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DOI: 10.1055/s-0031-1281740
© Georg Thieme Verlag KG Stuttgart · New York
Die schwierige Diagnose der kortikobasalen Degeneration: Unterscheidung zwischen klinischem Syndrom und pathologischer Diagnose
The Diagnostic Challenge of Corticobasal Degeneration: Distinction between Clinical Syndrome and PathologyPublikationsverlauf
Publikationsdatum:
18. Oktober 2011 (online)

Zusammenfassung
Die kortikobasale Degeneration (CBD) ist eine neurodegenerative Erkrankung, welche durch einen progressiven Verlauf, asymmetrische extrapyramidale Symptome wie Rigor und Dystonie sowie variable kortikale Symptome wie Apraxie, kortikale Sensibilitätsstörungen, Alien-Limb-Phänomen und Reflexmyoklonus charakterisiert ist. Pathologisch finden sich charakteristische Veränderungen mit Taupathologie. Es gibt aber auch Patienten mit neurodegenerativen Erkrankungen anderweitiger Pathologie, welche sich klinisch wie eine CBD präsentieren. Daher hat sich zur Beschreibung des klinischen Bildes der Begriff des kortikobasalen Syndroms (CBS) durchgesetzt, während der Begriff CBD für die pathologische Entität reserviert ist. Auf der anderen Seite können sich Patienten mit typischer CBD-Pathologie klinisch wie eine Alzheimer-Krankheit (AD) oder progressive supranukleäre Paralyse (PSP) präsentieren. Anhand von zwei Kasuistiken zeigen wir diese klinisch-pathologische Heterogenität illustrativ auf. Der erste Patient entwickelte die typischen klinischen Symptome einer progressiven supranukleären Blicklähmung, wobei sich in der postmortalen Untersuchung des Gehirns eine CBD darstellte. Die zweite Patientin präsentierte sich mit einem CBS, während pathologisch die Befunde einer Alzheimer Krankheit erhoben wurden. Diese exemplarischen Falldarstellungen unterstreichen die Wichtigkeit, zwischen dem klinischen Syndrom des CBS und der pathologisch-definierten Entität der CBD zu unterscheiden.
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.
Schlüsselwörter
kortikobasales Syndrom - kortikobasale Degeneration - progressive supranukleäre Blicklähmung - Pathologie - frontotemporale Demenz
Keywords
corticobasal syndrome - corticobasal degeneration - progressive supranuclear palsy - pathology - frontotemporal dementia
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Dr. Oliver Kellner
Alterspsychiatrie, Integrierte Psychiatrie Winterthur
Postfach 144
8408 Winterthur
Schweiz
eMail: oliver.kellner@ipw.zh.ch