Delusional Misidentification Syndrome with Response to Donepezil and Behavioral Intervention in a Patient with Dementia
Received Date: Jun 11, 2017 / Accepted Date: Jul 17, 2017 / Published Date: Jul 21, 2017
Introduction: Delusional Misidentification Syndrome (DMS) encompasses a group of disorders in which a person persistently believes the identity of people, places, or objects are altered. Historically, described in psychotic disorders, DMS prevalence is 15.8% in Alzheimer's disease (AD) and 16.6% in dementia with Lewy bodies (DLB). We present a case of DMS in a patient with dementia that incorporates elements of mirrored self-misidentification and phantom boarder syndrome and therapeutic response to a combination of a behavioral intervention and donepezil.
Case: Patient is a 75-year-old white female presented with four months history of DMS and visual hallucinations. Patient perceived her own reflection in picture glass as an older lady who was trying to steal her "boyfriends." Her "boyfriends" were three pictures of soldiers in her apartment. MMSE was 27/30 (WORLD) and 23/30 (Serial 7s). MRI showed biparietal and right hippocampal atrophy. NPT showed impaired language, spatial abilities, memory, and executive control. She scored <1 percentile on category word fluency, judgement of line orientation, raw complex figures and Beery VMI. Patient was diagnosed with probable AD, using NINCDS-ADRDA and findings on neuropsychological testing (NPT) and MRI. DLB was excluded using McKeith's criteria. After a failed trial of risperidone, she received donepezil and family was instructed to remove photographs. MMSE stable with resolution of the mirrored selfmisidentification at 4 months follow up.
Conclusion: Patient's poor response to risperidone is consistent with previous studies suggesting limitations of antipsychotic treatment for psychotic symptoms in AD. Removal of potential symptom trigger along with an acetylcholinesterase inhibitor resulted in remission for up to 4 months. The potentiating effect of donepezil on the cholinergic component of the visuo-amygdaloid pathway/dorsal visual pathway may account for these changes.
Keywords: Delusional misidentification syndrome; Dementia; Poserior cortical atrophy; Alzheimer's disease; Cholinesterase inhibitors
Delusional misidentification syndromes (DMS) encompass a group of delusional disorders in which patients persistently believe that people, places, objects, or events have somehow been altered. They are usually associated with schizophrenia spectrum, but also reported with organic disorders like dementias, epilepsy, traumatic brain injury, subarachnoid hemorrhage  and stroke . The prevalence in Alzheimer’s dementia (AD) and dementia with Lewy Bodies (DLB) is estimated at 16%, with the prevalence somewhat higher in DLB . We present a case that incorporated elements of mirrored selfmisidentification (delusion that one’s reflection in the mirror is another person) and phantom boarder (delusion that someone uninvited is living in one’s house), resolved with combination of behavioral intervention and donepezil.
Ms. H was a 75-year-old female who presented with a four months history of visual hallucinations (VH) and DMS. She reported living in her apartment with 3 boyfriends and recently noticed an older woman trying to steal the boyfriends from her. Ms. H called the police several times because of the older woman. According to her daughter, the “boyfriends” were photographs of three soldiers, pictured from waist up (no legs visible in the pictures). Ms. H believed that those men lost their legs in a war. She carried those pictures around, believing they men couldn’t walk and needed her help. She acknowledged that they were photographs but believed that they had material bodies with certain needs. One of the pictures were of her late husband in his youth but patient didn’t acknowledge it. Ms. H also reported that she could see the reflection of the older woman trying to climb onto her boyfriends which was in fact her own reflection in the glass of picture frames. She claimed that old woman in reflection always wore the same jewelry and clothes as her which she must had stolen from her as well.
She scored 27 on MMSE WORLD and 23 with Serial 7s. Neuropsychological testing (NPT) showed impaired language, spatial abilities, immediate memory and executive control (Table 1). Dalyed recall was borderline abnormal. MRI showed bi-parietal, occipital and right hippocampal atrophy (Figures 1-3). She presented to us after a failed trial of risperidone. Given that her presentation and workup was more consistent with AD, she was started on donepezil 5 mg, titrated to 10 mg/day. The family was also instructed to put the photographs away to remove the presumed trigger.
|Cognitive Test||Description||(Raw scores & Interpretation)||Percentiles adjusted for age||Percentiles adjusted for age, sex & Education||Interpretation|
|MMSE||Used as baseline to assess general cognitive ability||World 27, serial seven 23||-||-||-|
|NAART IQ||Estimated premorbid intelligence||110||-||-||High Average|
|Boston naming test||Confrontational word retrieval||46||5||-||Borderline|
|CIFA letter word fluency||Language and academic skills||26||42||58||Normal|
|CIFA category word fluency||Language and academic skills||21||1||1||Defect|
|Beery Developmental test of visual-motor integration||Visual-motor construction & Integration||18||1||-||Defect|
|Clock drawing test||Visual-motor construction, visual-motor planning and organization||-||-||-||-|
|Benton judgement of
line orientaion test
|Line and angle discrimintaion||0||<1||-||Defect|
|Benton facial recognition test||Facial recgonition, Capgras-like visual decomposition of face||17||-||-||Defect|
|Rey complex figure copy||Visual-motor construction and Organization||5||<1||<1||Defect|
|Hopkins verbal learnig test (trials 1-3)||Verbal short term memory||14||8||14||Borderline|
|Hopkins verbal learning test (delayed recall)||delayed verbal memory and recall (delay 20-25 mins)||6||24||34||Normal|
|Hopkins verbal learning test retention (%)||Verbal memory retention||100||84||86||Normal|
|Hopkins delayed recognition index||-||6||2||4||Borderline|
|WMS-R logical memory
|Logical learning and memory||11||2||2||Defect|
|WMS-R logical memory
|Delayed logical memory and recall (delay: 20-25 mins)||9||8||12||Borderline|
|Wms-R Visual Reproduction
|Visual learning and memory||22||18||21||Normal|
|Wms-R Visual Reproduction
|Delayed visual memory and recall (delay: 20-25 mins)||3||10||12||Borderline|
|Wais iii digit span forward||Short-term memory, working memory and recall||5||16||18||Normal|
|Wais iii digit span backward||Short-term memory, working memory and recall||3||5||4||Borderline|
|Wais iii total digit span (forward + backward)||-||8||7||7||Borderline|
|Trail making test-a
(secs to complete)
|Visual attention and executive control||49||34||38||Normal|
|Trial making test-b
(secs to complete)
|Visual attention and executive control||d/c||-||-||Defect|
|DKEFS sorting test -
|Verbal and spatial concept formation and executive control||2||2||-||Defect|
|DKEFS sorting test -
|Verbal and spatial concept formation and executive control||6||2||-||Defect|
|DKEFS sorting test
- repeated sorts
|Verbal and spatial concept formation and executive control||1||50||-||Normal|
|Geriatric depression scale||-||7||-||-||Mild|
|Purpose in life scale||-||3||-||-||Average|
|Lawton brody adl/iadl,
|Self-reported activities of daily living||2||8||7||Borderline|
|Lawton brody adl/iadl,
|Informant reported activities of daily living||4||3||3||Borderline|
Table 1: Neuropsychiatric test results.
At 4 months follow-up, she demonstrated marked improvement stating, “That old lady is finally gone!” The photographs were put back after 2 weeks, as per her request, without recurrence of DMS. She still acknowledged the presence of her soldier friends but it was not distressful. Her MMSE remained stable.
Patient was diagnosed with probable AD, using NINCDS-ADRDA which was further supported on NPT and MRI . Patient did not meet the Mckeith’s Criteria for probable DLB as no cognitive fluctuations, parkinsonism or REM sleep behavioral disorder were observeded . Possible DLB still remained in differential given prominent visuospatial deficits, visual hallucinations (VH), exectutive dysfunction and the fact that AD and DLB frequently overlap. Further diagnositic modalities including transcranial magnetic stimulation study Posterior cortical atrophy (PCA) variant was also a consideration given prominent space and object percepion deficits, constructional dysparaxia seen on NPT and marked parieto-occipital and temporal atrophy seen on MRI. However, our patient lacked other frequently seen PCA features including Balint's syndrome (simultanagnosia, oculomotor apraxia, optic ataxia) or Gerstmann's syndrome (acalculia, agraphia, finger agnosia, left/right disorientation) .
Neuroanatomical correlations for VH and DMS include right mesiotemporal atrophy or involvement of ventral and dorsal visual streams. Ismail Z., in his review reported that delusions in AD are predominantly a right hemispheric phenomenon with medial temporal lobes specifically involved in DMS . A disconnection from right temporo-limbic regions may result into lack of insight into the visual stimuli and hence the wrong emotional responses attached to them.
The involvement of ventral (occipito-temporal) and dorsal (occipito-parietal) visual streams can also explain these symptoms. These pathways, through connections to the limbic system, are involved in processing facial recognition and generating affective responses, based on the familiarity to the stimulus. Any disruption along these pathways can lead to a false interpretation of the visual stimulus . Reeves et al. suggested a role of ventral visual pathway based on the poor performance on rapid visual processing tests that have documented association with these pathways . Diffusion tensor imaging studies also showed the involvement of an indirect occipito-temporal pathway (inferior longitudinal fasciculus) . Furthermore, post-mortem studies have shown increased tax burden along these pathways as well as in right hippocampal and parahippocampal regions [11-13].
Several studies have described the role of cholinesterase inhibitors (ChEI) in managing neuropsychiatric symptoms of AD, DLB, and Parkinon’s disease dementia (PDD) [14-16]. ChEI have also been reported to be beneficial in managining DMS in cases of DLB , vascular dementia  and PDD . In addition, antipsychotics and simple behavioral interventions have been used for DMS with some success, although antipsychotics are not without risk and side effects in such patients [20,21] (see Table 2). In our case, a combination of behavioral modification with donepezil was effective. Further doubleblind studies are needed to evaluate the role of ChEI in treating DMS associated with neurodegeneration.
|Study||# of Patients||DMSs Type||Underlying Pathology||Treatment||Outcome|
|Gil-Ruiz, et al.||1||Mirror Sign||Probable DLB||Behavioral Intervention||Mirror Sign replaced by Imposters/ another DMS|
|Oulis P, et al.||1||Capgras||Paranoid Schizophrenia complicated by Vascular Dementia||Donepezil,
|Peritogiannis V, et al.||1||Capgras||Vascular Dementia||Donepezil||Resolved|
|Reimers, et al.||2||Capgras||DLB||Donepezil||Resolved|
|Roane, et al.||3||Capgras,
|PDD||Clozapine||Resolved in 2/3 cases|
|Shiotsuki||1||Capgras||PDD||Failed trial with Donepezil,
Increased dose of levodopa/ carbidopa
|Marantz||1||Capgras||DLB||Spontaneous resolution with worsening of the disease|
|Pagonabarraga J, et al.||5||PDD||A) Quetiapine in 3 patients. B) Ziprasidone in 1 patients C) Riviastigmine initiated in 1 patient 4/5 patients were already on it||Improvement with Quetiapine in 2/3 patients. Improvement with Ziprasidone 1/1 patient. Marked improvement was observed in the one in which therapy was initiated with rivastigmine|
Table 2: Cases reporting successful management of DMS in neurodegenerative disorders
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