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Das klinische Syndrom der posterioren kortikalen Atrophie

Posterior cortical atrophy — the clinical syndrome

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Zusammenfassung

Unter der posterioren kortikalen Atrophie („posterior cortical atrophy“, PCA) wird ein klinisches Syndrom mit typischen neuropsychologischen Ausfällen verstanden. In der vorliegenden Arbeit wird der klinische und neuropsychologische Befund von vier Patienten mit PCA vorgestellt und ein Überblick über Charakteristika und Differenzialdiagnosen dieses Syndroms gegeben. Leitsymptome der PCA sind Beeinträchtigungen visueller und räumlicher Funktionen (meist in Form eines Balint-Syndroms), variabel ergänzt durch visuelle Perzeptionsstörungen, topographische Desorientiertheit, visuelle Agnosie sowie Defiziten beim Lesen, Kopieren, Zeichnen und Rechnen. Histopathologisch finden sich bei der PCA meist Alzheimer-typische Veränderungen mit Schwerpunkt in hinteren Hirnabschnitten. Im Unterschied zur Normalvariante der Alzheimer-Demenz sind mnestische und sprachliche Leistungen über lange Zeit relativ gut erhalten. SPECT- oder PET-Untersuchungen zeigen Perfusions- und Metabolismusstörungen in beiden Parietal- und Okzipitallappen. Die Diagnose der PCA stützt sich auf neuropsychologische Befunde sowie auf bildgebende Verfahren.

Summary

Posterior cortical atrophy (PCA) is a syndrome that involves distinct neuropsychological deficits. This paper presents the clinical and neuropsychological findings recorded in four patients with PCA and reviews the characteristics of the syndrome and other conditions that need to be considered in the differential diagnosis. The cardinal symptoms of PCA are deficits of higher visual and spatial functions (mostly taking the form of Balint’s syndrome), variably associated with disorders of visual perception, topographical disorientation, visual object agnosia and prosopagnosia, and deficits affecting reading, copying, drawing, and calculation. PCA is mostly associated with histopathological changes similar to those found in dementia of Alzheimer type (DAT), which are located predominantly in posterior brain regions. Memory and language functions tend to be preserved better and for a longer time in PCA than in the normal variant of DAT. SPECT and PET show deficits of perfusion and metabolism in both parietal and occipital lobes. The diagnosis of PCA is based on neuropsychological and imaging findings.

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Authors and Affiliations

  1. Klinik für Neurologie, Medizinische Universität Innsbruck, Österreich

    E. Karner, C. Jenner, M. Delazer & T. Benke

  2. Klinik für Nuklearmedizin, Medizinische Universität Innsbruck, Österreich

    E. Donnemiller

  3. Universitätsklinik für Neurologie, Anichstraße 35, 6020, Innsbruck, Österrreich

    E. Karner

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  1. E. Karner
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  2. C. Jenner
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  3. E. Donnemiller
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  4. M. Delazer
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  5. T. Benke
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Correspondence to E. Karner.

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Karner, E., Jenner, C., Donnemiller, E. et al. Das klinische Syndrom der posterioren kortikalen Atrophie. Nervenarzt 77, 208–214 (2006). https://doi.org/10.1007/s00115-005-1987-5

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