Thomas Newmark, MD

History: ‘They’re making me crazy’

Ms. D, age 22, is brought to the emergency room by her older brother for psychiatric evaluation after a family argument. He tells us that his sister is out most nights, hanging out at nightclubs. When she’s home, he says, she locks herself in her room and avoids him and his younger brother, who also lives with them.

Recently, her brother says, Ms. D signed a contract to appear in pornographic videos. When he found out, he went to the studio’s producer and nullified the contract.

Ms. D, frustrated with her brother’s interference, tells us she dreams of becoming a movie star and going to college, but blames him for “holding me back” and keeping her unemployed.

Worse, she says, he and her two sisters are impostors who are “trying to hurt me” and are “making me go crazy.” She fears her “false brother” will take her house if she leaves, yet she feels unsafe at home because strangers—envious of “my beauty and intelligence”—peek into her windows and stalk her. She tells us her father is near and guards her—even though he died 4 years ago.

Ms. D, who lost her mother at age 2, began having psychotic episodes at age 19, a few months after her father’s death. At that time, she was hospitalized after insisting that her father had faked his death because of a conspiracy against him. A hospital psychiatrist diagnosed bipolar disorder and prescribed a mood stabilizer, but she did not take the medication and her psychosis has worsened.

Ms. D’s Mini-Mental State Examination score of 30 indicates that she is neither grossly confused nor has underlying dementia. However, she is emotionally labile with grossly disorganized thought processes and paranoid and grandiose delusions.

We could not locate other family members, so Ms. D’s family psychiatric history is unknown. She has casual relationships with men but does not have a boyfriend. She acknowledges that she frequents local nightclubs but denies using alcohol.

Blood work and other medical examination results are normal. Negative urine toxicology screen suggests she not abusing substances, and electrolytes and thyroid-stimulating hormone levels are normal. Negative rapid plasma reagin rules out tertiary syphilis. We do not order radiologic studies because her presentation does not suggest focal abnormality, and neurologic exam results are benign.

poll here

The authors’ observations

Patients with both paranoid delusions and manic features are challenging. Prognoses and treatment options for each group of symptoms differ substantially.

Ms. D’s grandiosity, pressured speech, tangential flight of ideas, and hypersexuality strongly suggest bipolar disorder. We could not rule out schizophrenia, however, because of her prominent hallucinations and paranoia.

Pharmacologic intoxication was not likely based on laboratory results and the longstanding, progressive course of Ms. D’s disorder. Organic pathology also was unlikely, given her normal neurologic examination and lack of other medical issues.

Treatment: Talk therapy

We tentatively diagnose Ms. D as having bipolar disorder type I with a manic episode and psychotic features. She does not meet DSM-IV-TR criteria for schizophrenia and lacks affective flattening, poverty of speech, avolition, and other negative symptoms typical of the disorder. We admit her to the inpatient psychiatric unit and prescribe lithium, 300 mg tid, and quetiapine, 50 mg bid.

An internal medicine (IM) resident visits Ms. D for 30 to 45 minutes daily during her hospitalization to check her medical status and to allow her to vent her frustration. A resident in psychiatry also interviews Ms. D for about one half-hour each day. The patient rarely interacts with other patients and speaks only with physicians and nurses.

Ms. D appears to trust the IM resident and confides in her about her brother. During their first meeting, she appears most disturbed that a man who “claims” to be her brother is sabotaging her life. She does not fear that this “impostor” will physically harm her but still distrusts him. She repeatedly reports that her late father is nearby or in the room above hers. She adds that she feels much safer in the hospital, where the “stalkers” cannot reach her.

At times, Ms. D tells the IM resident she has a twin. Other times, she believes her family is much larger than it is, and she sometimes laments that she is losing her identity. She often perseverates on Judgment Day, at which time she says her “fake” relatives will answer for their actions against her.

Ms. D’s delusions of grandiosity, tangentiality, circumferential speech, and flight of ideas persist through 4 days in the hospital. Her affect is extremely labile and occasionally inappropriate. She sometimes cries when discussing her father’s death, then stops, thinks a moment, and begins laughing. At this point, we increase lithium to 600 mg tid and quetiapine to 100 mg tid. She is suffering no side effects and infrequently requires haloperidol as a demand dose only.

 

poll here

The authors’ observations

A patient such as Ms. D who lives in a minimally supportive environment and has paranoid delusions could fabricate an explanation for what she perceives as family members’ incongruent behavior. She could create a reality in which these relatives are impostors.

Although this behavior is not unusual, Ms. D’s extreme reaction toward her siblings suggests Capgras syndrome, a rare misidentification disorder (Box). The syndrome is often missed in clinical practice, and its prevalence has not been quantified.

Capgras syndrome is seen most often in patients with paranoid schizophrenia—the highest functioning and most preserved schizophrenia patients. This association may indicate that both neurologic dysfunction and psychological background are necessary to produce the syndrome.

The belief that family members are impostors could point to a conspiracy theory or paranoid delusion. Ms. D’s suspicion and distrust toward her older brother indicate a paranoid state, and her other delusions—such as her belief that others are stalking her—suggest that her Capgras symptoms are another manifestation of paranoia.

Box

Capgras’ causes. Capgras delusions can occur secondary to neurologic lesions and often appear to have an organic cause, such as abnormal focal paroxysmal discharges.¹ These delusions can occur secondary to systemic infections, thyroid dysfunction, seizures, concussion, intoxication dementia, toxic encephalopathy, or head trauma.^1,2 Theories vary as to physiologic, structural, and neurologic causes (Table).

For Ms. D, structural brain deficits probably interacted with her psychosocial milieu to create Capgras delusions, though we did not perform confirmatory brain imaging or functional neurologic testing. Whereas right cortical lesions might impair recognition while preserving familiarity, Capgras syndrome preserves recognition but deadens the emotion that makes faces seem familiar. When focal lesions are found to cause Capgras delusion, however, the right hemisphere—specifically the frontal cortex—usually is affected.^2,3

Table

Proposed causes of Capgras syndrome

Physiologic
Frontal lobe damage may distort visual stimuli monitoring, thus impairing facial recognition.4
Disruption of neuronal connections within the right temporal lobe scrambles memories needed for facial recognition.5
Neurologic
Disconnection between brain hemispheres lead to cognitive but not affective recognition.6
Bifrontal pathology or other organic cause blurs “judgment of individuality or uniqueness,” as in prosopagnosia.3
Dorsal pathway impairment alters affective response to faces.7
Dissociation in the amygdala may distort affective response to faces.8
Psychological*
In depression, misidentification develops secondary to rationalizing feelings of guilt and inferiority.9
“Two-armed recognition”—one automatic and almost instantaneous, the other attentive and mnemonic—begins to falter.10
Suspicion, preoccupation with details leads to “agnosia through too great attention.”11
Avoidance of unconscious desires leads to recognition problems.12
Patient “projects and splits” family member into two persons; directs love toward real person and hate toward imagined impostor.13
In schizophrenia, world is viewed through primitive mechanisms, such as doubles and dualism.14
*Dependent on psychiatric comorbidity

poll here

The authors’ observations

When interviewing a patient with paranoid delusions, get as much detail as possible about his or her close relationships. Try to interview one or two family members or friends. The information can help determine whether Capgras symptoms underlie paranoia.

Brain imaging might uncover pertinent abnormalities, but the cost could outweigh any benefit. No evidence supports use of CT to diagnose Capgras syndrome. Some evidence supports use of brain MRI, but more research is needed.

No specific treatment exists for Capgras delusions apart from using antipsychotics to treat the psychosis based on clinical suspicion and constellation of symptoms.

Studies have shown no difference in response to atypical antipsychotics between patients with schizophrenia and comcomitant Capgras symptoms and those with schizophrenia alone. In clinical practice, we have found that treating Capgras symptoms does improve schizophrenia’s course.

Adjunctive psychotherapy has not been studied in Capgras syndrome, and directed, insight-guided therapy might not resolve deeply rooted delusions for some patients. With Ms. D, however, “talk therapy” helped us build rapport and gave us insight into her strained familial relationships. Establishing a therapeutic alliance with the patient and encouraging healthy relationships with his or her family and friends can mitigate the effects of Capgras paranoia.

 

Continued treatment: Gradual change

Day by day Ms. D’s mania subsides gradually, though she still fears that a stranger posing as her brother is stalking her. She talks about her brother less frequently, though she is clearly holding fast to her delusional beliefs.

We discharge Ms. D after 10 days. Although her symptoms have not resolved, she is markedly less manic and less agitated than at admission. We arrange treatment with outpatient psychiatry. She does not follow up with her original psychiatrist and is lost to follow-up.

Related resources

• PsychNet-UK. Disorder information sheet: Capgras (delusion) syndrome. www.psychnet-uk.com/dsm_iv/capgras_syndrome.htm.
  
• Bourget D, Whitehurst L. Capgras syndrome: a review of the neurophysiological correlates and presenting clinical features in cases involving physical violence. Can J Psychiatry 2004;49:719-25. Available at: www.cpa-apc.org/Publications/Archives/CJP/2004/november/bourget.asp.
  
• Barton JJ. Disorders of face perception and recognition. Neurol Clin 2003;21:521-48.
  
• Lewis S. Brain imaging in a case of Capgras’ syndrome. Br J Psychiatry 1987;150:117-21.
  
• Christodoulou GN. The syndrome of Capgras. Br J Psychiatry 1977;130:556-64.
  

Drug brand names

• Haloperidol • Haldol
  
• Lithium • Eskalith, others
  
• Quetiapine • Seroquel
  

Disclosures

The authors report no financial relationship with any company whose products are mentioned in this article, or with manufacturers of competing products.

History: ‘They’re making me crazy’

Ms. D, age 22, is brought to the emergency room by her older brother for psychiatric evaluation after a family argument. He tells us that his sister is out most nights, hanging out at nightclubs. When she’s home, he says, she locks herself in her room and avoids him and his younger brother, who also lives with them.

Recently, her brother says, Ms. D signed a contract to appear in pornographic videos. When he found out, he went to the studio’s producer and nullified the contract.

Ms. D, frustrated with her brother’s interference, tells us she dreams of becoming a movie star and going to college, but blames him for “holding me back” and keeping her unemployed.

Worse, she says, he and her two sisters are impostors who are “trying to hurt me” and are “making me go crazy.” She fears her “false brother” will take her house if she leaves, yet she feels unsafe at home because strangers—envious of “my beauty and intelligence”—peek into her windows and stalk her. She tells us her father is near and guards her—even though he died 4 years ago.

Ms. D, who lost her mother at age 2, began having psychotic episodes at age 19, a few months after her father’s death. At that time, she was hospitalized after insisting that her father had faked his death because of a conspiracy against him. A hospital psychiatrist diagnosed bipolar disorder and prescribed a mood stabilizer, but she did not take the medication and her psychosis has worsened.

Ms. D’s Mini-Mental State Examination score of 30 indicates that she is neither grossly confused nor has underlying dementia. However, she is emotionally labile with grossly disorganized thought processes and paranoid and grandiose delusions.

We could not locate other family members, so Ms. D’s family psychiatric history is unknown. She has casual relationships with men but does not have a boyfriend. She acknowledges that she frequents local nightclubs but denies using alcohol.

Blood work and other medical examination results are normal. Negative urine toxicology screen suggests she not abusing substances, and electrolytes and thyroid-stimulating hormone levels are normal. Negative rapid plasma reagin rules out tertiary syphilis. We do not order radiologic studies because her presentation does not suggest focal abnormality, and neurologic exam results are benign.

poll here

The authors’ observations

Patients with both paranoid delusions and manic features are challenging. Prognoses and treatment options for each group of symptoms differ substantially.

Ms. D’s grandiosity, pressured speech, tangential flight of ideas, and hypersexuality strongly suggest bipolar disorder. We could not rule out schizophrenia, however, because of her prominent hallucinations and paranoia.

Pharmacologic intoxication was not likely based on laboratory results and the longstanding, progressive course of Ms. D’s disorder. Organic pathology also was unlikely, given her normal neurologic examination and lack of other medical issues.

Treatment: Talk therapy

We tentatively diagnose Ms. D as having bipolar disorder type I with a manic episode and psychotic features. She does not meet DSM-IV-TR criteria for schizophrenia and lacks affective flattening, poverty of speech, avolition, and other negative symptoms typical of the disorder. We admit her to the inpatient psychiatric unit and prescribe lithium, 300 mg tid, and quetiapine, 50 mg bid.

An internal medicine (IM) resident visits Ms. D for 30 to 45 minutes daily during her hospitalization to check her medical status and to allow her to vent her frustration. A resident in psychiatry also interviews Ms. D for about one half-hour each day. The patient rarely interacts with other patients and speaks only with physicians and nurses.

Ms. D appears to trust the IM resident and confides in her about her brother. During their first meeting, she appears most disturbed that a man who “claims” to be her brother is sabotaging her life. She does not fear that this “impostor” will physically harm her but still distrusts him. She repeatedly reports that her late father is nearby or in the room above hers. She adds that she feels much safer in the hospital, where the “stalkers” cannot reach her.

At times, Ms. D tells the IM resident she has a twin. Other times, she believes her family is much larger than it is, and she sometimes laments that she is losing her identity. She often perseverates on Judgment Day, at which time she says her “fake” relatives will answer for their actions against her.

Ms. D’s delusions of grandiosity, tangentiality, circumferential speech, and flight of ideas persist through 4 days in the hospital. Her affect is extremely labile and occasionally inappropriate. She sometimes cries when discussing her father’s death, then stops, thinks a moment, and begins laughing. At this point, we increase lithium to 600 mg tid and quetiapine to 100 mg tid. She is suffering no side effects and infrequently requires haloperidol as a demand dose only.

 

poll here

The authors’ observations

A patient such as Ms. D who lives in a minimally supportive environment and has paranoid delusions could fabricate an explanation for what she perceives as family members’ incongruent behavior. She could create a reality in which these relatives are impostors.

Although this behavior is not unusual, Ms. D’s extreme reaction toward her siblings suggests Capgras syndrome, a rare misidentification disorder (Box). The syndrome is often missed in clinical practice, and its prevalence has not been quantified.

Capgras syndrome is seen most often in patients with paranoid schizophrenia—the highest functioning and most preserved schizophrenia patients. This association may indicate that both neurologic dysfunction and psychological background are necessary to produce the syndrome.

The belief that family members are impostors could point to a conspiracy theory or paranoid delusion. Ms. D’s suspicion and distrust toward her older brother indicate a paranoid state, and her other delusions—such as her belief that others are stalking her—suggest that her Capgras symptoms are another manifestation of paranoia.

Box

Capgras’ causes. Capgras delusions can occur secondary to neurologic lesions and often appear to have an organic cause, such as abnormal focal paroxysmal discharges.¹ These delusions can occur secondary to systemic infections, thyroid dysfunction, seizures, concussion, intoxication dementia, toxic encephalopathy, or head trauma.^1,2 Theories vary as to physiologic, structural, and neurologic causes (Table).

For Ms. D, structural brain deficits probably interacted with her psychosocial milieu to create Capgras delusions, though we did not perform confirmatory brain imaging or functional neurologic testing. Whereas right cortical lesions might impair recognition while preserving familiarity, Capgras syndrome preserves recognition but deadens the emotion that makes faces seem familiar. When focal lesions are found to cause Capgras delusion, however, the right hemisphere—specifically the frontal cortex—usually is affected.^2,3

Table

Proposed causes of Capgras syndrome

Physiologic
Frontal lobe damage may distort visual stimuli monitoring, thus impairing facial recognition.4
Disruption of neuronal connections within the right temporal lobe scrambles memories needed for facial recognition.5
Neurologic
Disconnection between brain hemispheres lead to cognitive but not affective recognition.6
Bifrontal pathology or other organic cause blurs “judgment of individuality or uniqueness,” as in prosopagnosia.3
Dorsal pathway impairment alters affective response to faces.7
Dissociation in the amygdala may distort affective response to faces.8
Psychological*
In depression, misidentification develops secondary to rationalizing feelings of guilt and inferiority.9
“Two-armed recognition”—one automatic and almost instantaneous, the other attentive and mnemonic—begins to falter.10
Suspicion, preoccupation with details leads to “agnosia through too great attention.”11
Avoidance of unconscious desires leads to recognition problems.12
Patient “projects and splits” family member into two persons; directs love toward real person and hate toward imagined impostor.13
In schizophrenia, world is viewed through primitive mechanisms, such as doubles and dualism.14
*Dependent on psychiatric comorbidity

poll here

The authors’ observations

When interviewing a patient with paranoid delusions, get as much detail as possible about his or her close relationships. Try to interview one or two family members or friends. The information can help determine whether Capgras symptoms underlie paranoia.

Brain imaging might uncover pertinent abnormalities, but the cost could outweigh any benefit. No evidence supports use of CT to diagnose Capgras syndrome. Some evidence supports use of brain MRI, but more research is needed.

No specific treatment exists for Capgras delusions apart from using antipsychotics to treat the psychosis based on clinical suspicion and constellation of symptoms.

Studies have shown no difference in response to atypical antipsychotics between patients with schizophrenia and comcomitant Capgras symptoms and those with schizophrenia alone. In clinical practice, we have found that treating Capgras symptoms does improve schizophrenia’s course.

Adjunctive psychotherapy has not been studied in Capgras syndrome, and directed, insight-guided therapy might not resolve deeply rooted delusions for some patients. With Ms. D, however, “talk therapy” helped us build rapport and gave us insight into her strained familial relationships. Establishing a therapeutic alliance with the patient and encouraging healthy relationships with his or her family and friends can mitigate the effects of Capgras paranoia.

 

Continued treatment: Gradual change

Day by day Ms. D’s mania subsides gradually, though she still fears that a stranger posing as her brother is stalking her. She talks about her brother less frequently, though she is clearly holding fast to her delusional beliefs.

We discharge Ms. D after 10 days. Although her symptoms have not resolved, she is markedly less manic and less agitated than at admission. We arrange treatment with outpatient psychiatry. She does not follow up with her original psychiatrist and is lost to follow-up.

Related resources

• PsychNet-UK. Disorder information sheet: Capgras (delusion) syndrome. www.psychnet-uk.com/dsm_iv/capgras_syndrome.htm.
  
• Bourget D, Whitehurst L. Capgras syndrome: a review of the neurophysiological correlates and presenting clinical features in cases involving physical violence. Can J Psychiatry 2004;49:719-25. Available at: www.cpa-apc.org/Publications/Archives/CJP/2004/november/bourget.asp.
  
• Barton JJ. Disorders of face perception and recognition. Neurol Clin 2003;21:521-48.
  
• Lewis S. Brain imaging in a case of Capgras’ syndrome. Br J Psychiatry 1987;150:117-21.
  
• Christodoulou GN. The syndrome of Capgras. Br J Psychiatry 1977;130:556-64.
  

Drug brand names

• Haloperidol • Haldol
  
• Lithium • Eskalith, others
  
• Quetiapine • Seroquel
  

Disclosures

The authors report no financial relationship with any company whose products are mentioned in this article, or with manufacturers of competing products.

History: ‘They’re making me crazy’

Ms. D, age 22, is brought to the emergency room by her older brother for psychiatric evaluation after a family argument. He tells us that his sister is out most nights, hanging out at nightclubs. When she’s home, he says, she locks herself in her room and avoids him and his younger brother, who also lives with them.

Recently, her brother says, Ms. D signed a contract to appear in pornographic videos. When he found out, he went to the studio’s producer and nullified the contract.

Ms. D, frustrated with her brother’s interference, tells us she dreams of becoming a movie star and going to college, but blames him for “holding me back” and keeping her unemployed.

Worse, she says, he and her two sisters are impostors who are “trying to hurt me” and are “making me go crazy.” She fears her “false brother” will take her house if she leaves, yet she feels unsafe at home because strangers—envious of “my beauty and intelligence”—peek into her windows and stalk her. She tells us her father is near and guards her—even though he died 4 years ago.

Ms. D, who lost her mother at age 2, began having psychotic episodes at age 19, a few months after her father’s death. At that time, she was hospitalized after insisting that her father had faked his death because of a conspiracy against him. A hospital psychiatrist diagnosed bipolar disorder and prescribed a mood stabilizer, but she did not take the medication and her psychosis has worsened.

Ms. D’s Mini-Mental State Examination score of 30 indicates that she is neither grossly confused nor has underlying dementia. However, she is emotionally labile with grossly disorganized thought processes and paranoid and grandiose delusions.

We could not locate other family members, so Ms. D’s family psychiatric history is unknown. She has casual relationships with men but does not have a boyfriend. She acknowledges that she frequents local nightclubs but denies using alcohol.

Blood work and other medical examination results are normal. Negative urine toxicology screen suggests she not abusing substances, and electrolytes and thyroid-stimulating hormone levels are normal. Negative rapid plasma reagin rules out tertiary syphilis. We do not order radiologic studies because her presentation does not suggest focal abnormality, and neurologic exam results are benign.

poll here

The authors’ observations

Patients with both paranoid delusions and manic features are challenging. Prognoses and treatment options for each group of symptoms differ substantially.

Ms. D’s grandiosity, pressured speech, tangential flight of ideas, and hypersexuality strongly suggest bipolar disorder. We could not rule out schizophrenia, however, because of her prominent hallucinations and paranoia.

Pharmacologic intoxication was not likely based on laboratory results and the longstanding, progressive course of Ms. D’s disorder. Organic pathology also was unlikely, given her normal neurologic examination and lack of other medical issues.

Treatment: Talk therapy

We tentatively diagnose Ms. D as having bipolar disorder type I with a manic episode and psychotic features. She does not meet DSM-IV-TR criteria for schizophrenia and lacks affective flattening, poverty of speech, avolition, and other negative symptoms typical of the disorder. We admit her to the inpatient psychiatric unit and prescribe lithium, 300 mg tid, and quetiapine, 50 mg bid.

An internal medicine (IM) resident visits Ms. D for 30 to 45 minutes daily during her hospitalization to check her medical status and to allow her to vent her frustration. A resident in psychiatry also interviews Ms. D for about one half-hour each day. The patient rarely interacts with other patients and speaks only with physicians and nurses.

Ms. D appears to trust the IM resident and confides in her about her brother. During their first meeting, she appears most disturbed that a man who “claims” to be her brother is sabotaging her life. She does not fear that this “impostor” will physically harm her but still distrusts him. She repeatedly reports that her late father is nearby or in the room above hers. She adds that she feels much safer in the hospital, where the “stalkers” cannot reach her.

At times, Ms. D tells the IM resident she has a twin. Other times, she believes her family is much larger than it is, and she sometimes laments that she is losing her identity. She often perseverates on Judgment Day, at which time she says her “fake” relatives will answer for their actions against her.

Ms. D’s delusions of grandiosity, tangentiality, circumferential speech, and flight of ideas persist through 4 days in the hospital. Her affect is extremely labile and occasionally inappropriate. She sometimes cries when discussing her father’s death, then stops, thinks a moment, and begins laughing. At this point, we increase lithium to 600 mg tid and quetiapine to 100 mg tid. She is suffering no side effects and infrequently requires haloperidol as a demand dose only.

 

poll here

The authors’ observations

A patient such as Ms. D who lives in a minimally supportive environment and has paranoid delusions could fabricate an explanation for what she perceives as family members’ incongruent behavior. She could create a reality in which these relatives are impostors.

Although this behavior is not unusual, Ms. D’s extreme reaction toward her siblings suggests Capgras syndrome, a rare misidentification disorder (Box). The syndrome is often missed in clinical practice, and its prevalence has not been quantified.

Capgras syndrome is seen most often in patients with paranoid schizophrenia—the highest functioning and most preserved schizophrenia patients. This association may indicate that both neurologic dysfunction and psychological background are necessary to produce the syndrome.

The belief that family members are impostors could point to a conspiracy theory or paranoid delusion. Ms. D’s suspicion and distrust toward her older brother indicate a paranoid state, and her other delusions—such as her belief that others are stalking her—suggest that her Capgras symptoms are another manifestation of paranoia.

Box

Capgras’ causes. Capgras delusions can occur secondary to neurologic lesions and often appear to have an organic cause, such as abnormal focal paroxysmal discharges.¹ These delusions can occur secondary to systemic infections, thyroid dysfunction, seizures, concussion, intoxication dementia, toxic encephalopathy, or head trauma.^1,2 Theories vary as to physiologic, structural, and neurologic causes (Table).

For Ms. D, structural brain deficits probably interacted with her psychosocial milieu to create Capgras delusions, though we did not perform confirmatory brain imaging or functional neurologic testing. Whereas right cortical lesions might impair recognition while preserving familiarity, Capgras syndrome preserves recognition but deadens the emotion that makes faces seem familiar. When focal lesions are found to cause Capgras delusion, however, the right hemisphere—specifically the frontal cortex—usually is affected.^2,3

Table

Proposed causes of Capgras syndrome

Physiologic
Frontal lobe damage may distort visual stimuli monitoring, thus impairing facial recognition.4
Disruption of neuronal connections within the right temporal lobe scrambles memories needed for facial recognition.5
Neurologic
Disconnection between brain hemispheres lead to cognitive but not affective recognition.6
Bifrontal pathology or other organic cause blurs “judgment of individuality or uniqueness,” as in prosopagnosia.3
Dorsal pathway impairment alters affective response to faces.7
Dissociation in the amygdala may distort affective response to faces.8
Psychological*
In depression, misidentification develops secondary to rationalizing feelings of guilt and inferiority.9
“Two-armed recognition”—one automatic and almost instantaneous, the other attentive and mnemonic—begins to falter.10
Suspicion, preoccupation with details leads to “agnosia through too great attention.”11
Avoidance of unconscious desires leads to recognition problems.12
Patient “projects and splits” family member into two persons; directs love toward real person and hate toward imagined impostor.13
In schizophrenia, world is viewed through primitive mechanisms, such as doubles and dualism.14
*Dependent on psychiatric comorbidity

poll here

The authors’ observations

When interviewing a patient with paranoid delusions, get as much detail as possible about his or her close relationships. Try to interview one or two family members or friends. The information can help determine whether Capgras symptoms underlie paranoia.

Brain imaging might uncover pertinent abnormalities, but the cost could outweigh any benefit. No evidence supports use of CT to diagnose Capgras syndrome. Some evidence supports use of brain MRI, but more research is needed.

No specific treatment exists for Capgras delusions apart from using antipsychotics to treat the psychosis based on clinical suspicion and constellation of symptoms.

Studies have shown no difference in response to atypical antipsychotics between patients with schizophrenia and comcomitant Capgras symptoms and those with schizophrenia alone. In clinical practice, we have found that treating Capgras symptoms does improve schizophrenia’s course.

Adjunctive psychotherapy has not been studied in Capgras syndrome, and directed, insight-guided therapy might not resolve deeply rooted delusions for some patients. With Ms. D, however, “talk therapy” helped us build rapport and gave us insight into her strained familial relationships. Establishing a therapeutic alliance with the patient and encouraging healthy relationships with his or her family and friends can mitigate the effects of Capgras paranoia.

 

Continued treatment: Gradual change

Day by day Ms. D’s mania subsides gradually, though she still fears that a stranger posing as her brother is stalking her. She talks about her brother less frequently, though she is clearly holding fast to her delusional beliefs.

We discharge Ms. D after 10 days. Although her symptoms have not resolved, she is markedly less manic and less agitated than at admission. We arrange treatment with outpatient psychiatry. She does not follow up with her original psychiatrist and is lost to follow-up.

Related resources

• PsychNet-UK. Disorder information sheet: Capgras (delusion) syndrome. www.psychnet-uk.com/dsm_iv/capgras_syndrome.htm.
  
• Bourget D, Whitehurst L. Capgras syndrome: a review of the neurophysiological correlates and presenting clinical features in cases involving physical violence. Can J Psychiatry 2004;49:719-25. Available at: www.cpa-apc.org/Publications/Archives/CJP/2004/november/bourget.asp.
  
• Barton JJ. Disorders of face perception and recognition. Neurol Clin 2003;21:521-48.
  
• Lewis S. Brain imaging in a case of Capgras’ syndrome. Br J Psychiatry 1987;150:117-21.
  
• Christodoulou GN. The syndrome of Capgras. Br J Psychiatry 1977;130:556-64.
  

Drug brand names

• Haloperidol • Haldol
  
• Lithium • Eskalith, others
  
• Quetiapine • Seroquel
  

Disclosures

The authors report no financial relationship with any company whose products are mentioned in this article, or with manufacturers of competing products.

HISTORY: A sudden vision

Ms. K, 73, was in reasonably good health when one day she suddenly noticed red, green, and yellow birds and butterflies covering her wall.

Ms. K, who lives alone, was frightened at first, but she did not immediately alert anyone because she thought she “was just seeing things, and they’ll go away.”

Instead, she saw more visions over the next 3 months. She once “watched” as two doctors and a nun carried a middle-aged burn victim into her apartment. She remembers seeing the doctors put a “patch” over the woman’s body. To Ms. K, this experience seemed so shockingly real that she called 911, reporting, “That woman should have been in the hospital!”

She reports that a pack of butterflies once “followed” her to the market. She vividly recalls how they crawled about her shoes and legs as she entered the store. When asked if anyone noticed her insect-covered extremities, she replied matter-of-factly, “Maybe it’s not for them to see, maybe it’s just for me,” as if her hallucinations were a divine gift.

Ms. K’s hallucinations usually occur at home, where she spends most of her time. She says that the images are fleeting, lasting from a few seconds to several minutes, and that the creatures fly silently around her room.

Ms. K’s daughter grew concerned that the hallucinations were increasingly diminishing her mother’s ability to care for herself. She brought Ms. K into our emergency department, from which the patient was admitted.

On admission, Ms. K said she had lost 20 lbs within 6 months, and that “concentrating on those things in the house” was impairing her sleep. She denied recent illness, trauma, loss of conscious ness, changes in medications, seizures, drug or alcohol use, suicidal or homicidal ideation, or specific stress in her life. She added that she often cooks for herself—only to lose her appetite after seeing bugs and other creatures crawl into her food.

Her medical history includes hypertension, type 2 diabetes mellitus, peripheral vascular disease, urinary incontinence, gastroesophageal reflux, glaucoma in her left eye, and bilateral cataracts. She denies any psychiatric history and adds that she had never experienced hallucinations until about 3 months before hospitalization. She also denies any history of auditory, tactile, or olfactory hallucinations.

Would you suspect a primary psychotic illness? What clinical tests might help us understand Ms. K’s progressively debilitating visual hallucinations?

The authors’ observations

Ms. K’s case places us at the crossroads of psychiatric disturbances and medical conditions that can present as or precipitate apparent psychiatric symptoms. Delirium, dementia, psychosis, endocrinopathies, encephalitis, electrolyte disturbances, drug abuse/withdrawal, and occipital or temporal lobe seizures are all possible differential diagnoses (Table 1).

A cognitive function screening and a battery of laboratory tests, imaging scans, and neurologic and vision exams are needed to uncover the cause of her hallucinations.

EVALUATION: Looking for clues

Ms. K’s left pupil was fixed at 6 mm and did not respond to light, while the right pupil was regular and reactive to light at 3 mm. Using a Snellen eye chart, her visual acuity was poor: 20/100 to 20/200 in her right eye and less than 20/200 in the left eye. She scored a 29 out of 30 on the Folstein Mini-Mental State Examination (MMSE), indicating her cognition was intact. The remainder of the neurologic exam was unremarkable.

At admission, Ms. K’s medications included metoprolol, 100 mg qd, for hypertension; lansoprazole, 30 mg qd, for gastroesophageal reflux; tolterodine, 2 mg bid, and oxybutynin, 10 mg qd, for urinary incontinence; repaglinide, 2 mg bid, for type 2 diabetes; and three ophthalmic agents: brimonidine, prednisolone, and dorzolamide/timolol. The patient had been maintained on these medications for more than 2 years with no recent changes in dosing.

Results of Ms. K’s lab studies were normal, including a basic metabolic panel, CBC, liver function tests, urinalysis, B12, thyroid panel, rapid plasma reagin test, and urine drug screen.

A head CT without contrast revealed chronic small-vessel ischemic white matter disease and a chronic infarct of the left cerebellar hemisphere. No acute intracranial hemorrhages, masses, or other abnormalities were noted. No seizures were seen on EEG.

Table 1

Common causes of visual hallucinations

Schizophrenia
Delirium
Dementias
Substance-induced psychosis
Electrolyte disturbances
Occipital and temporal lobe epilepsy
Charles Bonnet syndrome

What do the laboratory and imaging tests reveal about Ms. K’s hallucinations? Is her diagnosis delirium? Alzheimer’s or other type of dementia? Schizophrenia?

The authors’ observations

Visual hallucinations—often of deceased parents or siblings, unknown intruders, and animals—can occur in up to 25% of patients with Alzheimer’s-type dementia.¹ Also, patients with Lewy body dementia often present with well-formed visual hallucinations, which are thought to result from temporal lobe involvement by the characteristic Lewy bodies.

 

To diagnose dementia, DSM-IV requires the presence of multiple cognitive deficits manifested by memory impairment and one or more of the following:

• aphasia
• apraxia
• agnosia
• disturbance of executive functioning.²

Ms. K exhibited none of these characteristics, and she retained full executive function—she could balance her checkbook, buy groceries, and cook for herself. Also, her MMSE score was high.

Ms. K showed no consciousness fluctuations or attention deficits, two features commonly seen in delirium. She was alert and oriented throughout the interview, and her flow of thought, speech, language, and attention were appropriate. Therefore, delirium can be reasonably excluded.

The hallucinations probably do not signal onset of schizophrenia because of Ms. K’s age at presentation, lack of family history of psychotic disorder, and paucity of negative symptoms. Auditory hallucinations are much more common in psychosis, and isolated visual hallucinations rarely occur in schizophrenia.

Finally, Ms. K’s electrophysiologic, laboratory, and imaging studies revealed isolated systolic hypertension, low visual acuity, and a mild gait disturbance. Severe left lens opacification accounted for the patient’s discordant pupillary light reflex. None of these findings explained her visual hallucinations, however.

Is a non-psychiatric disorder causing Ms. K’s hallucinations? What type of medication might alleviate her symptoms?

The authors’ observations

Given Ms. K’s strong cognitive function and poor visual acuity, we concluded that her hallucinations may fit the criteria for Charles Bonnet syndrome (CBS), a poorly understood medical phenomenon.

CBS is characterized by complex visual hallucinations in visually impaired elderly patients without cognitive deficits (Table 2).^3,4 Swiss philosopher Charles Bonnet first described the disorder in 1760 to explain the vivid visual hallucinations of his 89-year-old grandfather, who had severe cataracts but no cognitive deficits.³ Bonnet’s grandfather claimed to have visions of men, women, birds, buildings, and tapestries.³

CBS is increasingly recognized and reported, but the medical community has never formed a universally accepted definition for this phenomenon. Persons with CBS react positively or negatively to their hallucinations, and the images may stimulate anxiety, anger, or mild paranoia. Research has focused on prevalence, risk indicators, and treatment.

Table 2

Charles Bonnet syndrome: fast facts

Visual hallucinations in older, visually impaired persons Gross cognitive deficits not present Prevalence of up to 14% of visually handicapped patients. Images disappear upon eye closure Social isolation may be a risk factor Treatment includes support and reassurance, typical and atypical antipsychotics, anticonvulsants, and 5-HT3 receptor antagonists

Teunisse et al determined that visual hallucinations plague up to 14% of sight-impaired persons.^4,5 The hallucinations vary widely: people, animals, flowers, vehicles, buildings, and sometimes complete scenes.^4,5 Significant risk factors for CBS include advanced age and low visual acuity.^4,5 Loneliness, introversion, and shyness are additional risk indicators in older, visually handicapped persons.⁶ Therefore, social isolation may be a predisposing factor.

Drug treatment of visual hallucinations in CBS currently includes antipsychotics, such as quetiapine (25 to 100 mg/d) and risperidone (0.25 to 1.0 mg/d).⁷ However, mixed results have been reported after use of antipsychotics in CBS; one patient’s visual hallucinations were exacerbated after risperidone was initiated.⁸ Case reports have also described the use of valproate, carbamazepine, and ondansetron in CBS.^9-11

Empathy and patient education are the cornerstones of CBS treatment.³ Patients need to be reassured that their visions are benign. For many, simply increasing the amount of ambient light in the home can reduce hallucinations.

TREATMENT A frog in the toilet

Ms. K was started on quetiapine, 25 mg bid, to try to promote restorative sleep and resolve her hallucinations. Up to 18% of persons treated with quetiapine report somnolence as an adverse effect, vs. 3 to 8% of those treated with risperidone.¹²

During her hospital stay, Ms. K experienced no visual hallucinations during the day but reported seeing a grayish-brown bullfrog in the toilet at night. This hallucination did not frighten her; she would simply close the bathroom door and wait until the bullfrog “disappeared.”

Her sleep improved, as did her appetite. She participated in daily group sessions and socialized with other patients.

After 12 days, Ms. K was discharged. To decrease her social isolation, we encouraged her to participate in a day program for seniors. We also continued her on quetiapine, 25 mg bid.

Five months later, her primary care physician reports that Ms. K remains symptom free while maintaining her quetiapine dosage.

Related resources

• Royal National Institute of the Blind: Fact sheet for Charles Bonnet syndrome. Available at: http://www.rnib.org.uk/info/cbsfin.htm
• Verstraten PFJ. The Charles Bonnet syndrome: Development of a protocol for clinical practice in a multidisciplinary approach from assessment to intervention. Available at: http://www.rehab-syn.enter.iris.se/kc-syn/cb.htm
• Adamczyk DT. Optometric educators. Am I seeing things? Optometry Today June 18, 1999:37-9. Available at: http://www.optometry.co.uk/articles/19990618/Adamczyk.pdf

 

Drug brand names

• Brimonidine Ophthalmic • Alphagan
• Carbamazepine • Tegretol
• Dorzolamide/Timolol • Cosopt
• Lansoprazole • Prevacid
• Metoprolol • Toprol XL
• Ondansetron • Zofran
• Oxybutynin • Ditropan XL
• Quetiapine • Seroquel
• Repaglinide • Prandin
• Risperidone • Risperdal
• Tolterodine • Detrol
• Valproate • Depakote

Disclosure

The authors report no financial relationship with any company whose products are mentioned in this article or with competing manufacturers.

HISTORY: A sudden vision

Ms. K, 73, was in reasonably good health when one day she suddenly noticed red, green, and yellow birds and butterflies covering her wall.

Ms. K, who lives alone, was frightened at first, but she did not immediately alert anyone because she thought she “was just seeing things, and they’ll go away.”

Instead, she saw more visions over the next 3 months. She once “watched” as two doctors and a nun carried a middle-aged burn victim into her apartment. She remembers seeing the doctors put a “patch” over the woman’s body. To Ms. K, this experience seemed so shockingly real that she called 911, reporting, “That woman should have been in the hospital!”

She reports that a pack of butterflies once “followed” her to the market. She vividly recalls how they crawled about her shoes and legs as she entered the store. When asked if anyone noticed her insect-covered extremities, she replied matter-of-factly, “Maybe it’s not for them to see, maybe it’s just for me,” as if her hallucinations were a divine gift.

Ms. K’s hallucinations usually occur at home, where she spends most of her time. She says that the images are fleeting, lasting from a few seconds to several minutes, and that the creatures fly silently around her room.

Ms. K’s daughter grew concerned that the hallucinations were increasingly diminishing her mother’s ability to care for herself. She brought Ms. K into our emergency department, from which the patient was admitted.

On admission, Ms. K said she had lost 20 lbs within 6 months, and that “concentrating on those things in the house” was impairing her sleep. She denied recent illness, trauma, loss of conscious ness, changes in medications, seizures, drug or alcohol use, suicidal or homicidal ideation, or specific stress in her life. She added that she often cooks for herself—only to lose her appetite after seeing bugs and other creatures crawl into her food.

Her medical history includes hypertension, type 2 diabetes mellitus, peripheral vascular disease, urinary incontinence, gastroesophageal reflux, glaucoma in her left eye, and bilateral cataracts. She denies any psychiatric history and adds that she had never experienced hallucinations until about 3 months before hospitalization. She also denies any history of auditory, tactile, or olfactory hallucinations.

Would you suspect a primary psychotic illness? What clinical tests might help us understand Ms. K’s progressively debilitating visual hallucinations?

The authors’ observations

Ms. K’s case places us at the crossroads of psychiatric disturbances and medical conditions that can present as or precipitate apparent psychiatric symptoms. Delirium, dementia, psychosis, endocrinopathies, encephalitis, electrolyte disturbances, drug abuse/withdrawal, and occipital or temporal lobe seizures are all possible differential diagnoses (Table 1).

A cognitive function screening and a battery of laboratory tests, imaging scans, and neurologic and vision exams are needed to uncover the cause of her hallucinations.

EVALUATION: Looking for clues

Ms. K’s left pupil was fixed at 6 mm and did not respond to light, while the right pupil was regular and reactive to light at 3 mm. Using a Snellen eye chart, her visual acuity was poor: 20/100 to 20/200 in her right eye and less than 20/200 in the left eye. She scored a 29 out of 30 on the Folstein Mini-Mental State Examination (MMSE), indicating her cognition was intact. The remainder of the neurologic exam was unremarkable.

At admission, Ms. K’s medications included metoprolol, 100 mg qd, for hypertension; lansoprazole, 30 mg qd, for gastroesophageal reflux; tolterodine, 2 mg bid, and oxybutynin, 10 mg qd, for urinary incontinence; repaglinide, 2 mg bid, for type 2 diabetes; and three ophthalmic agents: brimonidine, prednisolone, and dorzolamide/timolol. The patient had been maintained on these medications for more than 2 years with no recent changes in dosing.

Results of Ms. K’s lab studies were normal, including a basic metabolic panel, CBC, liver function tests, urinalysis, B12, thyroid panel, rapid plasma reagin test, and urine drug screen.

A head CT without contrast revealed chronic small-vessel ischemic white matter disease and a chronic infarct of the left cerebellar hemisphere. No acute intracranial hemorrhages, masses, or other abnormalities were noted. No seizures were seen on EEG.

Table 1

Common causes of visual hallucinations

Schizophrenia
Delirium
Dementias
Substance-induced psychosis
Electrolyte disturbances
Occipital and temporal lobe epilepsy
Charles Bonnet syndrome

What do the laboratory and imaging tests reveal about Ms. K’s hallucinations? Is her diagnosis delirium? Alzheimer’s or other type of dementia? Schizophrenia?

The authors’ observations

Visual hallucinations—often of deceased parents or siblings, unknown intruders, and animals—can occur in up to 25% of patients with Alzheimer’s-type dementia.¹ Also, patients with Lewy body dementia often present with well-formed visual hallucinations, which are thought to result from temporal lobe involvement by the characteristic Lewy bodies.

 

To diagnose dementia, DSM-IV requires the presence of multiple cognitive deficits manifested by memory impairment and one or more of the following:

• aphasia
• apraxia
• agnosia
• disturbance of executive functioning.²

Ms. K exhibited none of these characteristics, and she retained full executive function—she could balance her checkbook, buy groceries, and cook for herself. Also, her MMSE score was high.

Ms. K showed no consciousness fluctuations or attention deficits, two features commonly seen in delirium. She was alert and oriented throughout the interview, and her flow of thought, speech, language, and attention were appropriate. Therefore, delirium can be reasonably excluded.

The hallucinations probably do not signal onset of schizophrenia because of Ms. K’s age at presentation, lack of family history of psychotic disorder, and paucity of negative symptoms. Auditory hallucinations are much more common in psychosis, and isolated visual hallucinations rarely occur in schizophrenia.

Finally, Ms. K’s electrophysiologic, laboratory, and imaging studies revealed isolated systolic hypertension, low visual acuity, and a mild gait disturbance. Severe left lens opacification accounted for the patient’s discordant pupillary light reflex. None of these findings explained her visual hallucinations, however.

Is a non-psychiatric disorder causing Ms. K’s hallucinations? What type of medication might alleviate her symptoms?

The authors’ observations

Given Ms. K’s strong cognitive function and poor visual acuity, we concluded that her hallucinations may fit the criteria for Charles Bonnet syndrome (CBS), a poorly understood medical phenomenon.

CBS is characterized by complex visual hallucinations in visually impaired elderly patients without cognitive deficits (Table 2).^3,4 Swiss philosopher Charles Bonnet first described the disorder in 1760 to explain the vivid visual hallucinations of his 89-year-old grandfather, who had severe cataracts but no cognitive deficits.³ Bonnet’s grandfather claimed to have visions of men, women, birds, buildings, and tapestries.³

CBS is increasingly recognized and reported, but the medical community has never formed a universally accepted definition for this phenomenon. Persons with CBS react positively or negatively to their hallucinations, and the images may stimulate anxiety, anger, or mild paranoia. Research has focused on prevalence, risk indicators, and treatment.

Table 2

Charles Bonnet syndrome: fast facts

Visual hallucinations in older, visually impaired persons Gross cognitive deficits not present Prevalence of up to 14% of visually handicapped patients. Images disappear upon eye closure Social isolation may be a risk factor Treatment includes support and reassurance, typical and atypical antipsychotics, anticonvulsants, and 5-HT3 receptor antagonists

Teunisse et al determined that visual hallucinations plague up to 14% of sight-impaired persons.^4,5 The hallucinations vary widely: people, animals, flowers, vehicles, buildings, and sometimes complete scenes.^4,5 Significant risk factors for CBS include advanced age and low visual acuity.^4,5 Loneliness, introversion, and shyness are additional risk indicators in older, visually handicapped persons.⁶ Therefore, social isolation may be a predisposing factor.

Drug treatment of visual hallucinations in CBS currently includes antipsychotics, such as quetiapine (25 to 100 mg/d) and risperidone (0.25 to 1.0 mg/d).⁷ However, mixed results have been reported after use of antipsychotics in CBS; one patient’s visual hallucinations were exacerbated after risperidone was initiated.⁸ Case reports have also described the use of valproate, carbamazepine, and ondansetron in CBS.^9-11

Empathy and patient education are the cornerstones of CBS treatment.³ Patients need to be reassured that their visions are benign. For many, simply increasing the amount of ambient light in the home can reduce hallucinations.

TREATMENT A frog in the toilet

Ms. K was started on quetiapine, 25 mg bid, to try to promote restorative sleep and resolve her hallucinations. Up to 18% of persons treated with quetiapine report somnolence as an adverse effect, vs. 3 to 8% of those treated with risperidone.¹²

During her hospital stay, Ms. K experienced no visual hallucinations during the day but reported seeing a grayish-brown bullfrog in the toilet at night. This hallucination did not frighten her; she would simply close the bathroom door and wait until the bullfrog “disappeared.”

Her sleep improved, as did her appetite. She participated in daily group sessions and socialized with other patients.

After 12 days, Ms. K was discharged. To decrease her social isolation, we encouraged her to participate in a day program for seniors. We also continued her on quetiapine, 25 mg bid.

Five months later, her primary care physician reports that Ms. K remains symptom free while maintaining her quetiapine dosage.

Related resources

• Royal National Institute of the Blind: Fact sheet for Charles Bonnet syndrome. Available at: http://www.rnib.org.uk/info/cbsfin.htm
• Verstraten PFJ. The Charles Bonnet syndrome: Development of a protocol for clinical practice in a multidisciplinary approach from assessment to intervention. Available at: http://www.rehab-syn.enter.iris.se/kc-syn/cb.htm
• Adamczyk DT. Optometric educators. Am I seeing things? Optometry Today June 18, 1999:37-9. Available at: http://www.optometry.co.uk/articles/19990618/Adamczyk.pdf

 

Drug brand names

• Brimonidine Ophthalmic • Alphagan
• Carbamazepine • Tegretol
• Dorzolamide/Timolol • Cosopt
• Lansoprazole • Prevacid
• Metoprolol • Toprol XL
• Ondansetron • Zofran
• Oxybutynin • Ditropan XL
• Quetiapine • Seroquel
• Repaglinide • Prandin
• Risperidone • Risperdal
• Tolterodine • Detrol
• Valproate • Depakote

Disclosure

The authors report no financial relationship with any company whose products are mentioned in this article or with competing manufacturers.

HISTORY: A sudden vision

Ms. K, 73, was in reasonably good health when one day she suddenly noticed red, green, and yellow birds and butterflies covering her wall.

Ms. K, who lives alone, was frightened at first, but she did not immediately alert anyone because she thought she “was just seeing things, and they’ll go away.”

Instead, she saw more visions over the next 3 months. She once “watched” as two doctors and a nun carried a middle-aged burn victim into her apartment. She remembers seeing the doctors put a “patch” over the woman’s body. To Ms. K, this experience seemed so shockingly real that she called 911, reporting, “That woman should have been in the hospital!”

She reports that a pack of butterflies once “followed” her to the market. She vividly recalls how they crawled about her shoes and legs as she entered the store. When asked if anyone noticed her insect-covered extremities, she replied matter-of-factly, “Maybe it’s not for them to see, maybe it’s just for me,” as if her hallucinations were a divine gift.

Ms. K’s hallucinations usually occur at home, where she spends most of her time. She says that the images are fleeting, lasting from a few seconds to several minutes, and that the creatures fly silently around her room.

Ms. K’s daughter grew concerned that the hallucinations were increasingly diminishing her mother’s ability to care for herself. She brought Ms. K into our emergency department, from which the patient was admitted.

On admission, Ms. K said she had lost 20 lbs within 6 months, and that “concentrating on those things in the house” was impairing her sleep. She denied recent illness, trauma, loss of conscious ness, changes in medications, seizures, drug or alcohol use, suicidal or homicidal ideation, or specific stress in her life. She added that she often cooks for herself—only to lose her appetite after seeing bugs and other creatures crawl into her food.

Her medical history includes hypertension, type 2 diabetes mellitus, peripheral vascular disease, urinary incontinence, gastroesophageal reflux, glaucoma in her left eye, and bilateral cataracts. She denies any psychiatric history and adds that she had never experienced hallucinations until about 3 months before hospitalization. She also denies any history of auditory, tactile, or olfactory hallucinations.

Would you suspect a primary psychotic illness? What clinical tests might help us understand Ms. K’s progressively debilitating visual hallucinations?

The authors’ observations

Ms. K’s case places us at the crossroads of psychiatric disturbances and medical conditions that can present as or precipitate apparent psychiatric symptoms. Delirium, dementia, psychosis, endocrinopathies, encephalitis, electrolyte disturbances, drug abuse/withdrawal, and occipital or temporal lobe seizures are all possible differential diagnoses (Table 1).

A cognitive function screening and a battery of laboratory tests, imaging scans, and neurologic and vision exams are needed to uncover the cause of her hallucinations.

EVALUATION: Looking for clues

Ms. K’s left pupil was fixed at 6 mm and did not respond to light, while the right pupil was regular and reactive to light at 3 mm. Using a Snellen eye chart, her visual acuity was poor: 20/100 to 20/200 in her right eye and less than 20/200 in the left eye. She scored a 29 out of 30 on the Folstein Mini-Mental State Examination (MMSE), indicating her cognition was intact. The remainder of the neurologic exam was unremarkable.

At admission, Ms. K’s medications included metoprolol, 100 mg qd, for hypertension; lansoprazole, 30 mg qd, for gastroesophageal reflux; tolterodine, 2 mg bid, and oxybutynin, 10 mg qd, for urinary incontinence; repaglinide, 2 mg bid, for type 2 diabetes; and three ophthalmic agents: brimonidine, prednisolone, and dorzolamide/timolol. The patient had been maintained on these medications for more than 2 years with no recent changes in dosing.

Results of Ms. K’s lab studies were normal, including a basic metabolic panel, CBC, liver function tests, urinalysis, B12, thyroid panel, rapid plasma reagin test, and urine drug screen.

A head CT without contrast revealed chronic small-vessel ischemic white matter disease and a chronic infarct of the left cerebellar hemisphere. No acute intracranial hemorrhages, masses, or other abnormalities were noted. No seizures were seen on EEG.

Table 1

Common causes of visual hallucinations

Schizophrenia
Delirium
Dementias
Substance-induced psychosis
Electrolyte disturbances
Occipital and temporal lobe epilepsy
Charles Bonnet syndrome

What do the laboratory and imaging tests reveal about Ms. K’s hallucinations? Is her diagnosis delirium? Alzheimer’s or other type of dementia? Schizophrenia?

The authors’ observations

Visual hallucinations—often of deceased parents or siblings, unknown intruders, and animals—can occur in up to 25% of patients with Alzheimer’s-type dementia.¹ Also, patients with Lewy body dementia often present with well-formed visual hallucinations, which are thought to result from temporal lobe involvement by the characteristic Lewy bodies.

 

To diagnose dementia, DSM-IV requires the presence of multiple cognitive deficits manifested by memory impairment and one or more of the following:

• aphasia
• apraxia
• agnosia
• disturbance of executive functioning.²

Ms. K exhibited none of these characteristics, and she retained full executive function—she could balance her checkbook, buy groceries, and cook for herself. Also, her MMSE score was high.

Ms. K showed no consciousness fluctuations or attention deficits, two features commonly seen in delirium. She was alert and oriented throughout the interview, and her flow of thought, speech, language, and attention were appropriate. Therefore, delirium can be reasonably excluded.

The hallucinations probably do not signal onset of schizophrenia because of Ms. K’s age at presentation, lack of family history of psychotic disorder, and paucity of negative symptoms. Auditory hallucinations are much more common in psychosis, and isolated visual hallucinations rarely occur in schizophrenia.

Finally, Ms. K’s electrophysiologic, laboratory, and imaging studies revealed isolated systolic hypertension, low visual acuity, and a mild gait disturbance. Severe left lens opacification accounted for the patient’s discordant pupillary light reflex. None of these findings explained her visual hallucinations, however.

Is a non-psychiatric disorder causing Ms. K’s hallucinations? What type of medication might alleviate her symptoms?

The authors’ observations

Given Ms. K’s strong cognitive function and poor visual acuity, we concluded that her hallucinations may fit the criteria for Charles Bonnet syndrome (CBS), a poorly understood medical phenomenon.

CBS is characterized by complex visual hallucinations in visually impaired elderly patients without cognitive deficits (Table 2).^3,4 Swiss philosopher Charles Bonnet first described the disorder in 1760 to explain the vivid visual hallucinations of his 89-year-old grandfather, who had severe cataracts but no cognitive deficits.³ Bonnet’s grandfather claimed to have visions of men, women, birds, buildings, and tapestries.³

CBS is increasingly recognized and reported, but the medical community has never formed a universally accepted definition for this phenomenon. Persons with CBS react positively or negatively to their hallucinations, and the images may stimulate anxiety, anger, or mild paranoia. Research has focused on prevalence, risk indicators, and treatment.

Table 2

Charles Bonnet syndrome: fast facts

Visual hallucinations in older, visually impaired persons Gross cognitive deficits not present Prevalence of up to 14% of visually handicapped patients. Images disappear upon eye closure Social isolation may be a risk factor Treatment includes support and reassurance, typical and atypical antipsychotics, anticonvulsants, and 5-HT3 receptor antagonists

Teunisse et al determined that visual hallucinations plague up to 14% of sight-impaired persons.^4,5 The hallucinations vary widely: people, animals, flowers, vehicles, buildings, and sometimes complete scenes.^4,5 Significant risk factors for CBS include advanced age and low visual acuity.^4,5 Loneliness, introversion, and shyness are additional risk indicators in older, visually handicapped persons.⁶ Therefore, social isolation may be a predisposing factor.

Drug treatment of visual hallucinations in CBS currently includes antipsychotics, such as quetiapine (25 to 100 mg/d) and risperidone (0.25 to 1.0 mg/d).⁷ However, mixed results have been reported after use of antipsychotics in CBS; one patient’s visual hallucinations were exacerbated after risperidone was initiated.⁸ Case reports have also described the use of valproate, carbamazepine, and ondansetron in CBS.^9-11

Empathy and patient education are the cornerstones of CBS treatment.³ Patients need to be reassured that their visions are benign. For many, simply increasing the amount of ambient light in the home can reduce hallucinations.

TREATMENT A frog in the toilet

Ms. K was started on quetiapine, 25 mg bid, to try to promote restorative sleep and resolve her hallucinations. Up to 18% of persons treated with quetiapine report somnolence as an adverse effect, vs. 3 to 8% of those treated with risperidone.¹²

During her hospital stay, Ms. K experienced no visual hallucinations during the day but reported seeing a grayish-brown bullfrog in the toilet at night. This hallucination did not frighten her; she would simply close the bathroom door and wait until the bullfrog “disappeared.”

Her sleep improved, as did her appetite. She participated in daily group sessions and socialized with other patients.

After 12 days, Ms. K was discharged. To decrease her social isolation, we encouraged her to participate in a day program for seniors. We also continued her on quetiapine, 25 mg bid.

Five months later, her primary care physician reports that Ms. K remains symptom free while maintaining her quetiapine dosage.

Related resources

• Royal National Institute of the Blind: Fact sheet for Charles Bonnet syndrome. Available at: http://www.rnib.org.uk/info/cbsfin.htm
• Verstraten PFJ. The Charles Bonnet syndrome: Development of a protocol for clinical practice in a multidisciplinary approach from assessment to intervention. Available at: http://www.rehab-syn.enter.iris.se/kc-syn/cb.htm
• Adamczyk DT. Optometric educators. Am I seeing things? Optometry Today June 18, 1999:37-9. Available at: http://www.optometry.co.uk/articles/19990618/Adamczyk.pdf

 

Drug brand names

• Brimonidine Ophthalmic • Alphagan
• Carbamazepine • Tegretol
• Dorzolamide/Timolol • Cosopt
• Lansoprazole • Prevacid
• Metoprolol • Toprol XL
• Ondansetron • Zofran
• Oxybutynin • Ditropan XL
• Quetiapine • Seroquel
• Repaglinide • Prandin
• Risperidone • Risperdal
• Tolterodine • Detrol
• Valproate • Depakote

Disclosure

The authors report no financial relationship with any company whose products are mentioned in this article or with competing manufacturers.