International Journal of Psychiatry in Clinical Practice
ISSN: 1365-1501 (Print) 1471-1788 (Online) Journal homepage: http://www.tandfonline.com/loi/ijpc20
The Charles Bonnet syndrome: symptomatic relief with atypical neuroleptics: a case series Joe Johnson, Richard C Barnes To cite this article: Joe Johnson, Richard C Barnes (2001) The Charles Bonnet syndrome: symptomatic relief with atypical neuroleptics: a case series, International Journal of Psychiatry in Clinical Practice, 5:2, 141-144 To link to this article: http://dx.doi.org/10.1080/136515001300374894
Published online: 12 Jul 2009.
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Article views: 17
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ijpc20 Download by: [Deakin University Library]
Date: 14 November 2015, At: 05:14
Ó
2001 Martin Dunitz Ltd
International Journal of Psychiatry in Clinical Practice 2001 Volume 5 Pages 141 ± 144
141
The Charles Bonnet syndrome: symptomatic relief with atypical neuroleptics: a case series JOE JOHNSON AND RICHARD C BARNES
Downloaded by [Deakin University Library] at 05:14 14 November 2015
Ormskirk and District General Hospital, Ormskirk, L39 2AZ, UK
We describe a consecutive case series of five patients who all fulfil published criteria for Charles Bonnet syndrome. Four of the five patients showed a good response to the atypical antipsychotic sulpiride. The nature of the Charles Bonnet syndrome is briefly reviewed. (Int J Psych Clin Pract 2001; 5: 141 ± 144)
Correspondence Address Dr RC Barnes, Sir Douglas Crawford Unit, Mossley Hill Hospital, Park Avenue, Liverpool L18 8BU Tel: 0151 250 6154 Fax: 0151 729 0227
Received 6 April 2000; revised 12 July 2000; accepted for publication 13 July 2000.
Keywords Charles Bonnet
sulpiride
INTRODUCTION
CASE REPORTS
T
CASE 1
he Charles Bonnet syndrome,1 first described in 1796, is a syndrome of visual hallucination s which are formed, complex, stereotyped, persistent or repetitive, in the absence of delusions or other hallucination s. Insight is partially or fully retained.2 The hallucinatio ns are often detailed and elaborate and do not seem likely to be distressin g to the patient. They are frequently associated with visual pathology, though not invariably so.3 The prevalence of Charles Bonnet syndrome (CBS) is uncertain. Studies generally conclude it is much more common in the elderly and may be underdiagnose d if patients are reluctant to discuss their symptoms for fear of the stigma of mental illness. Visual hallucination s can occur in around 12 ± 13% of visuall y impaired populations4 ,5 and CBS in 1 ± 2% of psychogeria tric patients.6 Whilst CBS has been studied extensively, it is generally accepted to have a poor prognosis unless the visual pathology can be corrected.7 Drug treatments are generally viewed pessimistica lly.3 ,8 We report on a consecutive series of five patients who fulfill the criteria for CBS. The majority of these have shown a good symptomatic response to the atypical antipsychotic sulpiride.
atypical
An 87-year-old retired farmer presented with a 6-8-week history of complex visual hallucination s. He reported seeing floodwater running into the house from the farmland beyond, with workmen laying pipes in the field and also laying drainage pipes through the middle of his front room. He also saw horses walking through the back garden and into the house. Aside from these problems, he felt generally physicall y well and, while he was distressed about the state of his house, had no other abnormal psychopatholo gy. There was no past psychiatri c history, or family history of psychiatri c illness. He had some arthritis relating to his work and was taking simple analgesi a for this. Routine blood tests performed recently were within normal limits for his age. On formal cognitive testing, he was well orientated as to person, place and time and showed no evidence of short term or long-term memory problems. He was able to report recent events in the news accurately and described his symptoms clearly. He had a past history of senile macular degeneration and a previous suspected small stroke. He had been registered blind for 11 years. His visual acuity showed finger counting at 1 m on the right, and light perception only on the left.
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He was commenced on sulpiride 200 mg nocte. Review after 4 weeks revealed that the hallucination s had completely resolved within a week of medication starting, but he was suffering some mild side-effects , particularl y akathisia. This was successfull y treated with procyclidin e 5 mg once a day. Subsequently, he reported being a little drowsy and an attempt was made at reducing the sulpiride to 100 mg at night. Within a week of this, his visual hallucination s began to return and the family increased the dose back to 200 mg at night, which successfull y resolved the symptoms.
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CASE 2 An 81-year-old woman, living alone, with a history of visual hallucination s of young men walking around in her house, she had good insight into the abnormality of her experiences , and on the whole found them quite comforting as company. Occasionall y she felt distressed, as she was embarrasse d when they watched her bathing. She had several years’ history of senile macular degeneration . There was no past psychiatric history, but her mother had become `senile’ in later life. She generally enjoyed fair physical health, although she was a little frail, commensurat e with her age. Routine blood tests revealed no gross abnormality. Visual acuity testing revealed light perception only on the right, 6/18 on left. Formal cognitive testing revealed no gross deficits; she was well orientated as to place and time and had no clinically significa nt memory deficits. A CT scan showed a left-sided cerebella r infarct with scattered lacunar infarcts, but no evidence of signific ant cortical atrophy. She was commenced on sulpirid e 100 mg at night but was unable to tolerate this due to excessive sedation. Subsequently, she was tried on risperidon e 0.5 mg nocte, steadily increased to 1 mg b.d., but with no therapeutic effect. She still has her symptoms, but is quite philosophica l about her experiences .
CASE 3 An 87-year-old man, initially seen on the medical ward with a relativel y acute onset of visual hallucination s over 2 ± 3 weeks, complaine d of seeing strands and `tentacles of skin’ coming out of his head and hands and hanging down over his eyes. He also complained of seeing dirt on his hands. He had good insight into the abnormalit y of his experiences and was rather upset that he had been asked to see a psychiatrist . Staff and family frequently noticed him pulling at these strands to try to remove them. He was aware that his experiences were abnormal and was puzzled that he was able to see them just as well with his blind eye as with his better one. There was no past psychiatric history or family history. His physical health was remarkably good, given his age, aside from some angina, which had been the cause of his medical admission in the first instance. Routine blood tests were unremarkabl e and he was taking antianginals and anti-hypertens ives.
A CT scan showed mild vascular changes. Formal cognitive testing scored 28/30 on a Mini Mental State Examination. His medical history showed visual problems related to senile macular degeneration and cataracts, which he was reluctant to have operated on. Visual acuity was 6/9 on the right, blind in the left eye. The patient was commenced on sulpiride 100 mg at night, subsequently increased to 200 mg at night. All visual hallucinatio ns completely resolved, much to the relief of both patient and family. Subsequentl y, he was discharge d from follow-up, as he was very distressed by the stigma of seeing a psychiatrist .
CASE 4 An 89-year-old man, living alone in sheltered accommodation, suffere d an acute onset of florid visual hallucination s on gazing through the window of his front room: seeing people walking by, setting up tents and camping on his lawn and also cathedrals, churches and trees. Generally, he found the experiences rather bothering and sometimes got himself ready to walk to church, since so many other people seemed to be going. He was in generally good physical health, although his mobility was quite severely limited by arthritis. Blood tests performed by his general practitioner were within acceptable limits. There was a past history of `depression ’ though he had never been referred to a psychiatrist . His mental state was euthymic when we saw him. Cognitive testing showed him to be fully orientated as to person, place and time, with no abnormalitie s of short-term memory; he was easily able to discuss recent events in the news. He had been registered blind for several years (cause not clear). Visual acuity showed finger counting at 1 m on the right and light perception only on the left. He was commenced on sulpiride 100 mg nocte, which he took for only a week and a half, with complete resolution of symptoms. He was seen at follow-ups at 1 month and 4 months, with no return of visual hallucinations. At 12 months, he was well and neuroleptic-f ree.
CASE 5 A 72-year-old man suffered from the onset of visual hallucinatio ns over a period of a week after he had been discharge d home following routine hernia surgery 10 days previously . There was no record of any post-operative cognitive problems and the surgery, performed under local anaesthetic, had been uneventful. He complained of seeing birds and animals flying and running around in the house, particularl y upstairs. He was not overly bothered about them, apart from concerns for their health, as he feared he might step on them. He had a history of pseudoparkinsonis m of vascular origin, but was otherwise physicall y reasonabl y well. Routine blood tests revealed a mildly elevated plasma viscosity and low thyroid-stimu lat-
Downloaded by [Deakin University Library] at 05:14 14 November 2015
Charles Bonnet syndrome
ing hormone levels. Both these abnormalities subsequentl y became normal without treatment. Although he said he had never got over his wife’s death 18 months previously , there was no previous psychiatri c history. He performed well on formal cognitive testing but there was evidence of some difficultie s with self-care, not just related to his poor vision and poor mobility. A CT scan showed a moderate degree of vascular changes and atrophy affecting the frontal lobes, though without focal lesions. Visual acuity showed finger counting at 1 m on the left, 6/60 on the right. He was commenced on sulpiride 100 mg nocte, subsequently slowly increased to 200 mg nocte and 200 mg b.d. He experience d some improvement in symptoms, in that he found he saw the animals less often and said that they were less bothersome to him when he did see them. Unfortunately , he was unable to continue on sulpiride due to worsening of his pseudo-parki nsonism, which necessitated psychogeriat ric admission. His visual hallucination s remitted following admission, and he was discharge d free from neuroleptics , but not experiencin g visual hallucination s.
DISCUSSION CBS does not fit exactly into current classificatio n systems. The nearest ICD-10 diagnosis2 5 would be Organic Hallucinosi s ± F06.0. There has been disagreement over the exact diagnostic criteria for CBS. Gold and Rabins’ criteria emphasiz e the formed and persistent nature of the hallucinatio ns, the maintenance of some degree of insight and the absence of other major psychopathol ogy.2 Teunisse and colleagues , in their large prospective study into the prevalence of CBS, applied similar criteria.9 Ball has criticized these definitions as they fail to mention the degree of cognitive impairment , and suggests the term ``Charles Bonnet plus’’ to describe cognitive impairment in CBS.1 0 Similarly, Berrios and Brook have suggested that CBS is an over-used term, and prefer it to be reserved only for those who exhibit no cognitive or visual impairment .1 1 In a series of patients, Pliskin and colleagues found that some degree of cognitive deficit in CBS was very common.1 2 They proposed CBS as an early marker for dementia, an observation that has been noted before.1 3 ,1 4 Of the five patients we report above, two (patients 1 and 5) have subsequently developed cognitive impairment sufficien t to warrant a diagnosi s of dementia. Patients 2, 3 and 5 all had signs of intracerebra l vascular disease, although at presentation this was not causing significant cognitive effects. This is a rather higher rate than that found in other studies,1 2 but may reflect the small size of our sample. There can also be considerabl e variation between radiologist s in reporting mild ischaemic changes. Complex visual hallucinatio ns can arise in a variety of conditions related to both ocular and central pathology.
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Ophthalmologically, macular degeneratio n seems particularly associated with such hallucination s,5 although many pathological conditions of the optic nerve or retina can cause spontaneous hallucination s.1 5 A large number of centrally acting drugs may also have this potential.1 6 Visual hallucinatio ns are recognized by many clinicians as being highly suggestive of organic pathology and, particularly in the elderly, the possible list of causes is vast. We feel that the fact that our patients do not necessaril y fulfil the stricter criteria for CBS does not change the significanc e of our findings. For all of these patients, the consistent and distressin g symptoms were prominent visual hallucinatio ns, and for four of them sulpiride was found to cause a significan t reduction in these symptoms. While sulpiride has been available for a considerabl e time, it has not been as widely used as other more conventional neuroleptics . It has, however, found some popularity among Old Age Psychiatrist s. This is probably due to its relativel y good side-effec t profile and tolerability ± a result of its fairly specific action. Its use in the above cases reflects one author’s (RB) preference for it in elderly patients for these reasons. Sulpiride is a substituted benzamide chemically related to metoclopramid e and trimethyl-benzomide, with a specific affinity for dopamine receptors. Its pharmacologi cal profile suggests that it is an atypical antipsychotic .1 7 It preferentiall y binds to D2 receptors and has little adrenergic , cholinergic , gamma-amino -buteric acidergic, histaminergi c or serotenergic affinity.1 8 It has been shown to be partially selective towards negative symptoms of schizophren ia at low doses and to have overt activity against positive symptoms at higher doses. Placebocontrolled double-blind studies have shown it to be an effective antipsychotic , comparable with several typical neuroleptics . It has a comparatively low incidence of extrapyramid al symptoms and a low toxicity profile on overdose. It may have a particular propensit y for causing hyperprolac tinaemia.1 9 Previous studies of CBS have generally reported unsatisfactor y results with medication. Siatkowski and colleagues 2 0 claimed some success using haloperidol , but there has been little enthusiasm for other conventional neuroleptics . There is more positive evidence concerning the use of carbamazepin e. Hosty reported a good clinical response to 200 mg daily, with a sustained remission in symptoms over several months.2 1 Bhatia and colleagues also found carbamazepin e 300 mg daily of benefit in a patient resistant to antipsychotic , antidepressan t and anxiolytic medications.2 2 More recently, Batra et al have successfull y treated the hallucination s using the atypical neuroleptic melparone, a low-potency neuroleptic from the butyrophenon e group.2 3 The origins of the visual hallucination s in CBS are unclear, but attention has tended to focus on abnormalities of either input or central processing . Reduction in visual stimulation underlie s the input deficit, although there may also be a contribution from social isolation or subthreshol d
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delirium. The central deficits are more complex. Explanations include: a loss of higher centre suppressio n of perceptual traces; a deafferentati on-state, where loss of input triggers intracerebra l perceptions; cortical irritability, with spontaneous discharge from visual association areas; and epileptiform activity in the visual cortex. There may also be a contribution from the higher psychic functions, including wishes, conflicts and past memories (see Fernandez et al7 for a review). The possibilit y of epileptiform activity playing a part may explain the positive results found with carbamazepi ne. It has been suggested that sulpiride may present a lower risk in patients with epilepsy, 2 4 which lends further credence to its choice in these patients. An increasing number of atypical antipsychotic drugs are becoming available, offering apparent benefits in efficacy as well as better side-effec t profiles . The above
series, in keeping with the work with melparone, suggests that they may also have a role in disorders which previousl y have not responded to conventional drug therapies. Further research is indicated if patients with this underdiagno sed and distressin g condition are going to be helped.
KEY POINTS
·
This case series suggests that sulpiride and possibly other atypical antipsychotics may be useful in the treatment of Charles Bonnet syndrome.
REFERENCES 1. Morisier GD (1938) Les hallucinations. Rev Otoneurophthalmol 16: 244 ± 352. 2. Gold K, Rabins PV (1989) Isolated visual hallucinations and the Charles Bonnet syndrome: A review of the literature and presentation of six cases. Compr Psychiatry 30: 90 ± 8. 3. Teunisse RJ, Zitram FG, Raes DCM (1994) Clinical evaluation of 14 patients with the Charles Bonnet syndrome (isolated visual hallucinations). Compr Psychiatry 35: 70 ± 5. 4. Brown GC, Murphy RP (1992) Visual symptoms associated with choroidal neovascularization: Photopsias and the Charles Bonnet syndrome. Arch Ophthalmol 110: 1251 ± 56. 5. Holroyd S, Rabins PV, Finkelstein D et al (1992) Visual hallucinations in patients with macular degeneration. Am J Psychiatry 149: 1701 ± 6. 6. Podoll K, Osterheider M, Noth J (1989) Das Charles Bonnet syndrom. Fortschr Neurol Psychiatr 57: 43 ± 60. 7. Fernandez A, Lichtshein G, Vieweg WVR (1997) The Charles Bonnet syndrome: A review. J Nerv Ment Dis 185: 195 ± 200. 8. Norton-Wilson L, Munir M (1987) Visual perceptual disorders resembling the Charles Bonnet syndrome. A study of 434 consecutive patients referred to a psychogeriatric unit. Fam Pract 4: 27 ± 31. 9. Teunisse RJ, Cruysberg JRM, Verbeek A, Zitram FG (1995) The Charles Bonnet syndrome. A large prospective study in the Netherlands. Br J Psychiatry 166: 254 ± 7. 10. Ball C (1995) Charles Bonnet syndrome (Letter). Br J Psychiatry 166: 677 ± 8. 11. Berrios GE, Brook P (1982) The Charles Bonnet syndrome and the problem of visual perceptual disorders in the elderly. Age Ageing 11: 17 ± 23. 12. Pliskin NH, Kiolbasa TA, Towle VL et al (1996) Charles Bonnet syndrome: An early marker for dementia. J Am Geriatr Soc 44: 1055 ± 61.
13. Crystal HA, Wolfson LI, Ewing S (1988) Visual hallucinations as the first symptom of Alzheimer’s disease (Letter). Am J Psychiatry 145: 10. 14. Haddad PM, Benbow SM (1992) Visual hallucinations as the presenting symptom of senile dementia. Br J Psychiatry 161: 263 ± 5. 15. Duane TD (1995) Duane’s Clinical Ophthalmology (rev. edn) Vol. 2; Ch. 7, 17 ± 9. Lippincott, Philadelphia. 16. Cummings JL, Miller BL (1987) Visual hallucinations ± Clinical occurrence and use in differential diagnosis. West J Med 146: 46 ± 51. 17. Caley CF, Weber SS (1995) Sulpiride: An antipsychotic with selective dopaminergic antagonist properties. Ann Pharmacotherapy 29: 152 ± 60. 18. Jenner P, Marsden CD (1981) Substituted benzomine drugs as selective neuroleptic agents. Neuropharmacology 20: 1285 ± 93. 19. Wagstaffe AJ, Fitton A, Benfield P (1994) Sulpiride: A review of its pharmaco-dynamic and pharmaco-kinetic properties and therapeutic efficacy in schizophrenia. CNS Drug 4: 313 ± 33. 20. Siatkowski RM, Zimmer B, Rosenberg PR (1990) The Charles Bonnet syndrome: Visual perceptive functioning in sensory deprivation. J Clin Neuroophthalmol 10: 215 ± 8. 21. Hosty G (1990) Charles Bonnet syndrome: A description of two cases. Acta Psychiatr Scand 82: 316 ± 7. 22. Bhatia MS, Khastgir U, Malik SC (1992) Charles Bonnet Syndrome. Br J Psych 161: 409 ± 10. 23. Batra A, Bartels M, Wormstall H (1997) Therapeutic options in Charles Bonnet syndrome. Acta Psychiatr Scand 96: 129 ± 33. 24. Bazire S (1999) Psychotropic Drug Directory, 159 ± 60. Quay Books Division, Mark Allen Publishing, Salisbury. 25. World Health Organization (1992) International Classification of Diseases (ICD-10). WHO, Geneva.
ISSN: 1365-1501 (Print) 1471-1788 (Online) Journal homepage: http://www.tandfonline.com/loi/ijpc20
The Charles Bonnet syndrome: symptomatic relief with atypical neuroleptics: a case series Joe Johnson, Richard C Barnes To cite this article: Joe Johnson, Richard C Barnes (2001) The Charles Bonnet syndrome: symptomatic relief with atypical neuroleptics: a case series, International Journal of Psychiatry in Clinical Practice, 5:2, 141-144 To link to this article: http://dx.doi.org/10.1080/136515001300374894
Published online: 12 Jul 2009.
Submit your article to this journal
Article views: 17
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ijpc20 Download by: [Deakin University Library]
Date: 14 November 2015, At: 05:14
Ó
2001 Martin Dunitz Ltd
International Journal of Psychiatry in Clinical Practice 2001 Volume 5 Pages 141 ± 144
141
The Charles Bonnet syndrome: symptomatic relief with atypical neuroleptics: a case series JOE JOHNSON AND RICHARD C BARNES
Downloaded by [Deakin University Library] at 05:14 14 November 2015
Ormskirk and District General Hospital, Ormskirk, L39 2AZ, UK
We describe a consecutive case series of five patients who all fulfil published criteria for Charles Bonnet syndrome. Four of the five patients showed a good response to the atypical antipsychotic sulpiride. The nature of the Charles Bonnet syndrome is briefly reviewed. (Int J Psych Clin Pract 2001; 5: 141 ± 144)
Correspondence Address Dr RC Barnes, Sir Douglas Crawford Unit, Mossley Hill Hospital, Park Avenue, Liverpool L18 8BU Tel: 0151 250 6154 Fax: 0151 729 0227
Received 6 April 2000; revised 12 July 2000; accepted for publication 13 July 2000.
Keywords Charles Bonnet
sulpiride
INTRODUCTION
CASE REPORTS
T
CASE 1
he Charles Bonnet syndrome,1 first described in 1796, is a syndrome of visual hallucination s which are formed, complex, stereotyped, persistent or repetitive, in the absence of delusions or other hallucination s. Insight is partially or fully retained.2 The hallucinatio ns are often detailed and elaborate and do not seem likely to be distressin g to the patient. They are frequently associated with visual pathology, though not invariably so.3 The prevalence of Charles Bonnet syndrome (CBS) is uncertain. Studies generally conclude it is much more common in the elderly and may be underdiagnose d if patients are reluctant to discuss their symptoms for fear of the stigma of mental illness. Visual hallucination s can occur in around 12 ± 13% of visuall y impaired populations4 ,5 and CBS in 1 ± 2% of psychogeria tric patients.6 Whilst CBS has been studied extensively, it is generally accepted to have a poor prognosis unless the visual pathology can be corrected.7 Drug treatments are generally viewed pessimistica lly.3 ,8 We report on a consecutive series of five patients who fulfill the criteria for CBS. The majority of these have shown a good symptomatic response to the atypical antipsychotic sulpiride.
atypical
An 87-year-old retired farmer presented with a 6-8-week history of complex visual hallucination s. He reported seeing floodwater running into the house from the farmland beyond, with workmen laying pipes in the field and also laying drainage pipes through the middle of his front room. He also saw horses walking through the back garden and into the house. Aside from these problems, he felt generally physicall y well and, while he was distressed about the state of his house, had no other abnormal psychopatholo gy. There was no past psychiatri c history, or family history of psychiatri c illness. He had some arthritis relating to his work and was taking simple analgesi a for this. Routine blood tests performed recently were within normal limits for his age. On formal cognitive testing, he was well orientated as to person, place and time and showed no evidence of short term or long-term memory problems. He was able to report recent events in the news accurately and described his symptoms clearly. He had a past history of senile macular degeneration and a previous suspected small stroke. He had been registered blind for 11 years. His visual acuity showed finger counting at 1 m on the right, and light perception only on the left.
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J Johnson and RC Barnes
He was commenced on sulpiride 200 mg nocte. Review after 4 weeks revealed that the hallucination s had completely resolved within a week of medication starting, but he was suffering some mild side-effects , particularl y akathisia. This was successfull y treated with procyclidin e 5 mg once a day. Subsequently, he reported being a little drowsy and an attempt was made at reducing the sulpiride to 100 mg at night. Within a week of this, his visual hallucination s began to return and the family increased the dose back to 200 mg at night, which successfull y resolved the symptoms.
Downloaded by [Deakin University Library] at 05:14 14 November 2015
CASE 2 An 81-year-old woman, living alone, with a history of visual hallucination s of young men walking around in her house, she had good insight into the abnormality of her experiences , and on the whole found them quite comforting as company. Occasionall y she felt distressed, as she was embarrasse d when they watched her bathing. She had several years’ history of senile macular degeneration . There was no past psychiatric history, but her mother had become `senile’ in later life. She generally enjoyed fair physical health, although she was a little frail, commensurat e with her age. Routine blood tests revealed no gross abnormality. Visual acuity testing revealed light perception only on the right, 6/18 on left. Formal cognitive testing revealed no gross deficits; she was well orientated as to place and time and had no clinically significa nt memory deficits. A CT scan showed a left-sided cerebella r infarct with scattered lacunar infarcts, but no evidence of signific ant cortical atrophy. She was commenced on sulpirid e 100 mg at night but was unable to tolerate this due to excessive sedation. Subsequently, she was tried on risperidon e 0.5 mg nocte, steadily increased to 1 mg b.d., but with no therapeutic effect. She still has her symptoms, but is quite philosophica l about her experiences .
CASE 3 An 87-year-old man, initially seen on the medical ward with a relativel y acute onset of visual hallucination s over 2 ± 3 weeks, complaine d of seeing strands and `tentacles of skin’ coming out of his head and hands and hanging down over his eyes. He also complained of seeing dirt on his hands. He had good insight into the abnormalit y of his experiences and was rather upset that he had been asked to see a psychiatrist . Staff and family frequently noticed him pulling at these strands to try to remove them. He was aware that his experiences were abnormal and was puzzled that he was able to see them just as well with his blind eye as with his better one. There was no past psychiatric history or family history. His physical health was remarkably good, given his age, aside from some angina, which had been the cause of his medical admission in the first instance. Routine blood tests were unremarkabl e and he was taking antianginals and anti-hypertens ives.
A CT scan showed mild vascular changes. Formal cognitive testing scored 28/30 on a Mini Mental State Examination. His medical history showed visual problems related to senile macular degeneration and cataracts, which he was reluctant to have operated on. Visual acuity was 6/9 on the right, blind in the left eye. The patient was commenced on sulpiride 100 mg at night, subsequently increased to 200 mg at night. All visual hallucinatio ns completely resolved, much to the relief of both patient and family. Subsequentl y, he was discharge d from follow-up, as he was very distressed by the stigma of seeing a psychiatrist .
CASE 4 An 89-year-old man, living alone in sheltered accommodation, suffere d an acute onset of florid visual hallucination s on gazing through the window of his front room: seeing people walking by, setting up tents and camping on his lawn and also cathedrals, churches and trees. Generally, he found the experiences rather bothering and sometimes got himself ready to walk to church, since so many other people seemed to be going. He was in generally good physical health, although his mobility was quite severely limited by arthritis. Blood tests performed by his general practitioner were within acceptable limits. There was a past history of `depression ’ though he had never been referred to a psychiatrist . His mental state was euthymic when we saw him. Cognitive testing showed him to be fully orientated as to person, place and time, with no abnormalitie s of short-term memory; he was easily able to discuss recent events in the news. He had been registered blind for several years (cause not clear). Visual acuity showed finger counting at 1 m on the right and light perception only on the left. He was commenced on sulpiride 100 mg nocte, which he took for only a week and a half, with complete resolution of symptoms. He was seen at follow-ups at 1 month and 4 months, with no return of visual hallucinations. At 12 months, he was well and neuroleptic-f ree.
CASE 5 A 72-year-old man suffered from the onset of visual hallucinatio ns over a period of a week after he had been discharge d home following routine hernia surgery 10 days previously . There was no record of any post-operative cognitive problems and the surgery, performed under local anaesthetic, had been uneventful. He complained of seeing birds and animals flying and running around in the house, particularl y upstairs. He was not overly bothered about them, apart from concerns for their health, as he feared he might step on them. He had a history of pseudoparkinsonis m of vascular origin, but was otherwise physicall y reasonabl y well. Routine blood tests revealed a mildly elevated plasma viscosity and low thyroid-stimu lat-
Downloaded by [Deakin University Library] at 05:14 14 November 2015
Charles Bonnet syndrome
ing hormone levels. Both these abnormalities subsequentl y became normal without treatment. Although he said he had never got over his wife’s death 18 months previously , there was no previous psychiatri c history. He performed well on formal cognitive testing but there was evidence of some difficultie s with self-care, not just related to his poor vision and poor mobility. A CT scan showed a moderate degree of vascular changes and atrophy affecting the frontal lobes, though without focal lesions. Visual acuity showed finger counting at 1 m on the left, 6/60 on the right. He was commenced on sulpiride 100 mg nocte, subsequently slowly increased to 200 mg nocte and 200 mg b.d. He experience d some improvement in symptoms, in that he found he saw the animals less often and said that they were less bothersome to him when he did see them. Unfortunately , he was unable to continue on sulpiride due to worsening of his pseudo-parki nsonism, which necessitated psychogeriat ric admission. His visual hallucination s remitted following admission, and he was discharge d free from neuroleptics , but not experiencin g visual hallucination s.
DISCUSSION CBS does not fit exactly into current classificatio n systems. The nearest ICD-10 diagnosis2 5 would be Organic Hallucinosi s ± F06.0. There has been disagreement over the exact diagnostic criteria for CBS. Gold and Rabins’ criteria emphasiz e the formed and persistent nature of the hallucinatio ns, the maintenance of some degree of insight and the absence of other major psychopathol ogy.2 Teunisse and colleagues , in their large prospective study into the prevalence of CBS, applied similar criteria.9 Ball has criticized these definitions as they fail to mention the degree of cognitive impairment , and suggests the term ``Charles Bonnet plus’’ to describe cognitive impairment in CBS.1 0 Similarly, Berrios and Brook have suggested that CBS is an over-used term, and prefer it to be reserved only for those who exhibit no cognitive or visual impairment .1 1 In a series of patients, Pliskin and colleagues found that some degree of cognitive deficit in CBS was very common.1 2 They proposed CBS as an early marker for dementia, an observation that has been noted before.1 3 ,1 4 Of the five patients we report above, two (patients 1 and 5) have subsequently developed cognitive impairment sufficien t to warrant a diagnosi s of dementia. Patients 2, 3 and 5 all had signs of intracerebra l vascular disease, although at presentation this was not causing significant cognitive effects. This is a rather higher rate than that found in other studies,1 2 but may reflect the small size of our sample. There can also be considerabl e variation between radiologist s in reporting mild ischaemic changes. Complex visual hallucinatio ns can arise in a variety of conditions related to both ocular and central pathology.
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Ophthalmologically, macular degeneratio n seems particularly associated with such hallucination s,5 although many pathological conditions of the optic nerve or retina can cause spontaneous hallucination s.1 5 A large number of centrally acting drugs may also have this potential.1 6 Visual hallucinatio ns are recognized by many clinicians as being highly suggestive of organic pathology and, particularly in the elderly, the possible list of causes is vast. We feel that the fact that our patients do not necessaril y fulfil the stricter criteria for CBS does not change the significanc e of our findings. For all of these patients, the consistent and distressin g symptoms were prominent visual hallucinatio ns, and for four of them sulpiride was found to cause a significan t reduction in these symptoms. While sulpiride has been available for a considerabl e time, it has not been as widely used as other more conventional neuroleptics . It has, however, found some popularity among Old Age Psychiatrist s. This is probably due to its relativel y good side-effec t profile and tolerability ± a result of its fairly specific action. Its use in the above cases reflects one author’s (RB) preference for it in elderly patients for these reasons. Sulpiride is a substituted benzamide chemically related to metoclopramid e and trimethyl-benzomide, with a specific affinity for dopamine receptors. Its pharmacologi cal profile suggests that it is an atypical antipsychotic .1 7 It preferentiall y binds to D2 receptors and has little adrenergic , cholinergic , gamma-amino -buteric acidergic, histaminergi c or serotenergic affinity.1 8 It has been shown to be partially selective towards negative symptoms of schizophren ia at low doses and to have overt activity against positive symptoms at higher doses. Placebocontrolled double-blind studies have shown it to be an effective antipsychotic , comparable with several typical neuroleptics . It has a comparatively low incidence of extrapyramid al symptoms and a low toxicity profile on overdose. It may have a particular propensit y for causing hyperprolac tinaemia.1 9 Previous studies of CBS have generally reported unsatisfactor y results with medication. Siatkowski and colleagues 2 0 claimed some success using haloperidol , but there has been little enthusiasm for other conventional neuroleptics . There is more positive evidence concerning the use of carbamazepin e. Hosty reported a good clinical response to 200 mg daily, with a sustained remission in symptoms over several months.2 1 Bhatia and colleagues also found carbamazepin e 300 mg daily of benefit in a patient resistant to antipsychotic , antidepressan t and anxiolytic medications.2 2 More recently, Batra et al have successfull y treated the hallucination s using the atypical neuroleptic melparone, a low-potency neuroleptic from the butyrophenon e group.2 3 The origins of the visual hallucination s in CBS are unclear, but attention has tended to focus on abnormalities of either input or central processing . Reduction in visual stimulation underlie s the input deficit, although there may also be a contribution from social isolation or subthreshol d
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delirium. The central deficits are more complex. Explanations include: a loss of higher centre suppressio n of perceptual traces; a deafferentati on-state, where loss of input triggers intracerebra l perceptions; cortical irritability, with spontaneous discharge from visual association areas; and epileptiform activity in the visual cortex. There may also be a contribution from the higher psychic functions, including wishes, conflicts and past memories (see Fernandez et al7 for a review). The possibilit y of epileptiform activity playing a part may explain the positive results found with carbamazepi ne. It has been suggested that sulpiride may present a lower risk in patients with epilepsy, 2 4 which lends further credence to its choice in these patients. An increasing number of atypical antipsychotic drugs are becoming available, offering apparent benefits in efficacy as well as better side-effec t profiles . The above
series, in keeping with the work with melparone, suggests that they may also have a role in disorders which previousl y have not responded to conventional drug therapies. Further research is indicated if patients with this underdiagno sed and distressin g condition are going to be helped.
KEY POINTS
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This case series suggests that sulpiride and possibly other atypical antipsychotics may be useful in the treatment of Charles Bonnet syndrome.
REFERENCES 1. Morisier GD (1938) Les hallucinations. Rev Otoneurophthalmol 16: 244 ± 352. 2. Gold K, Rabins PV (1989) Isolated visual hallucinations and the Charles Bonnet syndrome: A review of the literature and presentation of six cases. Compr Psychiatry 30: 90 ± 8. 3. Teunisse RJ, Zitram FG, Raes DCM (1994) Clinical evaluation of 14 patients with the Charles Bonnet syndrome (isolated visual hallucinations). Compr Psychiatry 35: 70 ± 5. 4. Brown GC, Murphy RP (1992) Visual symptoms associated with choroidal neovascularization: Photopsias and the Charles Bonnet syndrome. Arch Ophthalmol 110: 1251 ± 56. 5. Holroyd S, Rabins PV, Finkelstein D et al (1992) Visual hallucinations in patients with macular degeneration. Am J Psychiatry 149: 1701 ± 6. 6. Podoll K, Osterheider M, Noth J (1989) Das Charles Bonnet syndrom. Fortschr Neurol Psychiatr 57: 43 ± 60. 7. Fernandez A, Lichtshein G, Vieweg WVR (1997) The Charles Bonnet syndrome: A review. J Nerv Ment Dis 185: 195 ± 200. 8. Norton-Wilson L, Munir M (1987) Visual perceptual disorders resembling the Charles Bonnet syndrome. A study of 434 consecutive patients referred to a psychogeriatric unit. Fam Pract 4: 27 ± 31. 9. Teunisse RJ, Cruysberg JRM, Verbeek A, Zitram FG (1995) The Charles Bonnet syndrome. A large prospective study in the Netherlands. Br J Psychiatry 166: 254 ± 7. 10. Ball C (1995) Charles Bonnet syndrome (Letter). Br J Psychiatry 166: 677 ± 8. 11. Berrios GE, Brook P (1982) The Charles Bonnet syndrome and the problem of visual perceptual disorders in the elderly. Age Ageing 11: 17 ± 23. 12. Pliskin NH, Kiolbasa TA, Towle VL et al (1996) Charles Bonnet syndrome: An early marker for dementia. J Am Geriatr Soc 44: 1055 ± 61.
13. Crystal HA, Wolfson LI, Ewing S (1988) Visual hallucinations as the first symptom of Alzheimer’s disease (Letter). Am J Psychiatry 145: 10. 14. Haddad PM, Benbow SM (1992) Visual hallucinations as the presenting symptom of senile dementia. Br J Psychiatry 161: 263 ± 5. 15. Duane TD (1995) Duane’s Clinical Ophthalmology (rev. edn) Vol. 2; Ch. 7, 17 ± 9. Lippincott, Philadelphia. 16. Cummings JL, Miller BL (1987) Visual hallucinations ± Clinical occurrence and use in differential diagnosis. West J Med 146: 46 ± 51. 17. Caley CF, Weber SS (1995) Sulpiride: An antipsychotic with selective dopaminergic antagonist properties. Ann Pharmacotherapy 29: 152 ± 60. 18. Jenner P, Marsden CD (1981) Substituted benzomine drugs as selective neuroleptic agents. Neuropharmacology 20: 1285 ± 93. 19. Wagstaffe AJ, Fitton A, Benfield P (1994) Sulpiride: A review of its pharmaco-dynamic and pharmaco-kinetic properties and therapeutic efficacy in schizophrenia. CNS Drug 4: 313 ± 33. 20. Siatkowski RM, Zimmer B, Rosenberg PR (1990) The Charles Bonnet syndrome: Visual perceptive functioning in sensory deprivation. J Clin Neuroophthalmol 10: 215 ± 8. 21. Hosty G (1990) Charles Bonnet syndrome: A description of two cases. Acta Psychiatr Scand 82: 316 ± 7. 22. Bhatia MS, Khastgir U, Malik SC (1992) Charles Bonnet Syndrome. Br J Psych 161: 409 ± 10. 23. Batra A, Bartels M, Wormstall H (1997) Therapeutic options in Charles Bonnet syndrome. Acta Psychiatr Scand 96: 129 ± 33. 24. Bazire S (1999) Psychotropic Drug Directory, 159 ± 60. Quay Books Division, Mark Allen Publishing, Salisbury. 25. World Health Organization (1992) International Classification of Diseases (ICD-10). WHO, Geneva.
