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Nueva variante de la enfermedad de Creutzfeldt-Jakob: características neurológicas y pruebas de diagnóstico
M Zeidler1, G E Stewart, C R Barraclough
1National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh.
Lancet (London, England)
|October 7, 1997
Resumen
Este estudio detalla la presentación clínica de 14 casos de la nueva variante de la enfermedad de Creutzfeldt-Jakob (nvCJD), distinta de la clásica ECJ. Los primeros síntomas psiquiátricos y sensoriales, seguidos por el deterioro neurológico, caracterizan este nuevo fenotipo de ECJ.
Área de la Ciencia:
- Neurología Neurología.
- Patología Patología Patología.
- Enfermedades infecciosas Enfermedades infecciosas.
Sus antecedentes:
- Informes sobre un nuevo fenotipo clínico patológico de la enfermedad de Creutzfeldt-Jakob (ECJ), denominado nueva variante de ECJ (nvECJ).
- Sugiere un posible vínculo causal entre la ECJV y la encefalopatía espongiforme bovina (ESB).
- Documenta los primeros 14 casos de nvCJD en el Reino Unido.
Objetivo del estudio:
- Describir las características clínicas y los hallazgos diagnósticos de los casos iniciales de nvCJD en el Reino Unido.
- Para diferenciar nvCJD de otras formas de CJD.
Principales métodos:
- Determinación del caso a través de referencias de neurólogos y neuropatólogos.
- Recopilación de datos a través de entrevistas con pacientes y el examen de las notas de caso.
- Confirmación histológica del diagnóstico de nvCJD y análisis genético de la proteína priónica (PrP).
Principales resultados:
- Los 14 pacientes con ECJ nv (8 mujeres) tenían una edad media de 29 años al inicio y una duración mediana de la enfermedad de 14 meses.
- Todos los pacientes presentaron síntomas psiquiátricos tempranos (por ejemplo, depresión) y neurológicos (por ejemplo, ataxia, movimientos involuntarios).
- Las anomalías del electroencefalograma (EEG) eran comunes, pero los típicos complejos periódicos de ECJ estaban ausentes; la RM mostró una señal alta del tálamo en dos casos.
Conclusiones:
- La presentación clínica de estos casos de nvCJD es distinta, lo que sugiere un nuevo fenotipo posiblemente causado por un solo agente infeccioso.
- Si bien existe una superposición con la ECJ esporádica atípica, la confirmación neuropatológica sigue siendo esencial para el diagnóstico de la ECJ neuropática.
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