Clinicopathologic Subtype of Alzheimer's Disease Presenting As Corticobasal Syndrome

Overview

Journal Alzheimers Dement

Specialties Neurology
Psychiatry

Date 2019 Aug 11

PMID 31399334

Citations 19

Authors

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

Affiliations

Soon will be listed here.

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.

Citing Articles

Parkinsonism in Alzheimer's disease without Lewy bodies in association with nigral neuron loss: A data-driven clinicopathologic study.

Ono D, Sekiya H, Maier A, Murray M, Koga S, Dickson D Alzheimers Dement. 2025; 21(3):e14628.

PMID: 40042515 PMC: 11881629. DOI: 10.1002/alz.14628.

A Case of Combined Corticobasal Degeneration and Alzheimer's Disease Pathology: Clinical Presentation, and Diagnosis.

Baek S, Chae S, Kim J, Kim H Dement Neurocogn Disord. 2025; 24(1):69-73.

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Comparing classic-onset corticobasal syndrome to speech/language-onset corticobasal syndrome.

Garcia-Guaqueta D, Stephens Y, Ali F, Utianski R, Duffy J, Clark H Parkinsonism Relat Disord. 2024; 125:107025.

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Connectome-based modelling of neurodegenerative diseases: towards precision medicine and mechanistic insight.

Vogel J, Corriveau-Lecavalier N, Franzmeier N, Pereira J, Brown J, Maass A Nat Rev Neurosci. 2023; 24(10):620-639.

PMID: 37620599 DOI: 10.1038/s41583-023-00731-8.

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Donato L, Morda D, Scimone C, Alibrandi S, DAngelo R, Sidoti A Biomedicines. 2023; 11(7).

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