A 61 year old man after a traumatic brain injury resulting in right frontal and left temporoparietal contusions developed florid Fregoli-type misidentifications. Extensive neuropsychological testing demonstrated significant deficits in executive and memory functions. The patient's neuropsychological profile closely resembled that seen in previously reported patients with Capgras syndrome. Our findings are consistent with the hypothesis that a combination of executive and memory deficits may account for cases of delusional misidentification associated with brain lesions. However, the form which the delusion takes may be influenced by other factors including motivation.

OBJECTIVE

The aim of this paper is to document regular nocturnal intensification of delusional nihilistic and persecutory ideas (Cotard delusion) linked with extreme depersonalisation and hypervivid dreaming.

METHODS

A 17-year-old man presented with Cotard and Capgras delusions after sustaining multiple cognitive impairments secondary to traumatic brain injury.

RESULTS

Delusional ideation fully resolved within 14 days of commencement of olanzapine 5 mg daily.

CONCLUSIONS

This patient's experience of perceptual abnormalities and impairments in meta-abilities related to self-monitoring and critical inferencing lends support to multicomponent sensory processing accounts of brain injury related, content-specific delusional syndromes.

The hallmark of Cotard's syndrome is the delusion of being dead. It is usually seen in affective disorders, and its existence as a separate entity is controversial. A patient is described who presented with paranoid schizophrenia, Cotard's syndrome, the misidentification syndromes of Capgras and subjective doubles, and palinopsia (visual perseveration). Focal theories for these conditions are briefly outlined and the patient is discussed with reference to them. It is proposed that this case supports the concept that Cotard's syndrome is a distinct disorder. The possibility of cerebral dysfunction in the confluence of the parietal, temporal, and occipital regions accounting for this specific clinical presentation is considered.

Investigations of two cases of the Capgras delusion found that both patients showed face-processing impairments encompassing identification of familiar faces, recognition of emotional facial expressions, and matching of unfamiliar faces. In neither case was there any impairment of recognition memory for words. These findings are consistent with the idea that the basis of the Capgras delusion lies in damage to neuro-anatomical pathways responsible for appropriate emotional reactions to familiar visual stimuli. The delusion would then represent the patient's attempt to make sense of the fact that these visual stimuli no longer have appropriate affective significance.

The editors first describe the principal symptoms of the Capgras delusion, the Frégoli delusion, the delusion of intermetamorphosis and the delusion of subjective doubles before developing the argument that it would be appropriate for international psychiatric diagnostic systems to include these disorders. Furthermore the similarity between them, the reduplicative paramnesias and déjà and jamais vu are pointed out. By stressing a symptom-based approach it is possible to examine psychiatric, neurological and medical cases as arising from the same underlying disturbance in cognition function. The relationship between delusions of misidentification and other delusions is also touched upon.

The Capgras delusion has long been held to be specific for close personal relationships. This paper presents evidence that this is not the case and the implications of this for accepting psychodynamic explanations of the phenomenon are discussed. Parallels are drawn with the condition prosopagnosia and a hypothesis presented that any specificity is apparent rather than real. It is proposed that both prosopagnosia and the Capgras symptom represent abnormalities of visual perception occurring at different stages of this neurophysiological process.

Perception of familiar faces depends on a core system analysing visual appearance and an extended system dealing with inference of mental states and emotional responses. Damage to the core system impairs face perception as seen in prosopagnosia. In contrast, patients with Capgras delusion show intact face perception but believe that closely related persons are impostors. It has been suggested that two deficits are necessary for the delusion, an aberrant perceptual or affective experience that leads to a bizarre belief as well as an impaired ability to evaluate beliefs. Using functional magnetic resonance imaging, we compared neural activity to familiar and unfamiliar faces in a patient with Capgras delusion and an age matched control group. We provide evidence that Capgras delusion is related to dysfunctional activity in the extended face processing system. The patient, who developed the delusion for the partner after a large right prefrontal lesion sparing the ventromedial and medial orbitofrontal cortex, lacked neural activity to the partner's face in left posterior cingulate cortex and left posterior superior temporal sulcus. Further, we found impaired functional connectivity of the latter region with the left superior frontal gyrus and to a lesser extent with the right superior frontal sulcus/middle frontal gyrus. The findings of this case study suggest that the first factor in Capgras delusion may be reduced neural activity in the extended face processing system that deals with inference of mental states while the second factor may be due to a lesion in the right middle frontal gyrus.

Person identification disturbances in Alzheimer's disease (AD) add to the suffering of both patients and caregivers. We assessed the prevalence of person identification disturbances in the records of 217 outpatients with AD. These disturbances occurred in 25.4% (N = 55) and included transient misidentifications of familiar persons (N = 34), the Capgras syndrome (N = 11), misidentification of themselves in mirrors (N = 5), prosopagnosia (N = 3), misidentification of unfamiliar persons as familiar (N = 1), and misidentification of another person as oneself (N = 1). Transient misidentifications were easily corrected misperceptions, and the Capgras syndrome and mirror difficulties were associated with suspiciousness/paranoia and delusions. In AD, these findings suggest that misidentifications of familiar persons result from misinterpretations due to cognitive impairments, and the Capgras syndrome and mirror difficulties ensue when these misinterpretations are elaborated by paranoid delusions.

SEE MCKAY AND FURL DOI101093/AWW323 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Focal brain injury can sometimes lead to bizarre symptoms, such as the delusion that a family member has been replaced by an imposter (Capgras syndrome). How a single brain lesion could cause such a complex disorder is unclear, leading many to speculate that concurrent delirium, psychiatric disease, dementia, or a second lesion is required. Here we instead propose that Capgras and other delusional misidentification syndromes arise from single lesions at unique locations within the human brain connectome. This hypothesis is motivated by evidence that symptoms emerge from sites functionally connected to a lesion location, not just the lesion location itself. First, 17 cases of lesion-induced delusional misidentifications were identified and lesion locations were mapped to a common brain atlas. Second, lesion network mapping was used to identify brain regions functionally connected to the lesion locations. Third, regions involved in familiarity perception and belief evaluation, two processes thought to be abnormal in delusional misidentifications, were identified using meta-analyses of previous functional magnetic resonance imaging studies. We found that all 17 lesion locations were functionally connected to the left retrosplenial cortex, the region most activated in functional magnetic resonance imaging studies of familiarity. Similarly, 16 of 17 lesion locations were functionally connected to the right frontal cortex, the region most activated in functional magnetic resonance imaging studies of expectation violation, a component of belief evaluation. This connectivity pattern was highly specific for delusional misidentifications compared to four other lesion-induced neurological syndromes (P < 0.0001). Finally, 15 lesions causing other types of delusions were connected to expectation violation (P < 0.0001) but not familiarity regions, demonstrating specificity for delusion content. Our results provide potential neuroanatomical correlates for impaired familiarity perception and belief evaluation in patients with delusional misidentifications. More generally, we demonstrate a mechanism by which a single lesion can cause a complex neuropsychiatric syndrome based on that lesion's unique pattern of functional connectivity, without the need for pre-existing or hidden pathology.

Previous neuropsychological studies have demonstrated an association between person misidentification and right-hemisphere dysfunction. In the study reported here, we explore the contribution of facial and visual recognition impairments in a patient with right-hemisphere subcortical white-matter pathology in the frontal and parietal lobes and a diagnosis of vascular cognitive impairment. The patient displayed false recognition of unfamiliar faces and deficient retrieval of key biographic detail for famous faces. These results are discussed in the context of the contribution of deficiencies in the visual system and subcortical white-matter lesions to the development of Capgras delusion.

A 33-year-old male suffering from schizophrenia developed the typical symptoms of Cotard's syndrome, i.e., various delusions of negation and severe depressive symptoms. Atypical symptoms such as delusions of persecution and control related to body parts were also observed. These symptoms gradually improved by the treatment with sulpiride 300 mg/day. In the course of improvement of Cotard's syndrome, the patient developed Capgras syndrome. This report suggests that sulpiride is effective for Cotard's syndrome in schizophrenia. It also suggests that the symptoms of Cotard's syndrome are modified according to basic disorders, and this syndrome has a close connection with Capgras syndrome.

Capgras delusion is characterized by the misidentification of people and by the delusional belief that the misidentified persons have been replaced by impostors, generally perceived as persecutors. Since little is known regarding the neural correlates of Capgras syndrome, the cerebral metabolic pattern of a patient with probable Alzheimer's disease (AD) and Capgras syndrome was compared with those of 24-healthy elderly participants and 26 patients with AD without delusional syndrome. Comparing the healthy group with the AD group, the patient with AD had significant hypometabolism in frontal and posterior midline structures. In the light of current neural models of face perception, our patients with Capgras syndrome may be related to impaired recognition of a familiar face, subserved by the posterior cingulate/precuneus cortex, and impaired reflection about personally relevant knowledge related to a face, subserved by the dorsomedial prefrontal cortex.

The present report regarda a patient of temporal lobe epilepsy, who developed the classical features of Capgras phenomenon. The whole clinical picture responded to treatment. The possible roles of organic and psycho-social factors in the psychopathology of this peculiar delusion in this patient are discussed.

The Capgras syndrome of delusions that familiar persons are being impersonated by identical doubles has occasionally been associated with cerebral disease, often of the nondominant hemisphere. A patient with chronic right cerebral hemisphere dysfunction and complex partial seizures of right temporal origin manifested the Capgras syndrome in the postictal state, and the delusions ceased with optimal seizure control. The delusions could result from postictal disinhibition of dominant hemisphere recognition functions, or from dysfunction of nondominant hemisphere centers involved in perceptual integration.

The underlying diagnosis of Capgras delusion is not found to be exclusively paranoid schizophrenia, nor is the sex exclusively female, as once was believed. Organic factors play a predominant role in many patients, and 41% of the cases are males. The age at diagnosis in those considered to be schizophrenic is older than is usually expected.

A Capgras syndrome case is presented. The clinical profile appeared at the onset of treatment with Praziquantel for neurocysticerosis. A left-temporal cysticercum was shown by means of a CT scan. Delusion, accompanied by some neurological symptoms diminished but did not disappear after treatment. The hypotheses for Capgras syndrome are reviewed and it is concluded that it constitutes a cognitive-dysmnesic phenomenon (feeling of unreality, impossibility to integrate recently acquired information with old memory traces) and, as such, its origin is likely temporal.

BACKGROUND

Cenesthesia and cenesthopathy have played a fundamental role in 19th and early 20th century French and German psychiatry. Cenesthesia refers to the internal, global, implicit and affective perception of one's own body. The concept of cenesthopathy was coined by Dupre and Camus in 1907 to describe a clinical entity characterized by abnormal and strange bodily sensations.

OBJECTIVE

In this review, we examine the history of these concepts and the influence they have had on clinical, nosographical and phenomenological psychiatry and on cognitive neuropsychiatry.

METHODS

We performed a narrative review of the published research literature.

RESULTS

Classical French and German psychiatrists have written extensively on cenesthesia and cenesthopathy although these notions are no longer in the mainstream of contemporary psychiatry. However, they are still present in contemporary psychiatric nosography in the form of some controversial clinical entities clearly related to cenesthesia such as cenesthetic schizophrenia, hypochondriacal monothematic delusions, or the Capgras and the Cotard syndromes. These clinical entities are all associated with a state of depersonalization. We point out the similarities between Ey's description of the depersonalization syndrome, especially in psychosis, and the characteristics of cenesthesia. Philosophers like Sartre or Merleau-Ponty have developed the concept of cenesthesia, and in particular have added new concepts like "body schema". Similarly, phenomenological psychiatrists like Minkowski or Tellenbach have attempted to describe psychiatric disorders associated with cenesthesia and have also proposed new concepts (i.e. atmospheric sense) in order to understand them better. More recently, cognitive neuropsychiatry has tried to discover the mechanisms, which cause or contribute to the genesis of delusions. The majority of delusion theories developed in cognitive neuropsychiatry consider that the explanation of monothematic delusions involves one or two explanatory stages. The first stage corresponds to an abnormal experience (the experiential stage) while the second is related to abnormal reasoning (the inferential stage). This theoretical first stage has been considered to be the result of a highly unusual or bizarre perceptual experience. According to the authors, this experience refers to a phenomenon of depersonalization, a loss of cenesthesia, or a loss of a feeling of familiarity. For example, the neurocognitive models of the Capgras and Cotard syndromes have in common the belief that they are both based on various kinds of unusual experiences. These unusual experiences are thought to include affective or emotional experiences. Capgras' syndrome is possibly triggered by an abnormal affective experience in response to the sight of closed-person's face. Similarly, the Cotard syndrome may result from a general flattening of affective responses to external stimuli. The inferential stage can also differentiate between the two syndromes. Some empirical validation has already been obtained in Capgras' syndrome but not yet in Cotard's syndrome.

CONCLUSIONS

This review illustrates that the historical descriptions of cenesthesia and cenesthopathy remain relevant in contemporary neurocognitive models and more generally suggests that the comprehension of quite complex phenomena like delusion requires a multidisciplinary approach.

An experiment was carried out designed primarily to test A B Joseph's suggestion that patients with Capgras delusion may have problems integrating information between the two cortical hemispheres; and at the same time it was meant to examine J Cutting's ideas linking schizophrenia in general, and the Capgras delusion in particular, to right hemisphere dysfunction. Three patients with the Capgras delusion and three matched controls diagnosed as paranoid schizophrenics were briefly presented pairs of line-drawn object and photographs of faces randomly in the left visual field, the right visual field or bilaterally. The results with objects revealed no particular pattern of performance for either group; but, when faces were shown, the controls revealed the usual left visual field/right hemisphere advantage while for the Capgras group this was reversed. The results are not consistent with a simple prediction from Joseph's hypothesis but they are in accord with Cutting's theory-though they also pose some problems for it, which are discussed.

OBJECTIVE

To report a case of probable domperidone-induced tardive dyskinesia and withdrawal psychosis in an elderly woman.

METHODS

A 75-year-old woman was first assessed for cognitive decline and personality changes. On clinical examination, diffuse choreoathetoid movements were noted. Chronic domperidone use seemed the most likely cause for the movement disorder and was abruptly discontinued. Within a few days, she developed a severe psychotic syndrome with Capgras delusions; the movement disorder continued unabated. Both the movement disorder and psychotic symptoms promptly remitted with risperidone treatment.

CONCLUSIONS

Domperidone has, on rare occasions, been associated with acute extrapyramidal syndromes, especially in young children, but, to our knowledge, this is the first case of domperidone-induced tardive dyskinesia and withdrawal psychosis, adverse effects associated with chronic central nervous system dopaminergic blockade. Domperidone is a dopamine D₂ receptor antagonist that does not usually cross the blood-brain barrier. However, blood-brain barrier alterations occur with aging and dementia, which may explain central nervous system penetration of domperidone in our patient, diagnosed with probable frontotemporal dementia. The association was rated as probable on the Naranjo probability scale.

CONCLUSIONS

Chronic use of domperidone may, on rare occasions, induce neuropsychiatric syndromes similar to those seen with the use of antipsychotics. This may be more likely in situations in which the blood-brain barrier is damaged, as in vascular and degenerative dementias.

We have described a patient in whom EEG abnormalities, a seizure disorder, and Capgras syndrome developed two weeks after she started taking disulfiram. That disulfiram has been shown to inhibit dopamine beta-hydroxylase in vitro suggests an etiologic role for dopaminergic pathways in at least some cases of Capgras syndrome. Our experience with this patient suggests that convulsions and psychosis may occur as a side effect of standard dosages of disulfiram in patients with no previous history of psychosis or brain disease. Furthermore, the symptoms may resolve spontaneously without the long-term use of antipsychotic or anticonvulsant medication.

BACKGROUND

This study investigated a patient with a delusion of misidentification (DM) resembling a Capgras delusion. Instead of the typical Capgras delusion--the false belief that someone has been replaced by an almost identical impostor--patient MF misidentified his wife as his former business partner.

METHODS

Detailed investigation of MF's face processing, affective response and affect perception, and ability to evaluate, and reject, implausible ideas was undertaken.

RESULTS

MF's visual processing of identity, gender, and age of familiar and unknown faces was intact but he was unable to identify the facial expressions of anger, disgust, and fear, or to match faces across expressions. MF also showed a reduced affective responsiveness to his environment, and impaired reasoning ability.

CONCLUSIONS

We propose that MF's delusion of misidentification resulted from a combination of affective deficits, including impairment of both affective response and affect perception, in addition to an inability to evaluate, and reject, implausible ideas. These deficits, in combination with specific life events at the time of onset of the delusion, may have contributed to the form and content of the delusion. In addition, the results raise the possibility that the processing of face identity and facial expression are not as independent as previously proposed in models of face processing.

A case of Capgras syndrome associated with extreme violent behaviour is described. The patient's psychodynamics support the belief that the Capgras delusion initially arises from an altered affective response towards one or more others and proceeds to intolerable ambivalent feelings. This ambivalence is neutralised through the imagined existence of doubles.

We report the occurrence of Capgras' syndrome, or the delusion of doubles, in a patient with dementia with Lewy bodies. The patient believed that several similar-looking impostors had replaced his wife of over 50 years. Uncharacteristically, he adopted a friendly attitude with these impostors. This unusual convivial reaction to the impostors may result from differential involvement of the dual visual pathways processing facial recognition and emotional responses to faces. The delusion resolved spontaneously, coincident with worsening of the dementia. In a retrospective chart review of 18 autopsy-confirmed cases of dementia with Lewy bodies, delusions were reported in 5 subjects (27.8%), of whom 1 had misidentification delusions much like Capgras' syndrome.