Capgras-like visual decomposition in Lewy body dementia with therapeutic response to donepezil

Abstract

D efects in visual/spatial cognition, including hallucinations and Capgras syndrome, are common in Lewy body dementia (LBD).1 In Capgras syndrome, a person holds a delusion that a friend, spouse, parent, or other close family member has been replaced by an identical-looking impostor. These visual phenomena frequently induce anxiety and agitation, and the only treatment currently available is neuroleptics. Neuroleptics have severe side effects in LBD as well as a black box warning for increased risk of death and stroke in elderly patients with dementia. We report 2 cases of LBD where visual phenomena including Capgras-like syndrome responded favorably to the use of a cholinesterase inhibitor. Case reportsCase 1 A 79-year-old man presented with a 3-year history of progressive word-finding difficulties and memory decline. His symptoms fulfilled criteria for probable LBD including cognitive impairment, fluctuating attention/concentration, and REM sleep behavior disorder. 2Neuropsychological testing was consistent with LBD (table e-1 at Neurology.org/cp). He alleged that his wife was an intruder with similar facial features. He did not recognize his wife during these periods and was uncooperative with her efforts to assist him. He was started on donepezil 5 mg, which was subsequently titrated to 10 mg daily after 4 weeks, and it was well-tolerated. These episodes became less frequent within 2 weeks of initiation of treatment parallel to improvement in cognitive function (Mini-Mental State Examination [MMSE] 19-28). He then was able to reconstruct the image of her face, starting with a unique mole on her cheek. Within a year, severe depression necessitated psychiatric admission, during which donepezil was discontinued. He developed paranoia and experienced visual hallucinations of Podcast Practical ImplicationsConsider cholinesterase inhibitors for visual hallucinations or Capgras syndrome first.

Activation of Somatostatin Interneurons by Nicotinic Modulator Lypd6 Enhances Plasticity and Functional Recovery in the Adult Mouse Visual Cortex

“…The selective expression of Lypd6 in deep-layer subpopulations of SST interneurons could limit offtarget effects (Timmermann et al, 2012;Wang et al, 2015a,b) and make them attractive as novel therapeutic targets, compared with interventions that do not regard subtype or receptor subunit specificity. Novel pharmacological interventions may be combined with behavioral interventions like physical exercise (Kalogeraki et al, 2014), which may further enhance the impact on SST interneuron activity (Polack et al, 2013;Fu et al, 2014;Reimers et al, 2014;Pakan et al, 2016;Dipoppa et al, 2018).…”

Delusional Misidentification Syndrome with Response to Donepezil and Behavioral Intervention in a Patient with Dementia

“…Several studies have described the role of cholinesterase inhibitors (ChEI) in managing neuropsychiatric symptoms of AD, DLB, and Parkinon's disease dementia (PDD) [14][15][16]. ChEI have also been reported to be beneficial in managining DMS in cases of DLB [17], vascular dementia [18] and PDD [19]. 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).…”

Evidence-Based Review of Therapeutic Approaches in Dementia with Lewy Bodies

“…In an uncontrolled study, there was improvement in the visual symptoms on administering Donepezil in 13 patients with LBD (p=0.009) [25,26]. In a Case series, the use of Donepezil in patients with Capgras Syndrome was effective [27][28][29][30]. Not only does Donepezil cause regression of neuropsychiatric symptoms, like visual hallucinations; but increasing the dose of Donepezil has also been shown to treat relapses [27,28].…”