Creutzfeldt-Jakob Erkrankung versus Anti-NMDA-Rezeptor-Enzephalitis – eine Kasuistik

Zusammenfassung

Die klinische Differenzierung zwischen Autoimmun- und neurodegenerativen Erkrankungen kann im Einzelfall eine diagnostische Herausforderung bedeuten. Die Differentialdiagnostik rasch progredienter neurologischer und kognitiver Symptome schließt entzündliche oder neoplastische Enzephalopathien, zerebrale Tumore oder Vaskulitiden, aber auch seltene neurodegenerative Erkrankungen wie z. B. die Creutzfeldt-Jakob Erkrankung ein. Vor dem Hintergrund des Einsatzes von immunsuppressiven Maßnahmen ist die Detektion eines Autoimmunprozesses in Kombination mit einer Creutzfeldt-Jakob Erkrankung von erheblicher therapeutischer Bedeutung. Die folgende Kasuistik stellt einen differentialdiagnostisch interessanten und seltenen Fall vor, bei dem sowohl eine Autoimmun- als auch Prionerkrankung diskutiert wurde.

Abstract

Clinically differentiating between autoimmune and neurodegenerative disorders can often pose a diagnostic challenge. The differential diagnosis of rapidly progressing neurological and cognitive symptoms includes central nervous system tumours, cerebral vasculitis, and inflammatory, autoimmune, or paraneoplastic encephalopathies. Rarer neurodegenerative diseases such as Creutzfeldt-Jakob disease should also be considered. Detection of treatable causes, such as autoimmune disorders, remains important when potentially occurring in conjunction with Creutzfeldt-Jakob disease. The following report describes a rare case in which autoimmune encephalopathy and prion disease were considered as possible comorbidities.

Schlüsselwörter

Anti-NMDA receptor encephalitis - Creutzfeldt-Jakob disease - Immunotherapy

Key words

Anti-NMDA-Rezeptor-Enzephalitis - immunsuppressive Therapie - Creutzfeldt-Jakob-Erkrankung

Publication History

Received: 08 March 2021

Accepted: 14 October 2021

Article published online:
05 January 2022

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