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La enfermedad de Creutzfeldt-Jakob (ECJ) es un raro trastorno neurodegenerativo que se presenta con un rápido y progresivo deterioro de la memoria, cambios de comportamiento, falta de coordinación y/o alteraciones visuales. Esta forma parte de un grupo de enfermedades conocidas como encefalopatías espongiformes transmisibles (EET) o enfermedades priónicas, que son causadas por la acumulación de la proteína priónica patológica (PrP) en el sistema nervioso central, y que se puede producir de forma esporádica, hereditaria o adquirida. Los síntomas suelen comenzar alrededor de 60-70 años y a medida que avanza la enfermedad el daño neuronal progresivo debido a los depósitos de PrP conduce a un deterioro mental más pronunciado, a la aparición de trastornos del movimiento, ceguera y coma, alcanzando una tasa de mortalidad alta (90%) dentro del primer año.
Se presenta un caso de una mujer de 65 años de edad, que acude a urgencias por alteración del lenguaje progresivo y postura anormal en su brazo derecho, que había comenzado un mes antes. Después de detallados exámenes clínicos y neuropsicológicos se sospechó de la ECJ y se completó el estudio con exámenes complementarios tales como EEG, punción lumbar y resonancia magnética del cerebro. La paciente presentó deterioro cognitivo rápido y global, lo que le lleva a la muerte 2 meses después del ingreso. El estudio anatomopatológico posterior confirmó el diagnóstico de ECJ.
Es importante conocer la enfermedad para poder proporcionar una atención de enfermería adecuada. La rápida progresión de esta enfermedad y el nivel de dependencia hace necesario reevaluar continuamente este tipo de pacientes.
Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disorder presenting with rapidly progressive memory impairment, behavioral changes, lack of coordination and/or visual disturbances. This condition is part of a group of diseases known as spongiform encephalopathies or prionic diseases, which are caused by accumulation of a pathological prion protein (PrP) in the central nervous system, and may occur in sporadic, hereditary or acquired form. Symptoms usually begin around 60-70 years old and, as the disease advances, progressive neuronal damage due to PrP deposits leads to a more pronounced mental deterioration, appearance of movement disorders, blindness and coma, reaching a high mortality rate (90%) within first year.
We present a case of a 65 year-old woman who came to the emergency room complaining of progressive language disturbance and abnormal posture of her right arm that had begun one month earlier. After detailed clinical and neuropsychological examinations CJD was suspected and complementary examinations such as EEG, brain MRI and lumbar puncture were performed. The patient showed rapid and global cognitive deterioration, leading to her death two months after admission. A subsequent pathological study confirmed the diagnosis of CJD.
It is important to know the disease in order to provide adequate nursing care. The rapid progression of the disease and the level of dependence make it necessary to continually reappraise these patients.
Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disorder presenting with rapidly progressive memory impairment, behavioral changes, lack of coordination and/or visual disturbances. This condition is part of a group of diseases known as spongiform encephalopathies or prionic diseases, which are caused by accumulation of a pathological prion protein (PrP) in the central nervous system, and may occur in sporadic, hereditary or acquired form. Symptoms usually begin around 60-70 years old and, as the disease advances, progressive neuronal damage due to PrP deposits leads to a more pronounced mental deterioration, appearance of movement disorders, blindness and coma, reaching a high mortality rate (90%) within first year.
We present a case of a 65 year-old woman who came to the emergency room complaining of progressive language disturbance and abnormal posture of her right arm that had begun one month earlier. After detailed clinical and neuropsychological examinations CJD was suspected and complementary examinations such as EEG, brain MRI and lumbar puncture were performed. The patient showed rapid and global cognitive deterioration, leading to her death two months after admission. A subsequent pathological study confirmed the diagnosis of CJD.
It is important to know the disease in order to provide adequate nursing care. The rapid progression of the disease and the level of dependence make it necessary to continually reappraise these patients.
