CASE REPORT A Case of Capgras Syndrome With Frontotemporal Dementia Baris Metin, MD, PhD,* Mehmet K. Arikan, MD, PhD,*† 06/14/2019 on xb49PWcTrTwnNBxYZf3VaVIi+bbKrDculzKxD4UVNSjM0Z4o2bpF71rD53f4iR97XT+8XW+4kMtyfH+7ELAgxiUku60u23aAO/2LtfUeH4hQiDnsU7+Qqg== by http://journals.lww.com/cogbehavneurol from Downloaded Sukriye A. Kalem, PhD,‡ and Nevzat Tarhan, MD* Downloaded from syndromes (Kimura 1986), as well as in patients with http://journals.lww.com/cogbehavneurol Abstract: Capgras syndrome (CS), also called imposter syn- dementia (Harwood et al, 1999). For instance, Harwood drome, is a rare psychiatric condition that is characterized by the et al (1999) reported CS presentation in 10% of patients delusion that a family relative or close friend has been replaced who had been diagnosed with Alzheimer disease. by an identical imposter. Here, we describe a 69-year-old man Harciarek and Kertesz (2008) also claimed that mis- with CS who presented to the Kemal Arikan Psychiatry Clinic identification syndromes such as CS, reduplicative par- with an ongoing belief that his wife had been replaced by an amnesia of place, or phantom boarder phenomenon can by identical imposter. MRI showed selective anterior left temporal occur in other types of dementia such as Lewy body de- xb49PWcTrTwnNBxYZf3VaVIi+bbKrDculzKxD4UVNSjM0Z4o2bpF71rD53f4iR97XT+8XW+4kMtyfH+7ELAgxiUku60u23aAO/2LtfUeH4hQiDnsU7+Qqg== lobe atrophy. Quantitative EEG showed bilateral frontal and mentia and semantic dementia. temporal slowing. Neuropsychological profiling identified a CS has also been observed in patients with acquired broad range of deficits in the areas of naming, executive function, brain lesions. A comprehensive literature review of clinical and long-term memory. On the basis of these findings, we di- cases by Darby and Prasad (2016) showed that lesion-related agnosed frontotemporal dementia. This case demonstrates that delusional misidentification syndromes most commonly oc- CS can clinically accompany frontotemporal dementia. cur in cases where the right cerebral hemisphere is affected, Key Words: Capgras syndrome, frontotemporal dementia, in particular, the frontal lobe. CS can also manifest misidentification syndrome, magnetic resonance imaging throughout the course of other neurodegenerative disorders, such as Huntington disease, and can even be brought about (Cogn Behav Neurol 2019;32:134–138) by drug-induced psychosis (Gama Marques, 2015; Gama Marques and Carnot, 2016). CS = Capgras syndrome. FTD = frontotemporal dementia. CASE REPORT A 69-year-old, right-handed man presented to the Kemal Arikan Psychiatry Clinic with a persistent con- apgras syndrome (CS) clinically manifests as an viction that his wife of 43 years had been replaced by an C“imposter” delusion whereby the patient believes that identical imposter. The man was a well-educated person a person close to him or her (eg, a relative or friend) has andheldamaster’s degree in business administration. been replaced by an identical imposter (Kimura, 1986). He had retired 10 years ago, after working as a chief The delusion does not appear to be a facial recognition or accountant for various large-scale international compa- memory issue because CS patients often recognize that the nies. He had also lectured on business accounting at a imposter looks identical to the actual person they are re- respected institution in Turkey for a couple of years. ferring to (Barelle and Luaute, 2018; Berson, 1983). With regard to employment, the patient’s wife described However, the delusion can become a significant disability him as a highly conscientious, ambitious, and hard- on 06/14/2019 because the patient can become aggressive toward the working person. In his private life, she described him as a perceived imposter. cultured person who loved to read and had a full social Although CS is most frequently observed in patients life. The patient also enjoyed sports and had played in an with schizophrenia, it has also been identified in in- amateur-league football team until a few years ago. He dividuals with affective psychosis and organic psychotic was also a dutiful and caring father of two now-adult children and had seen to their education and personal development. Received for publication November 2, 2018; accepted February 10, 2019. From the *Department of Psychology, Uskudar University, Istanbul, Three years ago, the patient started experiencing Turkey; †Kemal Arikan Psychiatry Clinic, Istanbul, Turkey; and neuropsychological issues. Family members reported no- ‡Department of Neurology, School of Medicine, Istanbul University, ticing that the patient began to find it difficult to form Istanbul, Turkey. words (anomia). However, as this did not significantly The authors declare no conflicts of interest. ’ Correspondence: Mehmet K. Arikan, MD, PhD, Halaskargazi Cad. No: 103/ affect the patient s life, the family did not seek medical 4B, Osmanbey, Istanbul, Turkey 34371 (email: [email protected]). help at that time. A few months before his referral to our Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. clinic, the patient developed CS, which is what prompted 134 | www.cogbehavneurol.com Cogn Behav Neurol Volume 32, Number 2, June 2019 Copyright r 2019 Wolters Kluwer Health, Inc. All rights reserved. Cogn Behav Neurol Volume 32, Number 2, June 2019 Capgras Syndrome in Frontotemporal Dementia him to seek medical help. There was no family history of self-grooming and hygiene. His speech was spontaneous psychiatric or behavioral problems and, before this, the and was appropriate for the situation. His muscle strength patient had never been given a psychiatric or neurologic was normal, and no pathological reflexes were identified. diagnosis, nor had he ever used any psychotropic medi- Ataxia and dysmetria were absent, based on the finger-to- cations or illegal substances. His blood chemistry, a vita- nose test and performance of rapidly alternating move- min and hormone panel, and serological tests (such as for ments. Spontaneous gait and tandem walking were also venereal disease and the human immunodeficiency virus) normal; however, mild ideomotor apraxia was observed were all normal. when the patient was asked to mime brushing his teeth and When questioned in detail about his imposter delu- combing his hair, although his knowledge on the use of sion, the patient acknowledged that, although the im- these tools was intact. poster resembled his wife, he perceived his wife to be an imposter. This delusion persisted daily and caused mod- Neuroimaging—MRI erate to severe distress to the family, with the patient often An MRI including sagittal and axial T2-weighted exhibiting negative emotional reactions (namely ag- and coronal FLAIR sequences showed prominent atrophy gression) toward his wife. Sometimes, he even prevented of the patient’s left temporal lobe, including the hippo- her from doing household chores because only “his wife” campus. Interestingly, the atrophy was unilateral and was was allowed to do them. The patient also misidentified his not detected in the right temporal lobe or either frontal home, frequently not believing it to be his real house with lobe (Figure 1). his own furniture. The patient’s wife also reported that the patient had Neuroimaging—EEG recently developed an excessive and impulsive desire for Quantitative EEG and absolute power mapping was sexual activity. For example, he often suggested that he performed to quantify the power of commonly analyzed and his wife have “public” sexual intercourse (ie, while in EEG power bands: Delta (1–4 Hz), theta (4–8 Hz), alpha the presence of their children and grandchildren), which (8–12 Hz), beta (12–25 Hz), and high-beta (25–30 Hz) caused a great deal of embarrassment. As well as mis- showed a significant increase in slow theta and delta waves identifying his wife, the patient also often accused her of in the frontal and temporal lobe electrodes as compared to having an affair with another man. In addition, he was the population mean (ie, z-scored results) (Figures 2 and consistently compulsive in vocalizing his political beliefs 3). A significant increase in delta and delta bands suggests and frequently forced conversations on the topic of po- a slowing of brain activity. This result is consistent with litical issues, even with strangers (eg, on public transport). the MRI findings in that slowing was more prominent on According to his wife, the patient also extensively con- the left side of the brain. fabulated stories about visits to countries and places that he had never been to. All of these symptoms followed the Neuropsychological Testing onset of CS, and it was their cumulative detrimental effect The patient completed an interview-based neuro- that led the family to seek medical help. psychological battery composed of various tests measuring memory, language, executive functions, abstract reason- ASSESSMENTS ing, and visuospatial functions (Table 1). The tests were administered by an experienced neuropsychologist (S.A.K.). Neurologic Examination All of the tests are international neuropsychological measures On examination, the patient was cooperative and except the Verbal Memory Processes Scale (Öktem, 1992), was oriented to time, place, and people. He exhibited good which was developed in Turkey. However, all of the tests have FIGURE 1. MRI showing atrophy in the left temporal lobe (indicated with arrows). (Figure 1 can be viewed in color online at www. cogbehavneurol.com.) Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.