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Abnormale Repräsentationen im visuellen Kortex von Albinismus-Patienten

Hilfsmittel bei der Diagnostik und Modell der kortikalen Selbstorganisation

Abnormal representations in the visual cortex of patients with albinism

Diagnostic aid and model for the investigation of the self-organisation of the visual cortex

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Zusammenfassung

Ein charakteristisches Kennzeichen von Albinismus-Patienten ist die Fehlprojektion der Sehnerven, die einen abnormalen Eingang der Informationen in den visuellen Kortex bedingt. Im vorliegenden Beitrag wird erläutert, wie durch die Detektion der Fehlprojektion mit visuell evozierten Potenzialen die klinische Diagnose von Albinismus unterstützt werden kann. Darüber hinaus wird verdeutlicht, dass Albinismus aufgrund der Projektionsabnormalität der Sehnerven ein Modell für die Selbstorganisation des menschlichen visuellen Kortex darstellt. Dazu werden aktuelle Ergebnisse vorgestellt, die mit funktioneller Magnetresonanztomographie, visuell evozierten Potenzialen und statischer Gesichtsfeldperimetrie erhoben wurden.

Abstract

A characteristic feature of patients with albinism is the misrouting of the optic nerves, which causes the visual cortex to receive an abnormal input. This report details how the detection of misrouting using visual evoked potentials assists the clinical diagnosis of albinism. Further, it shows how the projection abnormality observed in patients with albinism provides a model for investigating the self-organisation of the human visual cortex. This is highlighted by recent findings that were obtained using functional magnetic resonance imaging, visual evoked potentials, and static visual field perimetry.

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Hoffmann, M., Schmidtborn, L. & Morland, A. Abnormale Repräsentationen im visuellen Kortex von Albinismus-Patienten. Ophthalmologe 104, 666–673 (2007). https://doi.org/10.1007/s00347-007-1589-7

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