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Review
. 2023 Feb;64(2):121-126.
doi: 10.1007/s00108-022-01461-2. Epub 2023 Jan 20.

[Rare forms of dementia-Atypical variants of Alzheimer's dementia]

[Article in German]
Christine A F von Arnim  1 , Ingo Uttner  2
Affiliations
Review

[Rare forms of dementia-Atypical variants of Alzheimer's dementia]

[Article in German]
Christine A F von Arnim et al. Inn Med (Heidelb). 2023 Feb.

Abstract
in English, German

In Germany, around 1.8 million people currently suffer from dementia and the numbers are increasing. The main cause of dementia is Alzheimer's disease. This is classically manifested in the form of an amnestic syndrome but also encompasses various atypical variants, especially in younger patients and in the clinical routine are not always easy to recognize. These are described in this narrative review with case studies. Posterior cortical atrophy (PCA) presents with visual disorders, in the logopenic variants of primary progressive aphasia (lvPPA) impaired word retrieval is the main symptom, in the frontal variant of Alzheimer's disease behavioral disorders are prominent and in corticobasal syndrome (CBS) an akinetic rigid Parkinson's syndrome with alien limb phenomenon. As the clinical presentation of these atypical variants shows an overlap with other dementia disorders, the differential diagnosis is often challenging. In this context amyloid biomarkers can provide valuable services.

Demenzerkrankungen werden immer häufiger. In Deutschland sind aktuell rund 1,8 Mio. Menschen an Demenz erkrankt. Die Hauptursache von Demenzerkrankungen ist die Alzheimer-Erkrankung. Diese äußert sich klassischerweise in Form eines amnestischen Syndroms, umfasst aber auch verschiedene atypische Varianten, die vor allem jüngere Patienten betreffen und im klinischen Alltag nicht immer einfach zu erkennen sind. Diese werden im folgenden narrativen Review, teils mit Kasuistiken unterlegt, beschrieben. Die posteriore kortikale Atrophie (PCA) präsentiert sich mit visuellen Störungen, bei der logopenischen Variante der primär progredienten Aphasie (lvPPA) stehen Wortfindungsstörungen im Vordergrund, bei der frontalen Variante der Alzheimer-Erkrankung Verhaltensstörungen und beim kortikobasalen Syndrom (CBS) ein akinetisch-rigides Parkinson-Syndrom mit Apraxie und „Alien-limb-Phänomen“. Da das klinische Bild dieser atypischen Varianten eine Überlappung mit anderen Demenzerkrankungen zeigt, ist die Differenzialdiagnose oft herausfordernd. Amyloid-Biomarker können hier wertvolle Dienste leisten.

Keywords: Alzheimerʼs disease; Aphasia, primary progressive/logopenic variant; Corticobasal syndrome; Frontotemporal dementia; Posterior cortical atrophy.

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References

Literatur

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