Neuronale Zeroidlipofuszinose bei einem adulten American Staffordshire Terrier

Zusammenfassung

Der Fall eines weiblichen, 5 Jahre alten American Staffordshire Terriers mit hochgradigen progressiven neurologischen Ausfallserscheinungen, insbesondere in Form von Ataxie und Balanceproblemen, wurde pathomorphologisch aufgearbeitet und genetisch untersucht. In Neuronen unterschiedlicher Lokalisationen des Zentralnervensystems zeigte sich eine Akkumulation von feingranulärem, schwach eosinophilem oder hellbräunlichem Material im Zytoplasma. Zusätzlich bestanden eine hochgradige Degeneration und ein Verlust von Purkinjeund inneren Körnerzellen des Kleinhirns. Das akkumulierte PAS-positive, argyrophile, autofluoreszierende Material wies elektronenmikroskopisch eine lamelläre Struktur auf und wurde als Lipofuszin interpretiert. Eine genetische Analyse bestätigte das Vorliegen einer Variante im ARSG-Gen, das für das lysosomale Enzym Arylsulfatase G kodiert. Der Fallbericht beschreibt eine neuronale Zeroidlipofuszinose bei einem adulten Hund, die Ähnlichkeiten mit der Kufs-Krankheit des Menschen aufweist.

Schlüsselwörter

Hund - zerebelläre Degeneration - lysosomale Speicherkrankheit - Erbkrankheit

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