Introduction Creutzfeldt-Jakob disease (CJD) is an infectious disease attributed to a proteinaceous infectious particle (prion), which attacks the central nervous system. The form of acquisition is still unknown and may occur by four known mechanisms of transmission: sporadic, hereditary, iatrogenic and variant (vCJD), which occurs by eating contaminated beef carrier of mad cow disease.
Methods Reporting of cases of CJD has occurred in Bahia between August 2007 and December 2010.
Results There have been 14 suspected cases of sporadic CJD, eight confirmed, two probable (WHO criteria), one discarded and three under investigation. Of these, six were male and white. The median age was 68 years old, ranging from 40 to 77. Four had complete higher education, four high school and two elemetary education. Ten died. None of the suspected cases underwent iatrogenic exposure and had no family history of disease. All had cerebellar ataxia, 80% had progressive dementia and extra-pyramidal signs and 60% had psychiatric disorders, pyramidal signs, myoclonus and sleep disturbances. Of the specific tests performed five had typical result by electroencephalogram; six with typical diffusion by MRI/CT; five presented Protein 14-3-3; none of them presented mutation of prion; four patients had spongiform encephalopathy brain by biopsy; four positive by immunohistochemistry for PRPNPc. Only three patients underwent necropsy had histological positive result.
Conclusion Since this is a new and yet unknown disease in our country, needs to improve the clinical diagnosis as a way to better understand the natural history of it and thereby promote effective measure control.
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