DEMENTIA WITH LEWY BODIES AND CHARLES BONNET SYNDROME Jonathan D. Walker, MD,* Michael A. Keys, MD†
Background: Ophthalmologists may be the first to hear about formed visual hallucinations in the setting of visual loss. Although such hallucinations are likely benign and occur in association with the Charles Bonnet syndrome, it is important to be aware of the strong association of hallucinations with dementia with Lewy bodies because the latter diagnosis is associated with significant prognostic and therapeutic implications for the patient. Methods: Single case report. Results: A patient with macular disease was presumed to have formed hallucinations due to the Charles Bonnet syndrome and was subsequently diagnosed with dementia with Lewy bodies after being admitted to a psychiatric facility. Conclusion: Both patients with the Charles Bonnet syndrome and patients with dementia with Lewy bodies can present with formed visual hallucinations. Ophthalmologists and retina specialists, in particular, should be familiar with the features of dementia with Lewy bodies because the diagnosis of this condition can allow appropriate intervention and help prevent drug-related side effects. If there is any suspicion of early dementia in such patients, they may benefit from neuropsychiatric evaluation. RETINAL CASES & BRIEF REPORTS 2:27–30, 2008
From *Indiana University School of Medicine, Fort Wayne, Indiana; and †Older Adult Mental Health Program, Deaconess Hospital, Cincinnati, Ohio.
these symptoms to other physicians for fear of being thought of as psychotic, and ophthalmologists can play an important role in reassuring such patients by eliciting these symptoms and discussing the fact that the hallucinations are a predictable consequence of their vision loss.2 However, there has also been increasing recognition in the neuropsychiatric literature of an entity known as dementia with Lewy bodies (DLB), and it is important for ophthalmologists to be aware of this form of dementia because affected patients usually have a history of complex visual hallucinations that may be similar to those seen in CBS.3 Because both problems tend to occur in elderly patients, it is likely that the diseases may overlap (i.e., patients with CBS may also happen to have early dementia). The distinction between DLB and the other types of dementia is significant because these patients may be far more susceptible to side effects of common psychiatric medications and may have a better response to drugs that improve cognition and memory relative to patients with Alzheimer disease. Ophthalmologists are therefore in a key position to recognize the potential existence of this diagnosis and suggest the possibility to other members of the health care team.
O
phthalmologists and retina specialists, in particular, are not uncommonly faced with patients complaining of visual hallucinations. There are numerous types of hallucinations that can be caused by problems along the visual pathways and higher centers, and an awareness of the various presentations is important to rule out organic disease.1 A common benign cause of hallucinations that presents to ophthalmologists is Charles Bonnet syndrome (CBS), complex visual hallucinations in the setting of vision loss without other psychiatric conditions. It has been pointed out that patients with CBS may not reveal
J.D.W. had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. He has no financial interests. M.A.K. currently is on the speaker’s bureau for Forest Pharmaceuticals and Pfizer Pharmaceuticals. In the past, he has served on the speaker’s bureau or been a consultant with Eli Lilly, Novartis, and Janssen Pharmaceuticals. Reprint requests: Jonathan D. Walker MD, Indiana University School of Medicine, 7900 West Jefferson, Suite 300, Fort Wayne, IN 46804; e-mail: [email protected]
27
RETINAL CASES & BRIEF REPORTSℜ
28
Case Report A 72-year-old man was referred for progressive diabetic macular edema. At presentation, vision was 20/100 in the right eye and 20/200 in the left eye. He had diffuse cystic macular edema and was treated with several grid lasers in an attempt to control the damage (this was before the widespread use of intravitreal therapies for macular edema). In spite of treatment, his vision slowly deteriorated to counting fingers in both eyes over several years. During this period, he began to complain of vague unformed hallucinations that slowly evolved into more definite complex hallucinations of people and objects. Initially, he recognized that the hallucinations were not real, and he did not manifest any overt signs of dementia. He was reassured that the hallucinations were part of CBS and that they were likely to subside with time, although it was suggested that if the hallucinations became problematic or he began to develop other problems the issue should be discussed with his primary care physician. Shortly after this point, he was lost to follow-up. He returned 3 years later with counting fingers vision in both eyes and chronic macular damage for which there was no ophthalmic treatment. The family related that after he was lost to follow-up he began to have more and more problems with hallucinations and that he became delusional and acted upon the hallucinations. For instance, he would see hallucinations of people standing around his bed at night, and he began to patrol his house with a shotgun for protection. The family obtained an inpatient psychiatric evaluation at another institution, and the clinical diagnosis of DLB was made. He started treatment with low doses of risperidone. He responded quite well to this treatment with marked resolution of his symptoms and was able to be discharged from the hospital. Both the patient and the family reported that he was better able to care for himself, and although he was now living with his family rather than living alone, he was much more functional. His dementia was more manageable, and he was no longer delusional.
Discussion This case highlights the possible overlap between CBS and DLB. CBS is described as the occurrence of complex visual hallucinations, often of a pleasant nature, in individuals with vision loss but also preserved intellectual function and preserved insight that the hallucinations are not real.2 Complex (or formed) visual hallucinations consist of recognizable images of objects or persons and should be distinguished from elementary hallucinations (also called simple or unformed visual hallucinations) that consist of various types of lights, such as flashes, stars, and sparks. Elementary visual phenomena occur far more frequently than CBS in the setting of vision loss. Awareness of CBS is important for all clinicians involved in geriatric care. Menon et al2 cited a host of reports indicating that patients may be reluctant to admit to the hallucinations out of fear of being given a psychiatric diagnosis. In addition to the reluctance of patients to discuss the symptoms, identification of CBS is hampered by the unfamiliarity of medical personnel with the entity and the consequent tendency to incorrectly diagnose mental illness and initiate in-
●
2008
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appropriate therapy.2,4 In fact, simply identifying the problem is perhaps the best intervention for patients with CBS because the ophthalmologist can be instrumental in reassuring such patients about the nature of their symptoms and alleviating their anxiety. However, given that many patients with CBS are elderly, there is a need to be aware of the possible coexistence of early dementia as a contributing factor to the symptoms. DLB is thought to be the second most common cause of neurodegenerative dementia (after Alzheimer disease), accounting for 15% to 25% of dementia cases at autopsy.3 Lewy bodies are eosinophilic inclusion bodies found within the cytoplasm of neurons in the cerebral cortex and limbic system. Lewy bodies are also seen in Parkinson disease, but they are concentrated primarily in the substantia nigra of the basal ganglia rather than in the cerebral cortex. DLB is largely a clinical diagnosis; there is no specific laboratory test that identifies patients with the disease. However, there are two main features that serve to distinguish DLB from Alzheimer disease: motor problems suggestive of Parkinson disease and complex visual hallucinations. The motor symptoms tend to appear within 1 year after the development of cognitive or behavioral symptoms and may significantly worsen with the use of antipsychotic medications. The visual hallucinations associated with DLB are perhaps of greatest significance to the ophthalmologist. The hallucinations occur in ⬇80% of patients and may be the symptom that best distinguishes DLB from other dementias.5 The visual hallucinations in DLB generally are well formed and include visions of people or animals, not unlike those experienced with CBS, although the hallucinations in DLB tend to be more threatening.3,6 In fact, a certain amount of subclinical overlap may be present in elderly patients with hallucinations due to CBS. Although patients with CBS by definition do not have obvious dementia, neuropsychiatric testing for such patients often demonstrates early cognitive impairment, and CBS may be more of a marker for early cognitive difficulties than is traditionally thought.7 The diagnosis of DLB is significant because it is associated with profound therapeutic ramifications. Patients with DLB have an increased risk of marked intolerance to dopamine-blocking antipsychotic medications resulting in excessive parkinsonian symptoms. Both conventional agents such as haloperidol and the newer atypical antipsychotics such as risperidone and olanzapine have been associated with this phenomenon. Problems can include severe akinesia, autonomic dysfunction, and neuroleptic malignant syndrome, all of which may occur at even very low doses and may be life threatening.5 This hypersensi-
29
DEMENTIA WITH LEWY BODIES AND CHARLES BONNET SYNDROME
tivity to antipsychotic medication is compounded by the fact that they are also prone to psychosis triggered by therapies for their parkinsonian symptoms. In essence, these patients are most at risk from the very drugs that would be used to treat their primary symptoms unless the diagnosis is suspected.5 It should be pointed out that this case is unusual in that the patient responded well to risperidone and did not have any significant parkinsonian symptoms or other side effects. Although risperidone has been used successfully in combination with L-dopa to treat DLB,8 it is generally thought that the risk of severe side effects is too great to justify its use as first-line treatment of this disease.9 On the other hand, treatment with cholinesterase inhibitors tends to be well tolerated and can offer meaningful benefit to patients with DLB, especially with symptoms such as apathy, confusion, and hallucinations.10 Recognizing that a patient might have DLB may expedite the use of such medications. Unfortunately, as with Alzheimer disease, no therapy seems to alter the natural progression of the underlying neurodegeneration or time to death. Average survival is ⬇8 years, which is similar to that in Alzheimer disease.3 The ophthalmologist may be the first physician that hears about a patient’s problems with hallucinations. The existence of DLB creates a dilemma if one thinks that such a patient simply has CBS. It is almost as though the diagnosis of CBS should be considered a diagnosis of exclusion, although this is frustrating because perhaps the most useful intervention for a patient with CBS is to reassure them about the benign nature of their symptoms. However, because of the possible overlap between benign CBS symptoms and early dementia, the ophthalmologist has an obligation to at least consider the possibility of the presence of true cognitive decline or psychiatric illness that may be exacerbating both the severity of hallucinations and the patient’s reactions to the hallucinations. The complexity of the situation is enhanced by the fact that there is no simple way to both reassure a patient about the benign nature of their symptoms and discuss a possible association with a life-changing neurodegenerative disorder. The problem is made even more difficult because it is unlikely that the ophthalmologist would have expertise in identifying or ruling out early cognitive difficulties. On the other hand, if every patient with CBS was subjected to the rigors of a formal neuropsychiatric evaluation, the cost in terms of both utilization of resources and the unnecessary stress imposed on the patient and family would be unacceptable.
Traditionally, the evaluation of patients for early cognitive impairment includes a careful history looking for any evidence of cognitive or behavioral changes and at least a brief examination of cognitive abilities such as the Mini-Mental Status Examination.11,12 Such an intervention is well outside the usual focus of a typical ophthalmic practice. Perhaps the best approach for the ophthalmologist is to discuss the patient’s perception of their hallucinations as well as the family’s sense of how the patient is doing in terms of overall cognitive function. If there is no overt indication of behavioral or cognitive changes, then the patient should be reassured about the benign nature of the symptoms. It may also be reasonable to send a letter explaining the nature of CBS symptoms to their primary care physician, perhaps with the caveat to both the patient and the physician that if the hallucinations become intrusive or evidence of cognitive problems develop then further evaluation may be warranted. If the discussion with the patient and family is suggestive of early cognitive or behavioral problems, it may be more reasonable to proceed with a more formal evaluation by the primary care physician or, depending on the degree of concern, a referral to a neurologist or geriatric psychiatrist with expertise in the management of neurodegenerative disorders. The potential for poor compliance with treatment and follow-up would also need to be recognized, as in this case where the patient was lost to follow-up shortly after the hallucinations began. Finally, by suggesting the possibility of DLB in this setting, the ophthalmologist can help the patient’s other health care providers recognize that such a patient may be more likely to have severe, potentially life-threatening reactions to some of the medications that would be most likely used to treat their symptoms. Key words: Charles Bonnet syndrome, dementia with Lewy bodies, visual hallucinations. References 1. 2.
3.
4.
5.
Manford M, Andermann F. Complex visual hallucinations. Clinical and neurobiological insights. Brain 1998;121:1819–1840. Menon GJ, Rahman I, Menon SJ, Dutton GN. Complex visual hallucinations in the visually impaired: the Charles Bonnet syndrome. Surv Ophthalmol 2003;48:58–72. Geldmacher DS. Dementia with Lewy bodies: diagnosis and clinical approach. Cleve Clin J Med 2004;71:789–790, 792– 794, 797–798. Jacob A, Prasad S, Boggild M, Chandratre S. Charles Bonnet syndrome— elderly people and visual hallucinations. BMJ 2004;328:1552–1554. Stewart JT. Defining diffuse Lewy body disease. Tetrad of symptoms distinguishes illness from other dementias. Postgrad Med 2003;113:71–75.
30 6.
7.
8.
RETINAL CASES & BRIEF REPORTSℜ Mosimann UP, Rowan EN, Partington CE, et al. Characteristics of visual hallucinations in Parkinson disease dementia and dementia with Lewy bodies. Am J Geriatr Psychiatry 2006;14:153–160. Pliskin NH, Kiolbasa TA, Towle VL, et al. Charles Bonnet syndrome: an early marker for dementia? J Am Geriatr Soc 1996;44:1055–1061. Kato K, Wada T, Kawakatsu S, Otani K. Improvement of both psychotic symptoms and Parkinsonism in a case of dementia with Lewy bodies by the combination therapy of risperidone and L-DOPA. Prog Neuropsychopharmacol Biol Psychiatry 2002; 26:201–203.
9.
10. 11.
12.
●
2008
●
VOLUME 2
●
NUMBER 1
Baskys A. Lewy body dementia: the litmus test for neuroleptic sensitivity and extrapyramidal symptoms. J Clin Psychiatry 2004;65:S16 –22. McKeith I, Mintzer J, Aarsland D, et al. Dementia with Lewy bodies. Lancet Neurol 2004;3:19–28. Knopman DS, Boeve BF, Petersen RC. Essentials of the proper diagnoses of mild cognitive impairment, dementia, and major subtypes of dementia. Mayo Clin Proc 2003;78: 1290–1308. Tangalos EG, Smith GE, Ivnik RJ, et al. The Mini-Mental State Examination in general medical practice: clinical utility and acceptance. Mayo Clin Proc 1996;71:829–837.
Background: Ophthalmologists may be the first to hear about formed visual hallucinations in the setting of visual loss. Although such hallucinations are likely benign and occur in association with the Charles Bonnet syndrome, it is important to be aware of the strong association of hallucinations with dementia with Lewy bodies because the latter diagnosis is associated with significant prognostic and therapeutic implications for the patient. Methods: Single case report. Results: A patient with macular disease was presumed to have formed hallucinations due to the Charles Bonnet syndrome and was subsequently diagnosed with dementia with Lewy bodies after being admitted to a psychiatric facility. Conclusion: Both patients with the Charles Bonnet syndrome and patients with dementia with Lewy bodies can present with formed visual hallucinations. Ophthalmologists and retina specialists, in particular, should be familiar with the features of dementia with Lewy bodies because the diagnosis of this condition can allow appropriate intervention and help prevent drug-related side effects. If there is any suspicion of early dementia in such patients, they may benefit from neuropsychiatric evaluation. RETINAL CASES & BRIEF REPORTS 2:27–30, 2008
From *Indiana University School of Medicine, Fort Wayne, Indiana; and †Older Adult Mental Health Program, Deaconess Hospital, Cincinnati, Ohio.
these symptoms to other physicians for fear of being thought of as psychotic, and ophthalmologists can play an important role in reassuring such patients by eliciting these symptoms and discussing the fact that the hallucinations are a predictable consequence of their vision loss.2 However, there has also been increasing recognition in the neuropsychiatric literature of an entity known as dementia with Lewy bodies (DLB), and it is important for ophthalmologists to be aware of this form of dementia because affected patients usually have a history of complex visual hallucinations that may be similar to those seen in CBS.3 Because both problems tend to occur in elderly patients, it is likely that the diseases may overlap (i.e., patients with CBS may also happen to have early dementia). The distinction between DLB and the other types of dementia is significant because these patients may be far more susceptible to side effects of common psychiatric medications and may have a better response to drugs that improve cognition and memory relative to patients with Alzheimer disease. Ophthalmologists are therefore in a key position to recognize the potential existence of this diagnosis and suggest the possibility to other members of the health care team.
O
phthalmologists and retina specialists, in particular, are not uncommonly faced with patients complaining of visual hallucinations. There are numerous types of hallucinations that can be caused by problems along the visual pathways and higher centers, and an awareness of the various presentations is important to rule out organic disease.1 A common benign cause of hallucinations that presents to ophthalmologists is Charles Bonnet syndrome (CBS), complex visual hallucinations in the setting of vision loss without other psychiatric conditions. It has been pointed out that patients with CBS may not reveal
J.D.W. had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. He has no financial interests. M.A.K. currently is on the speaker’s bureau for Forest Pharmaceuticals and Pfizer Pharmaceuticals. In the past, he has served on the speaker’s bureau or been a consultant with Eli Lilly, Novartis, and Janssen Pharmaceuticals. Reprint requests: Jonathan D. Walker MD, Indiana University School of Medicine, 7900 West Jefferson, Suite 300, Fort Wayne, IN 46804; e-mail: [email protected]
27
RETINAL CASES & BRIEF REPORTSℜ
28
Case Report A 72-year-old man was referred for progressive diabetic macular edema. At presentation, vision was 20/100 in the right eye and 20/200 in the left eye. He had diffuse cystic macular edema and was treated with several grid lasers in an attempt to control the damage (this was before the widespread use of intravitreal therapies for macular edema). In spite of treatment, his vision slowly deteriorated to counting fingers in both eyes over several years. During this period, he began to complain of vague unformed hallucinations that slowly evolved into more definite complex hallucinations of people and objects. Initially, he recognized that the hallucinations were not real, and he did not manifest any overt signs of dementia. He was reassured that the hallucinations were part of CBS and that they were likely to subside with time, although it was suggested that if the hallucinations became problematic or he began to develop other problems the issue should be discussed with his primary care physician. Shortly after this point, he was lost to follow-up. He returned 3 years later with counting fingers vision in both eyes and chronic macular damage for which there was no ophthalmic treatment. The family related that after he was lost to follow-up he began to have more and more problems with hallucinations and that he became delusional and acted upon the hallucinations. For instance, he would see hallucinations of people standing around his bed at night, and he began to patrol his house with a shotgun for protection. The family obtained an inpatient psychiatric evaluation at another institution, and the clinical diagnosis of DLB was made. He started treatment with low doses of risperidone. He responded quite well to this treatment with marked resolution of his symptoms and was able to be discharged from the hospital. Both the patient and the family reported that he was better able to care for himself, and although he was now living with his family rather than living alone, he was much more functional. His dementia was more manageable, and he was no longer delusional.
Discussion This case highlights the possible overlap between CBS and DLB. CBS is described as the occurrence of complex visual hallucinations, often of a pleasant nature, in individuals with vision loss but also preserved intellectual function and preserved insight that the hallucinations are not real.2 Complex (or formed) visual hallucinations consist of recognizable images of objects or persons and should be distinguished from elementary hallucinations (also called simple or unformed visual hallucinations) that consist of various types of lights, such as flashes, stars, and sparks. Elementary visual phenomena occur far more frequently than CBS in the setting of vision loss. Awareness of CBS is important for all clinicians involved in geriatric care. Menon et al2 cited a host of reports indicating that patients may be reluctant to admit to the hallucinations out of fear of being given a psychiatric diagnosis. In addition to the reluctance of patients to discuss the symptoms, identification of CBS is hampered by the unfamiliarity of medical personnel with the entity and the consequent tendency to incorrectly diagnose mental illness and initiate in-
●
2008
●
VOLUME 2
●
NUMBER 1
appropriate therapy.2,4 In fact, simply identifying the problem is perhaps the best intervention for patients with CBS because the ophthalmologist can be instrumental in reassuring such patients about the nature of their symptoms and alleviating their anxiety. However, given that many patients with CBS are elderly, there is a need to be aware of the possible coexistence of early dementia as a contributing factor to the symptoms. DLB is thought to be the second most common cause of neurodegenerative dementia (after Alzheimer disease), accounting for 15% to 25% of dementia cases at autopsy.3 Lewy bodies are eosinophilic inclusion bodies found within the cytoplasm of neurons in the cerebral cortex and limbic system. Lewy bodies are also seen in Parkinson disease, but they are concentrated primarily in the substantia nigra of the basal ganglia rather than in the cerebral cortex. DLB is largely a clinical diagnosis; there is no specific laboratory test that identifies patients with the disease. However, there are two main features that serve to distinguish DLB from Alzheimer disease: motor problems suggestive of Parkinson disease and complex visual hallucinations. The motor symptoms tend to appear within 1 year after the development of cognitive or behavioral symptoms and may significantly worsen with the use of antipsychotic medications. The visual hallucinations associated with DLB are perhaps of greatest significance to the ophthalmologist. The hallucinations occur in ⬇80% of patients and may be the symptom that best distinguishes DLB from other dementias.5 The visual hallucinations in DLB generally are well formed and include visions of people or animals, not unlike those experienced with CBS, although the hallucinations in DLB tend to be more threatening.3,6 In fact, a certain amount of subclinical overlap may be present in elderly patients with hallucinations due to CBS. Although patients with CBS by definition do not have obvious dementia, neuropsychiatric testing for such patients often demonstrates early cognitive impairment, and CBS may be more of a marker for early cognitive difficulties than is traditionally thought.7 The diagnosis of DLB is significant because it is associated with profound therapeutic ramifications. Patients with DLB have an increased risk of marked intolerance to dopamine-blocking antipsychotic medications resulting in excessive parkinsonian symptoms. Both conventional agents such as haloperidol and the newer atypical antipsychotics such as risperidone and olanzapine have been associated with this phenomenon. Problems can include severe akinesia, autonomic dysfunction, and neuroleptic malignant syndrome, all of which may occur at even very low doses and may be life threatening.5 This hypersensi-
29
DEMENTIA WITH LEWY BODIES AND CHARLES BONNET SYNDROME
tivity to antipsychotic medication is compounded by the fact that they are also prone to psychosis triggered by therapies for their parkinsonian symptoms. In essence, these patients are most at risk from the very drugs that would be used to treat their primary symptoms unless the diagnosis is suspected.5 It should be pointed out that this case is unusual in that the patient responded well to risperidone and did not have any significant parkinsonian symptoms or other side effects. Although risperidone has been used successfully in combination with L-dopa to treat DLB,8 it is generally thought that the risk of severe side effects is too great to justify its use as first-line treatment of this disease.9 On the other hand, treatment with cholinesterase inhibitors tends to be well tolerated and can offer meaningful benefit to patients with DLB, especially with symptoms such as apathy, confusion, and hallucinations.10 Recognizing that a patient might have DLB may expedite the use of such medications. Unfortunately, as with Alzheimer disease, no therapy seems to alter the natural progression of the underlying neurodegeneration or time to death. Average survival is ⬇8 years, which is similar to that in Alzheimer disease.3 The ophthalmologist may be the first physician that hears about a patient’s problems with hallucinations. The existence of DLB creates a dilemma if one thinks that such a patient simply has CBS. It is almost as though the diagnosis of CBS should be considered a diagnosis of exclusion, although this is frustrating because perhaps the most useful intervention for a patient with CBS is to reassure them about the benign nature of their symptoms. However, because of the possible overlap between benign CBS symptoms and early dementia, the ophthalmologist has an obligation to at least consider the possibility of the presence of true cognitive decline or psychiatric illness that may be exacerbating both the severity of hallucinations and the patient’s reactions to the hallucinations. The complexity of the situation is enhanced by the fact that there is no simple way to both reassure a patient about the benign nature of their symptoms and discuss a possible association with a life-changing neurodegenerative disorder. The problem is made even more difficult because it is unlikely that the ophthalmologist would have expertise in identifying or ruling out early cognitive difficulties. On the other hand, if every patient with CBS was subjected to the rigors of a formal neuropsychiatric evaluation, the cost in terms of both utilization of resources and the unnecessary stress imposed on the patient and family would be unacceptable.
Traditionally, the evaluation of patients for early cognitive impairment includes a careful history looking for any evidence of cognitive or behavioral changes and at least a brief examination of cognitive abilities such as the Mini-Mental Status Examination.11,12 Such an intervention is well outside the usual focus of a typical ophthalmic practice. Perhaps the best approach for the ophthalmologist is to discuss the patient’s perception of their hallucinations as well as the family’s sense of how the patient is doing in terms of overall cognitive function. If there is no overt indication of behavioral or cognitive changes, then the patient should be reassured about the benign nature of the symptoms. It may also be reasonable to send a letter explaining the nature of CBS symptoms to their primary care physician, perhaps with the caveat to both the patient and the physician that if the hallucinations become intrusive or evidence of cognitive problems develop then further evaluation may be warranted. If the discussion with the patient and family is suggestive of early cognitive or behavioral problems, it may be more reasonable to proceed with a more formal evaluation by the primary care physician or, depending on the degree of concern, a referral to a neurologist or geriatric psychiatrist with expertise in the management of neurodegenerative disorders. The potential for poor compliance with treatment and follow-up would also need to be recognized, as in this case where the patient was lost to follow-up shortly after the hallucinations began. Finally, by suggesting the possibility of DLB in this setting, the ophthalmologist can help the patient’s other health care providers recognize that such a patient may be more likely to have severe, potentially life-threatening reactions to some of the medications that would be most likely used to treat their symptoms. Key words: Charles Bonnet syndrome, dementia with Lewy bodies, visual hallucinations. References 1. 2.
3.
4.
5.
Manford M, Andermann F. Complex visual hallucinations. Clinical and neurobiological insights. Brain 1998;121:1819–1840. Menon GJ, Rahman I, Menon SJ, Dutton GN. Complex visual hallucinations in the visually impaired: the Charles Bonnet syndrome. Surv Ophthalmol 2003;48:58–72. Geldmacher DS. Dementia with Lewy bodies: diagnosis and clinical approach. Cleve Clin J Med 2004;71:789–790, 792– 794, 797–798. Jacob A, Prasad S, Boggild M, Chandratre S. Charles Bonnet syndrome— elderly people and visual hallucinations. BMJ 2004;328:1552–1554. Stewart JT. Defining diffuse Lewy body disease. Tetrad of symptoms distinguishes illness from other dementias. Postgrad Med 2003;113:71–75.
30 6.
7.
8.
RETINAL CASES & BRIEF REPORTSℜ Mosimann UP, Rowan EN, Partington CE, et al. Characteristics of visual hallucinations in Parkinson disease dementia and dementia with Lewy bodies. Am J Geriatr Psychiatry 2006;14:153–160. Pliskin NH, Kiolbasa TA, Towle VL, et al. Charles Bonnet syndrome: an early marker for dementia? J Am Geriatr Soc 1996;44:1055–1061. Kato K, Wada T, Kawakatsu S, Otani K. Improvement of both psychotic symptoms and Parkinsonism in a case of dementia with Lewy bodies by the combination therapy of risperidone and L-DOPA. Prog Neuropsychopharmacol Biol Psychiatry 2002; 26:201–203.
9.
10. 11.
12.
●
2008
●
VOLUME 2
●
NUMBER 1
Baskys A. Lewy body dementia: the litmus test for neuroleptic sensitivity and extrapyramidal symptoms. J Clin Psychiatry 2004;65:S16 –22. McKeith I, Mintzer J, Aarsland D, et al. Dementia with Lewy bodies. Lancet Neurol 2004;3:19–28. Knopman DS, Boeve BF, Petersen RC. Essentials of the proper diagnoses of mild cognitive impairment, dementia, and major subtypes of dementia. Mayo Clin Proc 2003;78: 1290–1308. Tangalos EG, Smith GE, Ivnik RJ, et al. The Mini-Mental State Examination in general medical practice: clinical utility and acceptance. Mayo Clin Proc 1996;71:829–837.
