Enfermedad de Creutzfeld Jakob, desafío diagnóstico
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and fatal neurodegenerative disease caused by prions. It is the most common of spongiform encephalopathies. The most accepted hypothesis suggests that it initiates and propagates through conversion of normal prion protein (PrP) in an abnormal...
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| Publicado en: | Revista Médica Universitaria |
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| Autores principales: | , , , , , , , , |
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| Acceso en línea: | https://bdigital.uncu.edu.ar/fichas.php?idobjeto=7142 |
| Sumario: | Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and fatal neurodegenerative disease caused by prions. It is the most common of spongiform encephalopathies. The most accepted hypothesis suggests that it initiates and propagates through conversion of normal prion protein (PrP) in an abnormal conformational isoform (PrPres), which accumulates in the brain causing neuronal destruction. The cases of two patients assisted in the Department of Neurology of Hospital Lagomaggiore city of Mendoza in 2-year period are reported: A 49-year old female patient with symptoms of rapidly progressing cognitive impairment associated with gait disturbance and ataxia of 2 months of progression; and a 54-year old male with misconduct and temporo-spatial disorientation of 20 days of evolution. In both cases, EEG was compatible with CJD and 14.3.3 protein was positive, dying weeks after diagnosis. Both cases are discussed, which represented a diagnostic challenge given the low frequency and little recognition of this entity in our environment, must be considered in patients who develop a rapidly progressive dementia.
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