Two Forms of Presentation of Confirmed Sporadic Creutzfeldt–Jakob Disease

A López Serrano, S Bañón Escandell, MD Castaño Pérez, P Safont Gasó, P Esteve Atienzar, J Peris García and JM Seguí Ripoll

Department of Internal Medicine, Hospital de San Juan, Spain
Department of Clinical Medicine, Miguel Hernández University (UMH) of Elche, Spain
Department of Neurology, Hospital de San Juan, Spain
Department of Clinical Medicine, Miguel Hernández University (UMH) of Elche, Spain
Department of Infectious Diseases, Hospital Ramón y Cajal, Spain

^*Correspondance to: Alberto López-Serrano 

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Abstract:

Prion diseases are a rare type of neurodegenerative disease, and the sporadic Creutzfeldt-Jakob subtype is the most common. The disease occurs worldwide with an incidence of 1 case per million populations per year. Prion protein (PrP) gene polymorphism without genetic mutation results in an aberrant protein isoform being deposited intraneuronally, leading to spongiform neuronal degeneration. We report on two patients who were admitted to our Service with systemic and neurological disease, with fatal end after 14 and 9 months of follow-up, respectively. Diagnosis was confirmed by histologic examination of the brain.

Keywords:

Dementia; Sporadic Creutzfeldt-Jakob disease; Prion diseases; Protein 14-3-3 protein; Constitutional syndrome

Cite the Article:

López Serrano A, Bañón Escandell S, Castaño Pérez MD, Safont Gasó P, Esteve Atienzar P, Peris García J. Two Forms of Presentation of Confirmed Sporadic Creutzfeldt–Jakob Disease. Ann Clin Case Rep. 2016; 1: 1208.