Clinicopathologic subtype of Alzheimer's disease presenting as corticobasal syndrome

Nobutaka Sakae; Keith A. Josephs; Irene Litvan; Melissa E. Murray; Ranjan Duara; Ryan J. Uitti; Zbigniew K. Wszolek; Jay van Gerpen; Neil R. Graff-Radford; Dennis W. Dickson

doi:10.1016/j.jalz.2019.04.011

Clinicopathologic subtype of Alzheimer's disease presenting as corticobasal syndrome

Nobutaka Sakae, Keith A. Josephs, Irene Litvan, Melissa E. Murray, Ranjan Duara, Ryan J. Uitti, Zbigniew K. Wszolek, Jay van Gerpen, Neil R. Graff-Radford, Dennis W. Dickson

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

12 Scopus citations

Abstract

Introduction: The corticobasal syndrome (CBS) is associated with several neuropathologic disorders, including corticobasal degeneration and Alzheimer's disease (AD). Method: In this report, we studied 43 AD patients with CBS (AD-CBS) and compared them with 42 AD patients with typical amnestic syndrome (AD-AS), as well as 15 cases of corticobasal degeneration and CBS pathology. Results: Unlike AD-AS, AD-CBS had prominent motor problems, including limb apraxia (90%), myoclonus (81%), and gait disorders (70%). Alien limb phenomenon was reported in 26% and cortical sensory loss in 14%. Language problems were also more frequent in AD-CBS, and memory impairment was less frequent. AD-CBS had more tau pathology in perirolandic cortices but less in superior temporal cortex than AD-AS. In addition, AD-CBS had greater neuronal loss in the substantia nigra. Discussion: AD-CBS is a clinicopathological subtype of AD with an atypical distribution of Alzheimer-type tau pathology. Greater neuronal loss in the substantia nigra may contribute to Parkinsonism which is not a feature of typical AD.

Original language	English (US)
Pages (from-to)	1218-1228
Number of pages	11
Journal	Alzheimer's and Dementia
Volume	15
Issue number	9
DOIs	https://doi.org/10.1016/j.jalz.2019.04.011
State	Published - Sep 2019

Keywords

Alzheimer's disease
Corticobasal syndrome
Neurodegeneration
Neuropathology
Tau

ASJC Scopus subject areas

Epidemiology
Health Policy
Developmental Neuroscience
Clinical Neurology
Geriatrics and Gerontology
Cellular and Molecular Neuroscience
Psychiatry and Mental health

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10.1016/j.jalz.2019.04.011

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title = "Clinicopathologic subtype of Alzheimer's disease presenting as corticobasal syndrome",

abstract = "Introduction: The corticobasal syndrome (CBS) is associated with several neuropathologic disorders, including corticobasal degeneration and Alzheimer's disease (AD). Method: In this report, we studied 43 AD patients with CBS (AD-CBS) and compared them with 42 AD patients with typical amnestic syndrome (AD-AS), as well as 15 cases of corticobasal degeneration and CBS pathology. Results: Unlike AD-AS, AD-CBS had prominent motor problems, including limb apraxia (90%), myoclonus (81%), and gait disorders (70%). Alien limb phenomenon was reported in 26% and cortical sensory loss in 14%. Language problems were also more frequent in AD-CBS, and memory impairment was less frequent. AD-CBS had more tau pathology in perirolandic cortices but less in superior temporal cortex than AD-AS. In addition, AD-CBS had greater neuronal loss in the substantia nigra. Discussion: AD-CBS is a clinicopathological subtype of AD with an atypical distribution of Alzheimer-type tau pathology. Greater neuronal loss in the substantia nigra may contribute to Parkinsonism which is not a feature of typical AD.",

keywords = "Alzheimer's disease, Corticobasal syndrome, Neurodegeneration, Neuropathology, Tau",

author = "Nobutaka Sakae and Josephs, {Keith A.} and Irene Litvan and Murray, {Melissa E.} and Ranjan Duara and Uitti, {Ryan J.} and Wszolek, {Zbigniew K.} and {van Gerpen}, Jay and Graff-Radford, {Neil R.} and Dickson, {Dennis W.}",

note = "Publisher Copyright: {\textcopyright} 2019",

year = "2019",

month = sep,

doi = "10.1016/j.jalz.2019.04.011",

language = "English (US)",

volume = "15",

pages = "1218--1228",

journal = "Alzheimer's and Dementia",

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T1 - Clinicopathologic subtype of Alzheimer's disease presenting as corticobasal syndrome

AU - Sakae, Nobutaka

AU - Josephs, Keith A.

AU - Litvan, Irene

AU - Murray, Melissa E.

AU - Duara, Ranjan

AU - Uitti, Ryan J.

AU - Wszolek, Zbigniew K.

AU - van Gerpen, Jay

AU - Graff-Radford, Neil R.

AU - Dickson, Dennis W.

PY - 2019/9

Y1 - 2019/9

N2 - Introduction: The corticobasal syndrome (CBS) is associated with several neuropathologic disorders, including corticobasal degeneration and Alzheimer's disease (AD). Method: In this report, we studied 43 AD patients with CBS (AD-CBS) and compared them with 42 AD patients with typical amnestic syndrome (AD-AS), as well as 15 cases of corticobasal degeneration and CBS pathology. Results: Unlike AD-AS, AD-CBS had prominent motor problems, including limb apraxia (90%), myoclonus (81%), and gait disorders (70%). Alien limb phenomenon was reported in 26% and cortical sensory loss in 14%. Language problems were also more frequent in AD-CBS, and memory impairment was less frequent. AD-CBS had more tau pathology in perirolandic cortices but less in superior temporal cortex than AD-AS. In addition, AD-CBS had greater neuronal loss in the substantia nigra. Discussion: AD-CBS is a clinicopathological subtype of AD with an atypical distribution of Alzheimer-type tau pathology. Greater neuronal loss in the substantia nigra may contribute to Parkinsonism which is not a feature of typical AD.

AB - Introduction: The corticobasal syndrome (CBS) is associated with several neuropathologic disorders, including corticobasal degeneration and Alzheimer's disease (AD). Method: In this report, we studied 43 AD patients with CBS (AD-CBS) and compared them with 42 AD patients with typical amnestic syndrome (AD-AS), as well as 15 cases of corticobasal degeneration and CBS pathology. Results: Unlike AD-AS, AD-CBS had prominent motor problems, including limb apraxia (90%), myoclonus (81%), and gait disorders (70%). Alien limb phenomenon was reported in 26% and cortical sensory loss in 14%. Language problems were also more frequent in AD-CBS, and memory impairment was less frequent. AD-CBS had more tau pathology in perirolandic cortices but less in superior temporal cortex than AD-AS. In addition, AD-CBS had greater neuronal loss in the substantia nigra. Discussion: AD-CBS is a clinicopathological subtype of AD with an atypical distribution of Alzheimer-type tau pathology. Greater neuronal loss in the substantia nigra may contribute to Parkinsonism which is not a feature of typical AD.

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KW - Corticobasal syndrome

KW - Neurodegeneration

KW - Neuropathology

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Clinicopathologic subtype of Alzheimer's disease presenting as corticobasal syndrome

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