Ever Wonder if Your Mom was a Double? Part 3

Treatment for Capgras Syndrome

When looking for treatments of Capgras Syndrome, the search yielded little results. Most of the research offered psychodynamic interventions for patients suffering from Capgras Syndrome. The lack of neurological treatment for CS is largely due to the fact that not much is known organically about CS. In the past CS was thought to be purely a psychological issue, therefore explanations and subsequent treatments have been based on psychodynamic theory. Yet there is much debate over etiology, therefore, much research on psychopathology and neuropathology has continued. CS is usually co-morbid with schizophrenia and other mental illnesses. Treatment typically follows a therapeutic regimen of antipsychotic medication and supportive therapy in which areas of cognitive-behavioral strength are used to overcome weaker areas of functioning.  

Evidence is lacking that medications, principally antipsychotics, are effective in treating delusional disorder, yet delusions in the context of another psychotic disorder, such as schizophrenia, may respond better to treatment. It is necessary to note that subtypes of delusional disorder as specified in the Diagnostic and Statistical Manual of Mental Disorders (4th ed.; American Psychiatric Association, 1994) tend in general to be resistant to treatment. Particularly when assessing geriatric patients with delusions, it is important to consider delirium, dementia, and other organic etiologies underlying the thought disturbance.

Antipsychotics: 1^st Generation

The first-generation diphenylbutylpiperidine agent Pimozide (Orap) has been most closely studied in the context of the broader category of schizophrenia-like disorders but has been suggested by some to be differentially effective in the management of delusional disorder. Again, however, systematic trials are lacking (Manschreck, 2000) and initiation of the use of Pimozide to treat at least one form of delusional disorder (somatic delusions) has been traced to a case report involving no more than 5 patients (Elmer, George, & Peterson, 2000). These authors detailed the numerous adverse side effects associated with Pimozide use, particularly extrapyramidal reactions, the risks of Tardive Dyskinesia, and, notably, cardiac conduction defects. Other authors have not found Pimozide to be an effective agent in management of delusional disorder (Silva et al, 1998). A recent Cochrane review (Sultana & McMonagle, 2000) found that the use of Pimozide did prolong time to relapse in a range of psychotic disorders, including delusional disorder, although with a relatively wide confidence interval.

The cardiac side-effect profile of Pimozide is significant, and the U.S. Food and Drug Administration (FDA) has recently instructed the manufacturers of Pimozide to expand the warning label to reflect the risk of cardiac events (prolongation of the QT interval) when this medication is combined with numerous other compounds. This letter also addresses the risks of using Pimozide in patients with imbalances in serum potassium or magnesium (Food and Drug Administration, 2002). Baseline electrocardiography and special informed consent are recommended prior to its use.

Antipsychotics: 2^nd Generation

DeVane & Mintzer, (2003) reviewed studies which tested the efficacy of the drug Risperidone in a geriatric population with dementia, schizophrenia, delusional disorder, and other psychological and neurological disorder. There were some positive results, yet as Devane and Mintzer pointed out there were several problems with these studies that may invalidate their positive results. Just a few examples of the problems these studies had, the population were on several other drugs, the reductions of symptoms noted in the studies were of aggression and agitation and not of psychotic symptoms. DeVane and Mintzer (2003) did cite one study where adults and elderly patients were given low doses of Risperidone and rated on their improvement. On average, patients with schizophrenia, schizophreniform and delusional disorder were rated by therapists as having improved.

Lee et al. (2004) also did a meta-analysis of the research being done on Second Generation Atypical Antipsychotics, and found mixed results. Some studies showed a superiority of SGA’s to placebo, other studies showed little difference between second and first antipsychotics and others that found positive results. Their conclusion was that the evidence was mixed and in desperate need of further investigation before SGA’s can be recommended as a preferred treatment.

Even though atypical (i.e., second-generation) antipsychotics are increasingly prescribed for delusions or other behavioral or psychological manifestations of dementia and other neurological disease, none are indicated for this condition in the United States. These cautions notwithstanding, a recently published Expert Consensus Guideline for management of delusional disorder in older persons recommended risperidone, 0.75–2.5 mg/day, as the preferred treatment. Olanzapine, 5–10 mg/day, and quetiapine, 50–200 mg/day, were also listed as “high second line” treatments. Clozapine and first-generation antipsychotics were listed as third line treatments (Alexopoulos, Strehm, Carpenter, & Docherty, 2004).

Since SGA’s have low side effects much research has been done on this drug class, yet mostly with elderly populations. This is because elderly patients who have been unsuccessful with other medications have shown positive responses to SGA’s. Yet, SGA’s are not the perfect medication; they do cause weight and increase the risk for diabetes, which are huge risk factors for elderly patients. Their also associated with cardiac conduction problems, sedation, and orthostatic hypotension.

            Psychotropics have been shown not to be an effective medication for treating delusions. Because some patients with severe depression may express delusions (Maina, Albert, Bada, & Bogetto, 2001), and because delusions may have a strikingly obsessive quality to them, some authors have recommended treatment with antidepressants, particularly the selective serotonin reuptake inhibitors. This application, however, has not received systematic review, and the literature is limited to a handful of case studies. In a small case series (n = 4), patients with somatic delusions responded to the antidepressant clomipramine (Wada, Kawakatsu, Nadaoka, Okuyama, & Otani, 1999). As is the case for pharmacological treatment of delusional disorder, systematized studies of nondrug interventions are few. Some authors have presented data suggesting that cognitive-behavioral treatment was efficacious in treating delusions, but much of this is also based on single case studies (Garcelan & Yust, 2000).

Other authors have found that treatment of various psychoses, including delusional  psychosis, with an integrated model utilizing psychological and psychosocial treatments with a minimal medication regimen resulted in outcomes that were equal to or somewhat better than outcomes for patients treated with a standard medication regime. Lehtinen, Aaltonen, Koffert, Raekkoelaeinen, and Syvaelahti (2000) found that provision of an integrated treatment model allowed a much larger percentage of patients (42.9%) to avoid medication after the initial course of treatment. In contrast, only 5.9% of those in standard treatment groups required no medication.

We have only general guidelines to guide the use of psychopharmacology in delusional disorder. Limited data suggest that the disorder is not particularly responsive, and that response is not dose-related. It seems that low dose may be the best route for patients. The few case studies that address combined treatments suggest that patients have more sustained response and require less medication than those treated with drugs alone. There are not, however, standard rules that suggest the most efficacious integrated treatment regimens. Further study of the relative contributions of psychotherapy and medication in managing this challenging disorder are needed. However, it seems that an integrated treatment model have proven to be the most helpful thus far.

References

Alexopoulos, G. S., Strehm, J., Carpenter, D., & Docherty, J. P. ( 2004). The expert consensus guideline series: Using antipsychotic agents in older patients. Journal of Clinical Psychiatry, 65, (Suppl.2) 1– 41.

American Diabetes Association, et al. ( 2004). Consensus development conference on antipsychotic drugs and obesity and diabetes. Journal of Clinical Psychiatry, 65, 267– 272.

American Psychiatric Association. ( 1994). Diagnostic and statistical manual of mental disorders ( 4th ed.). Washington, DC: Author.

Alexander, M.P., Stuss, D.T., & Benson, D.F. (1979). Capgras's syndrome: A reduplicative phenomenon, Neurology, 29, 334-339.

Anderson, J.R. (1980). Cognitive psychology and its implications, San Francisco: W.H. Freeman.

Bekelman, D. B., & Hallenbeck, J. (2006). Capgras syndrome associated with morphine treatment, Journal of Palliative Medicine, 9, 810-813.

Berson, R. (1983). Capgras syndrome, American Journal of Psychiatry, 140, 969-978.

Berrios, G. E., Luque, R. (1995). Cotard’s syndrome: analysis of 100 cases, Acta

Psychiatr Scand, 91, 185-8.

Bourget, D., Whitehurst, L. (2004). Capgras syndrome: a review of the neurophysiological correlates and presenting clinical features in cases involving physical violence, Can J Psychiatry, 49, 719-25.

Brighetti, G., Bonifacci, P., Borlimi, R., & Ottaviani, C. (2007). ‘‘Far from the heart far from the eye’’: Evidence from the Capgras delusion, Cognitive Neuropsychiatry, 12, 189-197.

Christodoulou, G.N. (1977b). Treatment of the syndrome of doubles, Acta Psychiatrica Belgique, 77, 254-259.

DeVane, C. L., & Mintzer, J. ( 2003). Risperidone in the management of psychiatric and neurodegenerative disease in the elderly: An update. Psychopharmacology Bulletin, 31, 116– 132.

Doran, J. M. (1990). The Capgras syndrome: Neurological/neuropsychological perspectives, Neuropsychology, 4, 29-42.

Elmer, K. B., George, R. M., & Peterson, K. ( 2000). Therapeutic update: Use of Risperidone for the treatment of monosymptomatic hypochondriacal psychosis. Journal of the American Academy of Dermatology, 43, 683– 686.

Ellis, H. D., Lewis, M. B., Moselhy, H., & Young, A. W. (2000). Automatic without autonomic responses to familiar faces: Differential components of implicit face recognition in a case of Capgras delusion, Cognitive Neuropsychiatry, 5, 255 -269.

Enoch, D., Ball, H. (2001). Uncommon psychiatric syndromes, 4th edn. London : Arnold , 1–19.

Fialkov, M. J. and Robins, A. H. (1978). An unusual case of the Capgras syndrome, British Journal of Psychiatry, 132, 403-404.

Food and Drug Administration. ( 2002, Nov. 22). Letter to Teva Pharmaceuticals. Retrieved February 7, 2005, from www.fda.gov/cder/foi/appletter/2002/17473slr038ltr.pdf

Garcelan, S. P., & Yust, C. C. ( 2000). Effectiveness of individual cognitive-behavioural therapy applied to psychotic symptoms: Review of single case experimental designs applied to the treatment of delusional beliefs. Psychology in Spain, 4, 3– 12.

Gainotti, G. (1972). Emotional behavior and hemispheric side of the lesion, Cortex, 8, 41-55.

Gluckman, I.K. (1968). A case of Capgras syndrome, Australian and New Zealand Journal of Psychiatry, 2, 39-43.

Hacaen, H., & Angelergues, R. (1962). Agnosia for faces (prosopagnosia), Archives of Neurology, 7, 92-100.

Hayman, M.K., & Abrams, R. (1977). Capgras syndrome and cerebral dysfunction, British Journal of Psychiatry, 130, 68-71.

Koritar, E., & Steiner, W. (1988). Capgras syndrome: A synthesis of various viewpoints, Canadian Journal of Psychiatry, 33, 62-66.

Lee, P. E., Gill, S. S., Freedman, M., Bronskill, S. E., Hillmer, M. P., & Rochon, P. A. (2004). Atypical antipsychotic drugs in the treatment of behavioral and psychological symptoms of dementia:Systematic review. British Medical Journal, 329, 75– 78.

Lehtinen, V., Aaltonen, J., Koffert, T., Rakekkoelaeinen, V., & Syvaelahti, E. ( 2000). Two year outcome in first-episode psychosis treated according to an integrated model: Is immediate neuroleptization always needed?European Psychiatry, 15, 312– 320.

MacCallum, W. A.G. (1973). Capgras syndrome with an organic basis, British Journal of Psychiatry, 123, 639-642.

Manschreck, T. C. ( 2000). Delusional disorder and shared psychotic disorder. In B. J.Sadock & V. A.Sadock, Comprehensive textbook of psychiatry ( 7th ed., (pp. 1243– 1264). Baltimore: Williams & Wilkins.

Maina, G., Albert, U., Bada, A., & Bogetto, F. ( 2001). Occurrence and clinical correlates of psychiatric co-morbidity in delusional disorder. European Psychiatry, 16, 222– 228.

Milner, B. (1968). Visual recognition and recall after right temporal lobe excision in man,

Neuropsychologia, 6, 191-209.

Mullan, S., & Penfield, W. (1959). Illusions of comaprative interpretation and emotion, Archives of Neurology and Psychiatry, 81, 269-284.

Nejad A.G., Toofani K. (2006). A variant of Capgras syndrome with delusional conviction of inanimate doubles in a patient with grandmal epilepsy, Acta Neuropsychiatrica, 18, 52–54.

Paterson, A., & Zangwill, O.L. (1944). Disorders of visual spatial perception associated with lesions of the right cerebral hemisphere, Brain, 67, 331-358.

Pick, A. (1903). Zur pathologie des bekanntheitsgefu¨ hls (Bekanntheitsqualita¨ t), Neurol Centralblatt, 22, 2–7.

Ramachandran, V. S., & Blakeslee, S. (1998). Phantoms in the brain, Quill, 158-173.

Quinn, D. (1981). The Capgras syndrome: Two case reports and a review, Canadian Journal of Psychiatry, 26, 126-129.

Sautter, S. W., & Farkas, L. B. K. (1991). A neuropsychological profile of Capgras syndrome, Neuropsychology, 5, 139-150.

Schraberg, D., & Weitzel, W.D. (1979). Prosopagnosia and the Capgras syndrome, Journal of Clinical Psychiatry, 40, 313-316.

Schweinberger, R., & Burton , M. (2003). Covert recognition and the neural system for face processing, Cortex, 39, 9-30.

Sinkman, A. (2008). The syndrome of Capgras, Psychiatry, 71,371-378.

Silva, H., Jerez, S., Ramirez, A., Renteria, P., Aravena, N., Salazar, D., & LaBarca, R. ( 1998). Effects of pimozide on the psychopathology of delusional disorder. Progress in Neuropsychopharmacology and Biological Psychiatry, 22, 331– 340.

Staton, R.D., Brumback, R.A., & Wilson, H. (1982). Reduplicative paramnesia: A disconnection syndrome of memory, Cortex, 18, 23-36.

Stewart, J. T. (2004) Capgras syndrome related to diazepam treatment, Southern Medical Journal, 97, p65-66.

Sultana, A., & McMonagle, T. ( 2000). Pimozide for schizophrenia or related psychoses. In Cochrane Database of Systematic Reviews, 2000: Vol. 2Retrieved from www.cochran.org

Synodinou, C., Christodoulou, G.N., & Tzavaros, A. (1977). Capgras' syndrome and prosopagnosia, British Journal of Psychiatry, 132, 413-414.

Wada, T., Kawakatsu, S., Nadaoka, T., Okuyama, N., & Otani, K. (1999). Clomipramine treatment of delusional disorder, somatic type. International Clinical Psychopharmacology, 14, 181– 183.

Weston, M.J., & Whitlock, F.A. (1971). The Capgras syndrome following head injury, British Journal of Psychiatry, 119, 25-31.

Yalin, S., Varol Tas, F., Guvenir, T. (2008). The coexistence of Capgras, Fregoli and Cotard’s syndromes in an adolescent case, Archives of Neuropsychiatry, 45, 149-151.

Ever Wonder if You're Mom was Double? Part 2

Search for an Explanation

        What makes CS such an interesting subject for neuroscientists is the fact that CS patients believe that loved ones such as family members, long-time friends, and close co-workers have been replaced. But this delusional belief of doubles or impostors does not extend to those who they have no emotional connection with. In most CS cases the patients do not regard acquaintances, nurses, grocery store clerks or anyone else that they do not have a strong emotional tie with as a double or impostor (Ramachandran, 1998). Furthermore, in most CS case reports, patients believe that they are addressing their actual loved ones or family members when speaking to them on the phone (Yalin et al, 2008; Ramachandran, 1998). This point was further illustrated by a study done by Pick (1903), in which he studied a young man who believed that his mother had been replaced by an impostor when he looked at her. However, “her voice still elicited a strong feeling of familiarity.” This suggests that the cause of CS may be contained to an optical malfunction or lesion in the visual pathway; since patients do not experience any CS symptoms when listening to their loved ones.

        Ellis, Lewis, Moselhy & Young, (2000) and Schweinberger & Burton, (2003) wanted to see if there was any validity to the optical theory. They discovered that individuals with Capgras syndrome do not have any optical impairment. If patients did this finding would explain their inability to recognize family members, friends and co-workers faces. But the research showed that they do recognize faces; however, they lack the ability to match a face that they recognized with the any emotions. “Opposite to the pattern observed in prosopoagnosia, Capgras subjects recognize the structural features of familiar faces but may have impairments in the affective route to face recognition as documented by the SCR hyporesponsiveness”( Brighetti, Bonifacci, Borlimi & Ottaviani, 2007). Ellis et al. and Schweinberger et al. demonstrated that CS patients have no optical malfunctions and that they can recognize loved ones faces normally. CS patients possess the ability of emotional expression and of facial recognition; however, there seems to be a severing in the link between the primary emotional and recognition centers. In his book, “Phantoms in the Brain”, Ramachandran proposes his own theory for CS. He thought there was a neurological disconnection between temporal lobe areas involved with facial recognition, such as the Hippocampus, and the emotional center in the brain, the amygdala. Along with Ramachandran many other neuroscientists are arriving at similar conclusions, that CS has an organic basis in the brain.

        As stated before, investigations into the neurological causation of Capgras Syndrome are a recent phenomenon, taking place within the last forty years. One of the first studies that drew attention to the organic etiology of CS was done by Gluckman in 1968. The woman he studied believed that her husband was an impostor; she was subsequently diagnosed with paranoid Schizophrenia. Gluckman put her through a computerized axial tomography scan and found “severe cerebral atrophy” (Doran, 1990). This study and the growing suspicion of other researchers, that CS has an organic causation, opened the door to further neurological research. Three years later Weston & Whitlock (1971) presented their case of a twenty year old man who underwent severe head trauma and then exhibited symptoms of CS. Weston & Whitlock (1971) described the damage as “frontal lobe syndrome with evidence of bilateral temporoparietal damage, a severe memory defect, and mixed dysphasia along with generalized impairment” (Doran, 1990). They suggested that this damage to the temporal and parietal lobes lead to the patient’s inability to “integrate memory, perception, and affect.” And that these dysfunctions were primary factors in the formation of CS (Doran, 1990).

         Other studies have demonstrated a strong link between CS and physical disorders. In certain cases CS symptoms remitted at the same time that the physical illness remitted (Christodoulou, 1977b).  Further evidence of CS’s organic basis was found by MacCallum (1973). In three out of five cases MacCullum demonstrated the onset of CS as a result of “anoxia, basilar migraine, [and] alcoholic encephalopathy” (Doran, 1990). He also observed that when the physical symptoms were remedied so were the delusional perceptions and beliefs of the patients.  Based on these results, “MacCallum hypothesized that these organic conditions caused a change in the patients' affects and perceptions, which then led to the appearance of the Capgras syndrome. With a remission of the organic condition, affect and perception returned to normal” (Doran, 1990). These findings strongly support the suggestion that CS is caused by organic factors. Other studies have linked CS with “right-sided cerebral dysfunction” (Hayman & Abrams, 1977), which turns the focus of this discussion to current emerging theories of CS.

        Doran (1990) reviewed the current neurological theory on Capgras Syndrome. He pointed out that initial importance is given to the distinction between the reduplication of a person or place. In neurological literature the reduplication of a place is termed “reduplicative paramnesia”; however, within psychiatric literature the reduplication of a person is termed Capgras Syndrome. The common perception is that these reduplicative disorders are one in the same; the only difference between the two is the object of the duplication, a person or a place. Doran went on to say that, “The cerebral localization and mechanism of action could essentially be the same.” Alexander, Stuss & Benson (1979) and Staton, Brumback & Wilson (1982) both had cases in which duplication of a “person” and of a “place” were demonstrated in their patient. Lesions in the right hemisphere have been found to be the primary cause of duplicative symptoms.

        This finding leads to the central thrust of this theory, the right hemisphere. Research of the,

“Right hemisphere lesions may lead to disturbed visuospatial analysis (Paterson & Zangwill, 1944), impaired facial recognition and memory (Hacaen & Angelergues, 1962; Milner, 1968), abnormal sensations of general familiarity andjamais vu (Mullan & Penfield, 1959), and abnormally flat or euphoric disorders (Gainotti, 1972)”(Doran, 1990). Furthermore, the right hemisphere has connections between “facial recognition, visual memory, and feeling and familiarity” (Quinn, 1980; Schraberg & Weitzel, 1979; Synodinou, Christodoulou, & Tzavaros, 1977).

         According to these findings, Alexander (1979) and numerous other researchers concluded that the “right hemisphere is the seat of dysfunction producing the delusional symptom of Capgras” (emphasis in original).  The dysfunctions that occur if the right hemisphere is damaged fit the descriptions of Capgras Syndrome perfectly. Staton (1982) hypothesized that a disconnection occurs between old memory stores and new memory registration. He also suggests this area of damage happens specifically in the “right posterior hippocampus and right temporo-parieto-occipital junction.” Another recent study proposes that the dysfunction is subcortical (Anderson, 1980). Based on this evidence, it is hypothesized that Capgras Syndrome is the result of a disconnect between the “hippocampus and the hypothalamic/amygdala circuit” (Doran, 1990). In other words, a CS patient still recognizes a loved one in every sensory way, but does not recognize them emotionally; due to a disconnection between emotion and memory. This conclusion, again, supports the previous assertion that Capgras Syndrome is the result of an organic disconnection between emotion and memory made by Ramachandran, Ellis (2000), and Schweinberger (2003).

Ever Wonder if Your Mom was a Double? Part 1

           In 1923 there was an unusual case in Paris of a woman who complained to the police that there were strange children imprisoned in her basement. She was also convinced that there were more children imprisoned throughout the city. Her delusional state consisted of an elaborate system which centered around two main themes: first, the belief that she had been substituted at birth and was an heiress. Second, the belief of an elaborate plot against her that involved thefts, poisonings, changes in her body’s appearance and substitutions of her family, herself and others in her environment (Sinkman, 2008). Accompanying her misidentification symptoms were psychotic features, persecutory hallucinations and other signs of severe mental illness.  This odd set of symptoms caught the attention of French psychiatrist Joseph Capgras. Capgras’ and Reboul-Lachaux’s research on this woman, later referred to as Mme M., eventually became the basis for a new set of disorders, categorized as “misidentity” or “false identity” syndromes.

Delusional Misidentity Syndrome

            Research with Mme. M led subsequent researchers to discover a whole range of disorders which they classified as Delusional Misidentity Syndrome (DMS). Included in the category is, firstly, Fregoli Syndrome (FS), in which a patient “holds the belief that a persecutor takes on the appearance of various people at different times” (Sinkman, 2008). FS can be associated with a persecutory misidentification of people; however, this is not always the case. It can also be an over-misidentification of one face on others. FS can also extend to misidentification of places as well as people. Second is Intermetamorphosis Syndrome, where the patient believes that an individual can transform into another person. Third, Cotard Syndrome, a disorder consisting of the delusional perception that one is dead or that a particular body part is dead (Berrios & Luque, 1995). Finally, Capgras Syndrome (CS), which will be discussed in detail later on. Variations of misidentity syndromes have arisen in the past; nevertheless, those listed above are the main syndromes within the DMS category. Patients who are diagnosed with a misidentity syndrome often have co-occurring or comorbid disorders such as schizophrenia and paranoia, or other misidentity syndromes (Fialkov & Robins, 1978; Koritar & Steiner, 1988).

Capgras Syndrome

            Capgras Syndrome defined by Joseph Capgras was “the illusion of doubles” (Enoch & Ball, 2001). A current definition of classic Capgras syndrome is a state in which “the patient believes that a person usually closely related to him has been replaced by an exact double” (Nejad & Toofani, 2006). Since Capgras Syndrome was first discovered, there has been a dichotomy in thinking. Some believe the source of CS to be psychological; while others believe it to be organic, resulting from a brain lesion or atrophy (Gluckman, 1968).  Unfortunately, the bulk of research since CS was discovered has shed little light onto the neurological causes. It’s only been very recent that neurological research has been conducted on the disorder. So far explanations for its etiology have varied from “neuro-psychiatric or organic and neurological diseases, including dementia, head trauma, epilepsy, cerebrovascular disease, neurodegenerative disease, most commonly lewy body disease and multiple sclerosis” (Bourget, & Whitehurst, 2004). Behavioral and psychodynamic explanations for Capgras range from “psychosis, paranoia, pathological splitting of a significant other, and changes in the crucial interpersonal relationship” (Berson, 1983).