PROBABLE CREUTZFELDT JAKOB DISEASE: CASE REPORT
Yanes Brum Bello, Alexandre Cossenza Pettezzoni De Almeida, Daniela Mendonca Sueth, Eduardo Carvalho Belizario, Fabiola Sampaio Brandao, Marco Araujo Leite, Marco Orsini and Antonio Marcos Da Silva Catharino
DOI : 10.3844/amjsp.2014.13.15
Current Research in Medicine
Volume 5, 2014
Prion diseases are associated with the accumulation of an abnormal isoformof cellular prion protein (PrPSc), which is the principal constituent of prions. Prions replicate in lymphoreticular tissues before neuroinvasion, suggesting that lymphoreticular biopsy samples may allow early diagnosis by detection of PrPSc. Creutzfeldt-Jakob Disease (CJD) is the most frequent prion disease in humans. Clinical diagnosis of sporadic (CJD) is based on the evaluation of rapidly progressive dementia, ataxia, myoclonus, changes on the electroencephalogram and other neurological signs. We report a problable (CJD) case in Santa Barbara surgical center, Rio de Janeiro, Brazil. From our clinical case, we decided to do a brief review about (CJD) researching at MedLine and Pubmed, using terms Creutzfeldt-Jakob disease and Prion diseases. A definite diagnosis, however, is confined to cases that have been evaluated neuropathlogically or by equivalent diagnostic techniques. The range in clinical expression of the disease is better appreciated and the existence of "atypical" cases of CJD is increasingly recognized. The clinical characteristics, laboratory findings, differential diagnosis, mechanisms of transmission and the actual therapeutic approach are discussed.
© 2014 Yanes Brum Bello, Alexandre Cossenza Pettezzoni De Almeida, Daniela Mendonca Sueth, Eduardo Carvalho Belizario, Fabiola Sampaio Brandao, Marco Araujo Leite, Marco Orsini and Antonio Marcos Da Silva Catharino. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.