J Neurol Neurosurg Psychiatry 2000;69:645–648 645 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.69.5.645 on 1 November 2000. Downloaded from SHORT REPORT Misidentification syndromes related to face specific area in the fusiform gyrus Arthur J Hudson, Gloria M Grace Abstract consistently misidentifies a familiar place, The “delusional misidentification syn- sometimes including occupants, as a replica. dromes” are a group of uncommon and Twenty years later a more common but similar varied disorders in which, in typical form, phenomenon involving people, called the patient thinks that a particular famil- “l’illusion des sosies” or Capgras syndrome,2 iar person is someone else or a certain was described wherein an usually familiar per- familiar place is a duplicate. Although son, such as a family member, is viewed as a first identified and considered a memory stranger with an identical appearance. Since disorder by Pick, evidence in support of then, similar variants have been reported, this has been diYcult to identify. They including the Frégoli syndrome in which, as in have been most often seen in various psy- the case described in this account, the patient chotic and organic brain diseases but conceptually misidentifies a person as another lesions have been generally diVuse al- known person with an entirely diVerent though the right temporal lobe has been appearance.3 The cause of these delusional implicated. A patient was investigated misidentification syndromes, as they are collec- who abruptly developed a disorder tively known,4 is obscure although Pick1 main- copyright. wherein she misidentified her husband as tained that the phenomenon represented a dis- her deceased sister and claimed that her turbance of memory, specifically involving “a home was a duplicate of her real home sense of familiarity”, that plays “an important that were typical of Frégoli syndrome and part in remembrance, especially in recogni- Pick’s reduplicative paramnesia, respec- tion”. tively. A discrete area of brain damage, Although often described in relation to psy- probably ischaemic, in this patient was chotic states including schizophrenia, it is, seen on MRI in the anterior part of the nevertheless, widely considered that these syn- right fusiform gyrus and a smaller area in dromes have an anatomical basis because of the nearby anterior middle and inferior their frequent association with organic brain temporal gyri with associated parahip- disease.45 Brain lesions generally have been pocampal and hippocampal atrophy. A http://jnnp.bmj.com/ poorly localised and are often bilateral al- high order nervous system function that is though lesions in the right temporal lobe have devoted to the identification of faces is 5 6 located in the adjacent midportion of the been described. Sellal et al described both fusiform gyrus and a similar locus for Capgras syndrome and delusional misidentifi- environmental scenes, termed the para- cation for place in a case of viral encephalitis hippocampal place area, is present in the with damage to the anterior medial part of the bordering parahippocampal gyrus. The right temporal lobe and the medial frontal and misidentification phenomena in this case orbital frontal regions bilaterally. Hirstein and on September 25, 2021 by guest. Protected 7 can be explained by disruption of the con- Ramachandran postulated that Capgras syn- nections of these highly specialised areas drome could be explained by damage to Department of Clinical connections from face processing areas in the Neurological Sciences, with the most anterior inferior and medial London Health part of the right temporal lobe where long right temporal lobe to the limbic system. Sciences Centre term memory and mechanisms for the Hence, our finding of discrete lesions in the (University Campus), retrieval of information that are required right anterior inferior temporal lobe has 339 Windermere Road, for the visual recognition of faces and provided a rare opportunity to confirm the London, Ontario, N6A scenes are stored. suspected organic basis and anatomical locali- 5A5, Canada (J Neurol Neurosurg Psychiatry 2000;69:645–648) A J Hudson sation for these kinds of delusions. The visual G M Grace phenomenon in these disorders is distinct from Keywords: fusiform gyrus face area; parahippocampal prosopagnosia in which familiar faces are place area; delusional misidentification; Frégoli syn- Correspondence to: perceived but not recognised. None the less, Dr A J Hudson drome [email protected] pure prosopagnosia is associated with damage to nearby right temporal cortical areas that Received 24 November 1999 Pick,1 almost a century ago, described a include, predominantly, the lingual and poste- and in final form 5 June 2000 syndrome that he named “reduplicative param- rior half of the fusiform and parahippocampal Accepted 6 June 2000 nesia” in which a person repeatedly and gyri.89 www.jnnp.com 646 Hudson, Grace J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.69.5.645 on 1 November 2000. Downloaded from copyright. http://jnnp.bmj.com/ MRI of the patient’s brain. (A) A coronal T2 weighted image through the temporal lobe at the level of the hippocampus shows abnormal hyperintensity in the right middle and inferior temporal gyrus (small arrow in all figures) and fusiform gyrus (large arrow in all figures). (B) Proton density image of the same area as shown in A, illustrates enlargement of the right temporal horn (divided by cut into two sections as in C). There are also atrophic changes in the right hippocampus and parahippocampal gyrus and (not shown) in the fusiform gyrus. (C) Axial FLAIR image through the temporal lobe at the level of the inferior tip of the temporal horn shows the anteroposterior extent of hyperintensity in the right inferior temporal and fusiform gyri. (D) Schematic illustration of the inferior surface of the brain demonstrates the approximate on September 25, 2021 by guest. Protected extent of the damage (grey shade) in the fusiform gyrus as derived from the MRI in comparison with the putative location and extent of the fusiform face (hatched) area.14 CS=collateral sulcus; FG=fusiform gyrus; ITG=inferior temporal gyrus; LG=lingual gyrus; OTS=occipitotemporal sulcus; PG=parahippocampal gyrus. Case report address him by the sister’s name and would The subject, a 71 year old woman, began hav- become argumentative and hostile towards him ing frequent fainting attacks from intermittent as though he were intruding. Intermittently she complete heart block. This was confirmed would speak to him using his real name, when an electrocardiographic tracing obtained expressing their usual intimacy and clearly at during an episode of unconsciousness dis- such time recognising him as her husband. In closed an abrupt drop in her heart rate from 72 this context it is noteworthy that visual contact to 30 beats per minutes for 10 seconds. Within seemed necessary for misidentification in as a day or two of the recording she was given a much as she never misidentified him as her sis- permanent cardiac pacemaker with immediate ter on the telephone. In addition to misidenti- and complete arrest of fainting. However, dur- fying him she often referred to their home as a ing the first few days of the attacks, she began rented replica and would even pack her bags to misidentifying her husband as her elder sister return to their “real” home. Sometimes she who had died 3 years previously. She would thought other members of her family, all of www.jnnp.com Misidentification syndromes related to face specific area in the fusiform gyrus 647 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.69.5.645 on 1 November 2000. Downloaded from Table 1 Neuropsychological tests temporal lobe (figure). There was also atrophy of the right parahippocampal and hippocampal Classification range* gyri. Normal (average) Subnormal (impaired) Performance on neuropsychological testing (table), about 1 year after the onset of the syn- Test High to mid Low Mild Moderate to severe drome, was in the average to above average Wechsler adult intelligence scale-revised: range on tests of verbal intellectual skill and Verbal IQ + reasoning, most language functions, mental Subset scores Information + calculation, auditory attention, and concentra- Digit span + tion (digit span), oral and manual praxis, and Vocabulary + many aspects of verbal learning and memory. Arithmetic + Comprehension + However, there were deficits in visual percep- Similarities + tion and construction and her performance IQ Performance IQ + was significantly lower (25 points) than verbal Subset scores: Picture completion + IQ. Deficits were apparent in visual attention Picture arrangement + (visual span), memory for visual material Block design + (visual reproduction), and visual motor Object assembly + Digit symbol + processing speed (trail making test). Executive Wisconsin card sorting test:† processes such as word generation (semantic Number of categories + Perseverative responses + fluency) and organisational memory (Rey Non-perseverative responses + auditory verbal learning test) were mildly to Trail making test: moderately impaired, and several qualitative Part A + Part B + impairments of executive function were noted. Wide range achievement test - 3: For example, on delayed recall of the Rey com- Reading subtest + plex figure, perseveration was illustrated when Multilingual aphasia examination: Benton visual naming + her drawing resembled not the appropriate fig- Controlled oral word association: + ure but one drawn