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Die schwierige Diagnose der kortikobasalen Degeneration: Unterscheidung zwischen klinischem Syndrom und pathologischer Diagnose


Kellner, O; Sabova, K; Neumann, M; Jung, H H (2011). Die schwierige Diagnose der kortikobasalen Degeneration: Unterscheidung zwischen klinischem Syndrom und pathologischer Diagnose. Fortschritte der Neurologie, Psychiatrie, 79(11):660-667.

Abstract

Corticobasal degeneration (CBD) is a neurodegenerative disease characterised by linear progression, asymmetrical and extrapyramidal symptoms such as rigor and dystonia, as well as by variable cortical symptoms including apraxia, cortical sensory deficits, the alien limb phenomenon and myoclonism of the reflexes. Pathological changes of CBD consist of characteristic taupathology in the gray and white matter. However, there are also patients with neurodegenerative diseases with a different underlying pathology that nevertheless appear clinically as CBD. For that reason, the term corticobasal syndrome (CBS) is commonly used to describe the clinical features, whereas the term CBD is reserved for the pathological entity. Moreover, patients with the typical pathology of CBD can present clinical signs consistent with a clinical diagnosis of Alzheimer's disease (AD) or progressive supranuclear palsy (PSP). We demonstrate this clinico-pathological heterogeneity by presenting two illustrative case reports. The first patient developed the typical clinical symptoms of progressive supranuclear palsy, while exhibiting pathologically CBD. The second patient showed clinical signs of CBS, although pathologically she was diagnosed with Alzheimer's disease. These exemplary cases underscore the need to distinguish carefully between the clinical syndrome of CBS and the pathologically defined entity of CBD.

Corticobasal degeneration (CBD) is a neurodegenerative disease characterised by linear progression, asymmetrical and extrapyramidal symptoms such as rigor and dystonia, as well as by variable cortical symptoms including apraxia, cortical sensory deficits, the alien limb phenomenon and myoclonism of the reflexes. Pathological changes of CBD consist of characteristic taupathology in the gray and white matter. However, there are also patients with neurodegenerative diseases with a different underlying pathology that nevertheless appear clinically as CBD. For that reason, the term corticobasal syndrome (CBS) is commonly used to describe the clinical features, whereas the term CBD is reserved for the pathological entity. Moreover, patients with the typical pathology of CBD can present clinical signs consistent with a clinical diagnosis of Alzheimer's disease (AD) or progressive supranuclear palsy (PSP). We demonstrate this clinico-pathological heterogeneity by presenting two illustrative case reports. The first patient developed the typical clinical symptoms of progressive supranuclear palsy, while exhibiting pathologically CBD. The second patient showed clinical signs of CBS, although pathologically she was diagnosed with Alzheimer's disease. These exemplary cases underscore the need to distinguish carefully between the clinical syndrome of CBS and the pathologically defined entity of CBD.

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Additional indexing

Other titles:The diagnostic challenge of corticobasal degeneration: Distinction between clinical syndrome and pathology
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Neurology
04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:German
Date:2011
Deposited On:10 Nov 2011 14:27
Last Modified:05 Apr 2016 15:04
Publisher:Thieme
ISSN:0720-4299
Publisher DOI:https://doi.org/10.1055/s-0031-1281740
PubMed ID:22009274
Permanent URL: https://doi.org/10.5167/uzh-50708

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