A Case of Capgras Syndrome with Frontotemporal Dementia

cantly fi cult to form fi s wife described ’ nd it dif rodegenerative disorders, n patients with acquired fi iterature review of clinical † lobe. CS can also manifest and can even be brought about (Gama Marques, 2015; Gama Volume 32, Number 2, June 2019 CASE REPORT cation syndromes most commonly oc- s degree in business administration. ’ fi s life, the family did not seek medical ’ cation syndromes such as CS, reduplicative par- Cogn Behav Neurol A 69-year-old, right-handed man presented to the Three years ago, the patient started experiencing CS has also been observed i fi help at that time.clinic, A the few patient months developed before CS, his which referral is to what our prompted affect the patient words (anomia). However, as this did not signi Kemal Arikan Psychiatryviction Clinic that his with wifeidentical a of imposter. 43 persistent The years con- man hadandheldamaster been was replaced a by well-educatedHe an person had retiredaccountant 10 for years variousnies. large-scale ago, He international after had compa- workingrespected also as institution lectured a on inWith chief regard business Turkey to accounting employment, forhim at the a patient a as coupleworking a person. of In highly his years. privatecultured life, conscientious, person she described who ambitious, him lovedlife. as The to a and patient read also hard- and enjoyedamateur-league sports had football and a had team full playedwas until in social a an also few achildren years dutiful ago. and and He haddevelopment. caring seen father to of their two education now-adult andneuropsychological personal issues. Familyticing members that reported no- the patient began to cases by Darby and Prasaddelusional (2016) misidenti showed that lesion-related syndromes (Kimuradementia 1986), (Harwood as etet well al, al 1999). as (1999) Forwho in instance, reported Harwood CS patients presentation hadHarciarek with in 10% and been ofidenti Kertesz patients diagnosed (2008)amnesia also of with place, claimedoccur or Alzheimer in phantom that other boardermentia types mis- disease. phenomenon and of can semantic dementia dementia. such as Lewybrain body lesions. de- A comprehensive l cur in cases wherein the right particular, cerebralthroughout hemisphere the the is course affected, ofsuch frontal other as neu Huntington disease, by drug-induced psychosis Marques and Carnot, 2016). EPORT and Nevzat Tarhan, MD* R ‡ ed a ASE fi C ed in in- fi ndings, we di- cant disability fi fi ling identi fi 2019 Wolters Kluwer Health, Inc. All rights reserved. r Frontotemporal Dementia 138) – frontotemporal dementia. Sukriye A. Kalem, PhD, MD, PhD, Halaskargazi Cad. No: 103/ = Baris Metin, MD, PhD,* Mehmet K. Arikan, MD, PhD,* A Case of Capgras Syndrome With icts of interest. FTD fl Copyright 2019;32:134 cits in the areas of naming, executive function, delusion whereby the patient believes that fi ” Capgras syndrome, frontotemporal dementia, Kemal Arikan Psychiatry Clinic, Istanbul, Turkey; and † Capgras syndrome (CS), also called imposter syn- cation syndrome, magnetic resonance imaging fi www.cogbehavneurol.com

Although CS is most frequently observed in patients imposter | Capgras syndrome. apgras syndrome“ (CS) clinically manifests as an Department of Neurology, School of Medicine, Istanbul University, =

Turkey; ‡ Istanbul, Turkey. 4B, Osmanbey, Istanbul, Turkey 34371 (email: [email protected]). Cogn Behav Neurol because the patientperceived imposter. can become aggressive towardwith the schizophrenia,dividuals it with has affective psychosis also and been organic psychotic identi a person close tobeen him replaced or by herThe (eg, an delusion does a identical not relativememory appear imposter or issue to friend) because (Kimura, be CS has a 1986). patientsimposter facial often looks recognition recognize or identical that the ferring to the to actual personHowever, (Barelle the they delusion are and can re- become Luaute, a signi 2018; Berson, 1983). 134

Received for publication November 2,From 2018; accepted the February 10, *Department 2019. of Psychology, Uskudar University, Istanbul, C and long-term memory. On the basis of these Abstract: drome, is a rare psychiatricdelusion condition that that is a characterized family byby relative the an or identical close friend imposter.with has CS Here, been who we replaced presented describewith to a an the 69-year-old ongoing Kemal man identical belief Arikan imposter. that Psychiatry MRI Clinic his showedlobe selective wife atrophy. anterior had left Quantitative beentemporal temporal EEG replaced slowing. showed by bilateral an Neuropsychological frontal pro and The authors declare no con Correspondence: Mehmet K. Arikan, agnosed frontotemporal dementia. ThisCS can case clinically demonstrates accompany that frontotemporal dementia. Key Words: CS misidenti ( broad range of de Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.

Downloaded from http://journals.lww.com/cogbehavneurol by xb49PWcTrTwnNBxYZf3VaVIi+bbKrDculzKxD4UVNSjM0Z4o2bpF71rD53f4iR97XT+8XW+4kMtyfH+7ELAgxiUku60u23aAO/2LtfUeH4hQiDnsU7+Qqg== on 06/14/2019 Downloaded from http://journals.lww.com/cogbehavneurol by xb49PWcTrTwnNBxYZf3VaVIi+bbKrDculzKxD4UVNSjM0Z4o2bpF71rD53f4iR97XT+8XW+4kMtyfH+7ELAgxiUku60u23aAO/2LtfUeH4hQiDnsU7+Qqg== on 06/14/2019 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.)

FIGURE 2. Z-scored fast fourier transform summary. (Figure 2 can be viewed in color online at www.cogbehavneurol.com.) been validated for the Turkish population and this specific cue. The patient also had semantic, phonemic, and neo- patient’s age group; education-adjusted norms are also logistic paraphasias. During the Cookie Theft task from available. the Boston Diagnostic Aphasia Examination (Giles et al, 1996), the patient used neologisms and exhibited phone- Memory mic paraphasias. These results indicate severe naming and The Verbal Memory Processes Scale measures both language impairment. verbal short-term and long-term memory, including delayed retrieval and recognition after delay. During the test, Executive Functions (see Faria et al, 2015, for Details a 10-item word list is repeated eight times. Regarding of the Tests) short-term memory, the patient remembered only four of During the Digit Span Test, the patient was able to the items, indicating a severe deficit. For delayed recall, repeat only five digits forward and only two digits back- the patient was unable to recall any of the items, in- ward. This performance was interpreted as showing dicating a severe impairment. And, in terms of recognition problems with attention span and working memory. after delay, the patient recognized only half of the items During the Clock-Drawing Test, the patient wrote num- that he had been shown and falsely recognized five words. bers greater than 12 on the clock face and placed the clock These results indicate mixed-type memory impairment, hands incorrectly. This pattern indicates a planning and with both hippocampal and frontal deficits. conceptual deficit in addition to a perseverative pattern. On the Stroop Test, the patient could not name any of the Language colors under the incongruent condition. This performance On the Boston Naming Test (Lansing et al, 1999), shows problems with interference control and inhibition. the patient was able to name only four items (out of 31) During the Verbal Fluency Test, the patient is first spontaneously, indicating severe anomia. For the re- required to produce names from a certain category (eg, mainder of the test, he required a phonemic or semantic animals), then to name objects starting with the letters K,

FIGURE 3. EEG trace with prominent frontal slowing.

’ DISCUSSION TABLE 1. The Patient s Neuropsychological Evaluation of fi — Memory (Short-term, Long-term, Delayed Recall), Language, Taken together, the de cits displayed by our patient Executive Functions, Abstract Reasoning, and Visuospatial dysexecutive neuropsychological pattern, disinhibition, and Planning perseverations—and the MRI findings led to the diagnosis of Ability Test Interpretation probable frontotemporal dementia (FTD), in accordance with the Neary criteria (Mohandas and Rajmohan, 2009). Memory Verbal Memory Severely impaired short-term The most likely subtype of FTD is semantic dementia, based Processes memory and mixed-type on the patient’s prominent naming deficits and MRI scans of Scale memory impairment (frontal characteristic anterior temporal lobe atrophy (Rascovsky and limbic) and Grossman, 2013). On the other hand, the patient also Language Boston Naming Severe anomia Test exhibited severe executive dysfunction and impaired working Cookie Theft Severe language impairment memory, which is not typical of semantic dementia. This task combination of the severe behavioral phenotype and the Executive Digit Span Test Problems with attention span dysexecutive pattern suggests a behavioral variant of FTD Functions and working memory (Pressman and Miller, 2014). Clock-Drawing Planning and conceptual deficit The patient was prescribed an antipsychotic (ris- Test peridone, titrated to 3 mg) to control his delusions and Stroop Test Severely impaired interference sexual disinhibition. He benefited significantly from this control and inhibition treatment; his wife reported a marked decrease in his Verbal Fluency Severely impaired semantic disinhibited sexual behavior and CS symptoms after a few Test information processing Abstract WAIS–IV Severely impaired abstract days. Despite a lack of evidence (Boxer et al, 2013; Li reasoning Similarities reasoning et al, 2015), off-label therapeutic trials of donepezil (10 subtest mg) and memantine (20 mg) were initiated; however, ac- Visuospatial Benton Line Mild impairment cording to his wife’s report, the patient showed no im- planning Orientation provement in naming and memory functions following Test these medications. In the last visit after 6 months of Benton Facial Normal treatment initiation, the symptoms related to CS were still Recognition controlled with risperidone, and no improvement in the Test patient’s other cognitive symptoms was described. WAIS–IV = Wechsler Adult Intelligence Scale, Fourth Edition. It is noteworthy that the patient exhibited marked asymmetrical temporal lobe atrophy on an MRI. Asym- metrical temporal lobe involvement has been reported in A, and S. The patient managed to produce only two ani- 35% of patients with a behavioral variant of FTD (Whitwell mal names in the allotted 1 minute and only seven words et al, 2013), with some studies demonstrating that the side of the brain that is affected in FTD confers the types of neu- starting with the letters K, A, and S. This performance is fi consistent with severely impaired semantic information ropsychological de cits that will be displayed by patients. processing. For example, the right-sided variant is associated with behavioral dyscontrol, personality changes, aphasia, and prosopagnosia (Josephs et al, 2009), whereas the left-sided Abstract Reasoning variant primarily manifests as language deficits (Boone et al, We used the Similarities subtest of the Wechsler 1999; Razani et al, 2001). In the present case, MRI revealed Adult Intelligence Scale, Fourth Edition (Wechsler, 2008), exclusively left-sided involvement, although, interestingly, in ’ to measure the patient s abstract reasoning. The patient addition to language impairment, the patient also displayed could detect the similarity correctly for only two out of 20 severe behavioral problems (reflected in CS). items, indicating severe impairment. A recent study reported that the brain lesions underlying CS are located in the right frontal cortex and the Visuospatial Planning left retrosplenial cortex, which are associated with belief We used the Benton Judgment of Line Orientation evaluation and familiarity, respectively (Darby et al, Test (Qualls et al, 2000) and the Benton Facial Recog- 2017). Therefore, in our patient’s case, selective left tem- nition Test (Tranel et al, 2009) to measure the patient’s poral atrophy may not by itself have been responsible for visuospatial function. The Line Orientation Test involves the CS; it is possible that abnormalities in the neuronal finding the match of lines with different orientations to the networks within this region may have played a pathogenic lines in a response card. The patient’s performance was role in the clinical phenotype. Interestingly, other authors only slightly impaired as he scored 17 out of 30; a score of have also proposed that CS occurs due to a lack of com- 20 is considered to be normal. The Benton Facial Rec- munication between the sensory and limbic systems ognition Test involves recognition of a target face among (Ramachandran, 1998). a set of different faces. The patient’s score on this test was Harciarek and Kertesz (2008) studied a large group of normal (41 out of 54). These findings indicate that the patients with neurodegenerative disorders including Alzheimer patient’s visuospatial function was largely preserved. disease, FTD, Parkinson disease, and Parkinson-plus

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