Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease

Abstract

Our experience suggests the following regimen for the diagnosis of suspected Creutzfeldt-Jakob disease: light microscopy of various brain regions, which in typical cases may lead to definite diagnosis. Immunohistochemistry with antibodies against the prion protein is preferable in all suspected cases of Creutzfeldt-Jakob disease and is mandatory whenever a routine histological workup does not yield definite results. Additional special techniques can be applied if required.

Different protein profiles in inferior colliculus and cerebellum: A comparative proteomic study

“…This indicates the importance of 14-3-3 proteins for proper motor control, and may explain the increased protein levels we detected in the cerebellum. In contrast, 14-3-3 gamma protein is strongly increased in cerebrospinal fluids of patients with Creutzfeldt-Jacob disease, but 14-3-3 gamma deficient mouse strains show no altered pathogenesis of prion diseases or any other obvious phenotype (Kretzschmar et al, 1996;Wiltfang et al, 1999;Steinacker et al, 2005).…”

Risikofaktoren der sporadischen Creutzfeldt-Jakob-Krankheit

“…Die Patienten versterben häufig in Folge der Bettlägerigkeit an schwerwiegenden Infektionen, insbesondere an Bronchopneumonien (Brown P et al 1994, Poser und Zerr 2002. Kretzschmar et al 1996, WHO 1998und Zerr et al 2000a Sicher: Neuropathologisch bestätigt…”

A Unique Case of Sporadic Creutzfeldt-Jacob Disease Presenting as Progressive Supranuclear Palsy.

“…The diagnosis of CJDis usually confirmed by the presence of spongiform degeneration with deposition of prion protein (PrP), particularly in the cerebral cortex (1). Approximately, 90% of cases show common features such as subacute dementia, myoclonus, periodic synchronous discharge (PSD) on electroencephalogram (2), and neuronal protein (14-3-3, yenolase) in cerebrospinal fluid (CSF)(3), and usually die within a year of onset (2).…”