Capgras Delusion in Posterior Cortical Atrophy-A Quantitative Multimodal Imaging Single Case Study
Journal: 2020/June - Frontiers in Aging Neuroscience
Abstract:
Although Alzheimer's disease presents homogeneous histopathology, it causes several clinical phenotypes depending on brain regions involved. Beside the most abundant memory variant, several atypical variants exist. Among them posterior cortical atrophy (PCA) is associated with severe visuospatial/visuoperceptual deficits in the absence of significant primary ocular disease. Here, we report for the first time a case of Capgras delusion-a delusional misidentification syndrome, where patients think that familiar persons are replaced by identical "doubles" or an impostor-in a patient with PCA. The 57-year-old female patient was diagnosed with PCA and developed Capgras delusion 8 years after first symptoms. The patient did not recognize her husband, misidentified him as a stranger, and perceived him as a threat. Such misidentifications did not happen for other persons. Events could be interrupted by reassuring the husband's identity by the patient's female friend or children. We applied in-depth multimodal neuroimaging phenotyping and used single-subject voxel-based morphometry to identify atrophy changes specifically related to the development of the Capgras delusion. The latter, based on structural T1 magnetic resonance imaging, revealed progressive gray matter volume decline in occipital and temporoparietal areas, involving more the right than the left hemisphere, especially at the beginning. Correspondingly, the right fusiform gyrus was already affected by atrophy at baseline, whereas the left fusiform gyrus became involved in the further disease course. At baseline, glucose hypometabolism as measured by positron emission tomography (PET) with F18-fluorodesoxyglucose (FDG-PET) was evident in the parietooccipital cortex, more pronounced right-sided, and in the right frontotemporal cortex. Amyloid accumulation as assessed by PET with F18-florbetaben was found in the gray matter of the neocortex indicating underlying Alzheimer's disease. Appearance of the Capgras delusion was related to atrophy in the right posterior cingulate gyrus/precuneus, as well as right middle frontal gyrus/frontal eye field, supporting right frontal areas as particularly relevant for Capgras delusion. Atrophy in these regions respectively might affect the default mode and dorsal attention networks as shown by meta-analytical co-activation and resting state functional connectivity analyses. This case elucidates the brain-behavior relationship in PCA and Capgras delusion.
Keywords: Balint’s syndrome; Capgras delusion; MRI; delusional misidentification syndrome; posterior cortical atrophy.
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Front Aging Neurosci 12: 133

Capgras Delusion in Posterior Cortical Atrophy–A Quantitative Multimodal Imaging Single Case Study

Max Planck Institute for Human Cognitive and Brain Sciences, Leipzig, Germany
Clinic for Cognitive Neurology, University of Leipzig, Leipzig, Germany
Department of Nuclear Medicine, University of Leipzig, Leipzig, Germany
Edited by: Guido Gainotti, Università Cattolica del Sacro Cuore, Piacenza, Italy
Reviewed by: Nabin Koirala, Haskins Laboratories, United States; Sebastian Crutch, University College London, United Kingdom
*Correspondence: Matthias L. Schroeter, ed.gpm.sbc@teorhcs
Edited by: Guido Gainotti, Università Cattolica del Sacro Cuore, Piacenza, Italy
Reviewed by: Nabin Koirala, Haskins Laboratories, United States; Sebastian Crutch, University College London, United Kingdom
Received 2019 Sep 19; Accepted 2020 Apr 21.
This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

Abstract

Although Alzheimer’s disease presents homogeneous histopathology, it causes several clinical phenotypes depending on brain regions involved. Beside the most abundant memory variant, several atypical variants exist. Among them posterior cortical atrophy (PCA) is associated with severe visuospatial/visuoperceptual deficits in the absence of significant primary ocular disease. Here, we report for the first time a case of Capgras delusion—a delusional misidentification syndrome, where patients think that familiar persons are replaced by identical “doubles” or an impostor—in a patient with PCA. The 57-year-old female patient was diagnosed with PCA and developed Capgras delusion 8 years after first symptoms. The patient did not recognize her husband, misidentified him as a stranger, and perceived him as a threat. Such misidentifications did not happen for other persons. Events could be interrupted by reassuring the husband’s identity by the patient’s female friend or children. We applied in-depth multimodal neuroimaging phenotyping and used single-subject voxel-based morphometry to identify atrophy changes specifically related to the development of the Capgras delusion. The latter, based on structural T1 magnetic resonance imaging, revealed progressive gray matter volume decline in occipital and temporoparietal areas, involving more the right than the left hemisphere, especially at the beginning. Correspondingly, the right fusiform gyrus was already affected by atrophy at baseline, whereas the left fusiform gyrus became involved in the further disease course. At baseline, glucose hypometabolism as measured by positron emission tomography (PET) with F18-fluorodesoxyglucose (FDG-PET) was evident in the parietooccipital cortex, more pronounced right-sided, and in the right frontotemporal cortex. Amyloid accumulation as assessed by PET with F18-florbetaben was found in the gray matter of the neocortex indicating underlying Alzheimer’s disease. Appearance of the Capgras delusion was related to atrophy in the right posterior cingulate gyrus/precuneus, as well as right middle frontal gyrus/frontal eye field, supporting right frontal areas as particularly relevant for Capgras delusion. Atrophy in these regions respectively might affect the default mode and dorsal attention networks as shown by meta-analytical co-activation and resting state functional connectivity analyses. This case elucidates the brain-behavior relationship in PCA and Capgras delusion.

Keywords: Balint’s syndrome, Capgras delusion, delusional misidentification syndrome, MRI, posterior cortical atrophy

Acknowledgments

We thank the patient and spouse for giving permission to publish this case report.

Funding. MS has been supported by the German Research Foundation (DFG; SCHR 774/5-1), by the German Consortium for Frontotemporal Lobar Degeneration, funded by the German Federal Ministry of Education and Research (BMBF; FKZ O1GI1007A), by the Parkinson’s Disease Foundation (PDF-IRG-1307), and the Michael J. Fox Foundation (MJFF-11362).

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