Resumen

LEWIN, Sara et al. Enfermedades priónicas en el ser humano en Uruguay: registro de los últimos 25 años. Rev. Méd. Urug. [online]. 2012, vol.28, n.1, pp.35-42. ISSN 1688-0390.

Summary Introduction: prion diseases are progressive neurodegenerative disorders that may result in death and are distinguished by long incubation periods before presenting a clinical manifestation. Today they are scientifically interesting since they follow a protein auto-replicant model without the participation of nucleic acids. These diseases appear sporadically, they are hereditary and infectious as well. The appearance of bovine spongiform encephalopathy and its consequences on human beings and Creutzfeldt-Jacob disease variations reinforce the need for a strict epidemiological control in the field. Objective: to describe the reality of these diseases in Uruguay from 1984 through 2009. Method: we conducted a retrospective, descriptive review of clinical cases of prion diseases diagnosed in our country. Results: we managed to identify 43 cases of Creutzfeldt-Jacob disease in Uruguay (8 hereditary forms and 34 sporadic forms). Global incidence estimated rate was 0.7 cases per million inhabitants, considering both probable and definitive forms. Conclusions: we have exclusively identified sporadic and hereditary cases of Creutzfeldt-Jacob disease, we have not identified variations in connection with bovine spongiform encephalopathy or other kinds of prion diseases affecting human beings. Given the characteristics of the disease and the distribution of neurologists in the country enable a rather strict survey and epidemiological surveillance of these diseases in our country.

Palabras clave : PRION DISEASES; CREUTZFELDT-JAKOB SYNDROME; URUGUAY.

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