Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology

George Naasan; Tal Shany-Ur; Manu Sidhu; Cynthia Barton; Robin Ketelle; Suzanne M. Shdo; Joel H. Kramer; Bruce L. Miller; William W. Seeley

doi:10.1080/13554794.2019.1604973

Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology

George Naasan^*, Tal Shany-Ur, Manu Sidhu, Cynthia Barton, Robin Ketelle, Suzanne M. Shdo, Joel H. Kramer, Bruce L. Miller, William W. Seeley

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

4 Scopus citations

Abstract

Corticobasal syndrome and dementia with Lewy bodies are clinical presentations with unique and overlapping features but distinct pathological substrates. We report the case of an 80 year-old man who presented with apraxia, rigidity, slowness, right arm myoclonus, a 10-year history of probable REM-sleep behavior disorder, and later developed visual hallucinations. At autopsy, he had pathological features of corticobasal degeneration, and Lewy body disease confined to the brainstem. This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem.

Original language	American English
Pages (from-to)	26-33
Number of pages	8
Journal	Neurocase
Volume	25
Issue number	1-2
DOIs	https://doi.org/10.1080/13554794.2019.1604973
State	Published - 4 Mar 2019
Externally published	Yes

Bibliographical note

Funding Information:
This work was supported by grant number P50AG023501 from NIH National Institute on Aging, the Hellman Family Foundation, and the Consortium on Frontotemporal Dementia Research.

Publisher Copyright:
© 2019, © 2019 Informa UK Limited, trading as Taylor & Francis Group.

Keywords

Corticobasal syndrome
corticobasal degeneration
dementia with Lewy body disease
pathology
tau
α-synuclein

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10.1080/13554794.2019.1604973

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title = "Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology",

abstract = "Corticobasal syndrome and dementia with Lewy bodies are clinical presentations with unique and overlapping features but distinct pathological substrates. We report the case of an 80 year-old man who presented with apraxia, rigidity, slowness, right arm myoclonus, a 10-year history of probable REM-sleep behavior disorder, and later developed visual hallucinations. At autopsy, he had pathological features of corticobasal degeneration, and Lewy body disease confined to the brainstem. This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem.",

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AU - Kramer, Joel H.

AU - Miller, Bruce L.

AU - Seeley, William W.

N1 - Funding Information: This work was supported by grant number P50AG023501 from NIH National Institute on Aging, the Hellman Family Foundation, and the Consortium on Frontotemporal Dementia Research. Publisher Copyright: © 2019, © 2019 Informa UK Limited, trading as Taylor & Francis Group.

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AB - Corticobasal syndrome and dementia with Lewy bodies are clinical presentations with unique and overlapping features but distinct pathological substrates. We report the case of an 80 year-old man who presented with apraxia, rigidity, slowness, right arm myoclonus, a 10-year history of probable REM-sleep behavior disorder, and later developed visual hallucinations. At autopsy, he had pathological features of corticobasal degeneration, and Lewy body disease confined to the brainstem. This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem.

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Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology

Abstract

Bibliographical note

Keywords

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