[Creutzfeldt-Jakob disease: two new cases in Talavera de la Reina]

Creutzfeldt-Jakob disease is a prion spongiform encephalopathy that causes a rapidly progressive dementia whose neuropathological features are neuronal loss, proliferation of glial cells, and the presence of small vacuoles, which produces a spongiform appearance. Two cases detected in the year 2005 in the <> Hospital are presented. The first one is the case of a man who presented with intense malaise and weight loss, developing dementia in 4 months. The second one was a woman who presented with diplopia, headache and gait disturbances who developed severe neurological impairment with dementia in three months. In both cases the 14.3.3 protein in the cerebrospinal fluid was positive and the EEG showed a characteristic pattern. The genetic studies were negative and both were homozygous for methionine at codon 129. Given that the incidence of this disease in Castilla la Mancha is 0.6 cases/100000 inhabitants/year, the detection of 2 cases in less than a year in Talavera de la Reina (whose population is 150,000) surprised us, leading us to intensify surveillance.