucinations within other modalities. Seeing as the aforementioned percepts do not share all the characteristics of hallucinations, it remains unresolved how they should be referred to. Terms as release hallucinations, one that is reflecting its underlying pathogenesis, or confabulatory hallucinatory experiences have been proposed. Moreover, CBS has also been referred to as phantom vision syndrome and may occur in any ophthalmological disease. It is not particularly connected with loss of function along any level of the visual pathway. Although this syndrome is mostly associated with age-related macular degeneration, glaucoma and cataract, it could be related to almost any other ophthalmological conditions. The incidence of CBS alongside with mostly other ocular pathology is rising as population is ageing. Nonetheless, CBS remains commonly underreported, under recognized and/or misrecognized. Albeit the treatment recommendations and guidelines are not yet fully established, it is important to raise awareness of this specific and distinct condition, which inevitably implicates many differential diagnostic deliberations.

Understanding the Charles Bonnet syndrome: An updated review.