Case Report
Frégoli Syndrome Accompanied with Prosopagnosia in a Woman
with a 40-year History of Schizophrenia.
Yasushi Moriyama,1 Taro Muramatsu,2 Motoichiro Kato,2 Masaru Mimura,3Tomoko Akiyama,1 and Haruo Kashima,2
1
Department of Psychiatry, Komagino Hospital, Tokyo, Japan.
Department of Neuropsychiatry, Keio University School of Medicine, Tokyo, Japan.
3
Department of Neuropsychiatry, Showa University School of Medicine, Tokyo, Japan
2(Received for publication on May 28, 2007)(Revised for publication on August 2, 2007)(Accepted for publication on September 20, 2007)

Abstract: A 68-year-old woman with schizophrenia after a cerebrovascular accident resulting in right medial temporal and occipital damage developed Frégoli syndrome. Neuropsychological testing revealed that she had impairment in facial recognition compatible withprosopagnosia. The Frégoli syndrome disappeared in three month when, remarkably, herprosopagnosia also disappeared. Our findings are consistent with the hypothesis that combination of hyperactivations of temporo-limbic connection and frontal dysfunction may account for the development of Frégoli syndrome. (Keio J Med 56 (4) : 130－134, December 2007)Key words: Frégoli syndrome, prosopagnosia, schizophrenia, delusional misidentification syndrome,
fusiform gyrus

Introduction
Delusional Misidentification Syndrome (DMS) is traditionally regarded as having three major variants: Capgras syndrome, Frégoli syndrome, and the syndrome ofintermetamorphosis. In Capgras syndrome, the patient
believes that certain familiar people have been replaced
by a group of impostors resembling the original individuals. Frégoli syndrome, less common than Capgras syndrome, is the delusional misidentification of familiar
people disguised as others. In the intermetamorphosissyndrome, the least common of the three, the patient experiences changes both in the identity and the appearances of the misidentified persons.1-5
Much interest and controversy has prevailed over explanation of the etiology and pathogenesis of DMS andvarious psychodynamic and organic impairments havebeen proposed. Recent elucidation of mechanisms of facial identity and facial expression has made it possible tounderstand the DMS in the context of cognitive neuropsychology, and debate has centered around the relationship between Capgras syndrome and prosopagnosia.
Prosopagnosia is the neuropathological inability to rec-ognize familiar people by their faces, that leaves intact
recognition of personal identity from other identifying
cues, such as voices and names. Prosopanosia has been
observed after bilateral and, less frequently, unilateral lesions of the inferior occipitotemporal cortex.6, 7 Attempts
have been made to identify the mental operations that arehampered in prosopagnosia by referring to models offace processing. Among them, probably the most intriguing findings in regard to DMS are those of covert vs.
overt face recognition. When given a mixture of photographs of unfamiliar (novel) and familiar faces and asked
to sort them into two piles, prosopagnosics are unable to
do so; yet remarkably they register a stronger skin conductance response to familiar faces (as do normal subjects), implying that the face-processing machinery is
still connected to the limbic system.8 This observation
supports the view that there are two components to thevisual recognition of a familiar face, one of which is responsible for conscious recognition of the face and therecall of associated semantic information (the ventralroute), whereas the other is responsible for the limbicmediated emotional arousal which includes the feeling offamiliarity that accompanies the implicit recognition of aCorresponding Author: Yasushi Moriyama, MD. 273 Ura-takaomachi Hachioji, Tokyo, 193-8505 Japan TEL: 81-42-663-2222 Fax: 81-42-663-3286 E-mail:yamori@rg7. so-net. ne. jp130Keio J Med 2007; 56 (4): 130－134

1A131

1BFig. 1 MRI of the patient’s brain. Axial T2-weighted (Figure 1A) and FLAIR (Figure 1B) image show abnormal hyperintensity in theright parahippocampal, hippocampal, lingual, and fusiform gyri.

familiar face (the dorsal route), originally presented byBruce and Young.9, 10
In regard to the relationships between the two face processing routes and prosopagnosia or Capgras syndrome,
Ellis et al.11, 1 2 have suggested that Capgras syndrome
represents a ‘mirror image’ of prosopagnosia. In this hypothesis, the ventral route is responsible for overt facerecognition while the dorsal visual route is responsible
for the emotional responses to faces. If brain damage affects the first route, then familiar faces will evoke normalheightened affective responses (indexed by autonomicmeasures) but there will be no overt recognition: this is
prosopagnosia. If brain damage affects the second route,then intact overt recognition will be coupled with a lossof affective responsiveness. This latter case will generatecognitive dissonance, particularly when the person looks
exactly like a loved one but fails to evoke the expected
emotional response. One resolution of that dissonance is
to conclude that the person is an impostor.11, 12
Regarding this hypothesis, Hirstein and Ramachandran1 3 questioned that this hypothesis cannot illustrate
why the mere absence of this emotional arousal leads tosuch an extraordinarily far-fetched delusion, or why thepatient does not just think, ‘I know that is my father but I
no longer feel the warmth?’. With regard to these points,
they argued that some additional lesion, perhaps in theright frontal cortex, might be required to generate suchextreme delusions. They have also presented the ‘inferior
temporal-limbic disconnection hypothesis’; 1) the princi-pal cause of Capgras syndrome is a failure of communication between areas of ventral stream processing in thetemporal lobe; 2) and a disorder of the right frontal lobe,
which ordinarily serves as a global consistency-checking
mechanism, may cause such an extraordinarily farfetched delusion.13
As for Frégoli syndrome, Ramachandran applied thesame model and suggested that this may occur when thetemporal-limbic connection becomes hyperactive, rather
than disconnected.1 3, 1 4 However, to our knowledge, noclinical cases have yet been reported that from such aneuropsychological view. We herein present and discuss
the neuropsychological implications of an interestingcase in which a patient with schizophrenia developed Frégoli syndrome accompanied with prosopagnosia during
the subacute stage of cerebral infarction.
Case Report
The patient was a 68-year-old right-handed housewife
with a 40-year history of delusional schizophrenia. In
August 2004, she developed a cerebrovascular accident,
following which she developed confusion and gait disturbance. She was admitted to the Komagino Hospitalwhere MRI revealed a regional infarction in an area extending to the right parahippocampal, hippocampal, lingual and fusiform gyri. The right amygdala was unaffected (Fig 1). After two-months’ conservative treatment,
when she had recovered from the aforementioned neuro-132

Moriyama Y, et al: Frégoli Syndrome Accompanied with Prosopagnosia Schizophrenia

Table 1 Summary of other neuropsychological test results
WAIS-R verbal IQ
performance IQ
MemoryRAVLT
immediate
delayed
RCFT
copy
Executive functioning
Word Fluency Initial(sum of three tasks)
Category (sum of three tasks)
Wisconsin Card Sorting Test (Keio Version):
Categories (trial 1, 2)
Preservative errors (trial 1, 2)
Stroop test
part I
part III
part III - IBADS

Raw score83
unable to perform

Normal score or cut-off point

4-6-7-8-9/15*
8/15*
unable to perform- - - 13-15/1520
11.62/1520

2219*25.4±1.821
42.6±1.421

1-0*
8-6*
55 sec*78 sec*23 sec*4/24 point*

5.3±1.1 - 5.8±0.522
3.1±3.9 - 0.3±0.522
16.2±0.821
29.3±1.521
13.1±1.321>11/24 points23*: impaired

WAIS-R: Wechsler Adult Intelligence Scale -Revised
RAVLT: Rey Auditory verbal learning test
RCFT: Rey Osterrieth complex figure test
BADS: The behavioral assessment of the dysexecutive syndrome

logical symptoms, Frégoli syndrome developed. She began misidentifying a specific male patient as her husband. Although there was no physical similarity between
the male patient and her husband, and moreover, her husband had died four years previously, she was adamant inher conviction that the male patient was her husband in
disguise. The emergence of this rare DMS and the MRI
findings prompted us to investigate her visual recognition performance. It was found that she was unable toidentify her doctor or her daughter on photographs ormuted video. To evaluate her visual function thoroughly,
a comprehensive neuropsychological test battery, VPTA,
was administered twice.
The first evaluation was performed in October 2004,
when she presented with Frégoli syndrome. On this occasion, the VPTA revealed prosopagnosia and unilateralneglect. her performance in other subtests of VPTA wasrelatively preserved. On the Face Recognition Task inVPTA, her performance was impaired in 7 out of 8 subtests, i.e., famous face naming, famous face identification, famous face pointing, discrimination of two unfamiliar faces, matching two unfamiliar faces, recognitionof facial expression and emotion, and age judgement.
Immediately after the first evaluation, oral administration of risperidone (2 mg per day) was initiated, and Frégoli syndrome disappeared in the third month after theinfarction, when facial recognition had also improved asrevealed by VPTA (Fig. 2; Second evaluation, December2004). The patient’s other neuropsychological tests results are presented in Table 1; memory and executivefunctions were impaired. Notably, the poor performanceon BADS indicated dysexecutive syndrome.
Discussion
We described a patient with chronic schizophrenia whopresented with Frégoli syndrome and prosopagnosia after
infarction of the right cerebral hemisphere. There is little
doubt that the cerebral damage, which included the fusiform gyrus, the critical region for face recognition, wasresponsible for her prosopagnosia.
Prosopagnosics are unable to recognize familiar people
by their faces, but they usually do not misidentify people; their typical complaint is “all faces look alike”. The
remarkable feature of the present case is, therefore, coexistence of prosopagnosia and Frégoli syndrome. For
this exceptional phenomenon to occur in this case, thecondition of schizophrenia should be considered a key
contributing factor. In fact, the most common diagnostic
setting in which DMS including Frégoli syndrome is observed is that of schizophrenia. For example, Mojtabai2
reviewed 34 cases of Frégoli syndrome and foundschizophrenia to be the predominant diagnostic category.
However, because DMS developed only after the patient
had suffered a cerebral insult in the present case, it would
appear that neither cerebral infarction nor a preexisting
psychopathology of schizophrenia alone are sufficient toaccount for DMS, but that both are necessary to produce
these symptoms. A very plausible possibility is that someorganic impairment leads to the hyperactivation of theemotional system, as Ramachandran and Blakeslee14 hassuggested. In this case, one could reasonably speculate

Keio J Med 2007; 56 (4): 130－134

133

Fregoli (+)
Fregoli(+)
Fregoli (-)
Fregoli(-)

Percent
Percentcorrect (%)

100806040200
famous face famous face famous face discrimination matching two recognition of gendernamingidentification
pointingof twounfamiliar
facialjudgment
unfamiliar
faces
expression andfaces
emotionage judgment

Fig. 2 Face Recognition on *VPTA
Data are the patient’s performance on the Face Recognition Task of VPTA. In October 2004, performance was impaired with theexception of gender judgment. Remarkably, at the second evaluation on December 2004, when Frégoli syndrome
had disappeared, facial recognition performance had also improved*VPTA: Visual Perception Test for Agnosia(see text)that the cerebral infarction acted as the crucial organic
factor. Specifically, some reversible effect (e.g. edema)of the infarction appears to have impaired the critical siteof face recognition, while activating the input to the intact amygdala, thereby heightening the emotional significance of facial expression. Further research will be needed to confirm this hypothesis. The fact that the prosopagnosia and Frégoli syndrome proceeded and disappeared
simultaneously supports this view. The relatively rarecombination of these two features was presumed to becreated by this exceptional mechanism.
Additionally, because frontal dysfunction is most frequently reported cognitive impairment in schizophrenia,
one can speculate that DMS would also be related tosome frontal dysfunction. In this context, in her, dysexecutive syndrome, as revealed by poor performance ofBADS, which suggested frontal dysfunction, may havecompleted the picture of DMS. That Feinberg et al4 speculated that combination of executive and memory deficits may account for cases of Frégoli syndrome on traumatic brain injury is in line with this.
Another speculative but intriguing possibility concerns
the cognitive style of people with schizophrenia, which
is known as the jumping-to-conclusions bias.1 7, 1 8 The
significance of this reasoning style derives from the assumption that people with such a bias will form unwar-ranted conclusions on the basis of scant evidence. Because the patient could get little information from faces
while suffering from prosopagnosia, it is possible tospeculate that the long history of delusional schizophrenia had contributed to her belief that a specific male patient was her husband, according to the jumping-to-conclusions reasoning bias and the emotional dysregulation
for the face. This seems to be a reasonable mechanism
from the point of view of the cognitive dysfunction inschizophrenia, but accumulation of empirical findingssuch as the present case reports is needed.
Like other Frégoli cases in the literature, her misidentification only involved one specific person, i.e., her husband, which points to the psychological process as an indispensable factor. Collacot and Napier19 pointed out that
this particular misidentification may have functioned aswish fulfillment. One can speculate that both psychodynamic and organic factors described above contributed tothe emergence of Frégoli syndrome in this case.
This statement is one consideration based on the patient’s neuropsychological performances and on cognitive neuropsychiatric studies on schizophrenia. Therefore, the propositions made herein await further confirmation from future research. Moreover, our testing waslimited due to clinical considerations. However, given
the rarity of the disorder and the significance of face rec-Moriyama Y, et al: Frégoli Syndrome Accompanied with Prosopagnosia Schizophrenia

134

ognition theory, we believe that the present case is worth
reporting. Further research into related phenomena, specifically, prosopagnosia with or without Frégoli syndrome in the context of schizophrenia, may shed light on
the etiology and pathogenesis of DMS.
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