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1.
Rev. neurol. (Ed. impr.) ; 53(5): 281-286, 1 sept., 2011. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-91838

RESUMO

Introducción. La enfermedad de Creutzfeldt-Jakob es una encefalopatía espongiforme transmisible, en la que típicamente se produce el acúmulo de una isoforma alterada de la proteína priónica en el cerebro. La forma esporádica se caracteriza por presentar una demencia rápidamente progresiva, con síntomas y signos de afectación piramidal, extrapiramidal, cerebelosa, cortical y presencia de mioclonías. El diagnóstico se basa en los hallazgos clínicos en combinación con los resultados típicos del electroencefalograma, estudio de líquido cefalorraquídeo o la neuroimagen. Casos clínicos. Presentamos dos casos detectados entre los años 2008 y 2010 en el Hospital del Henares, en Coslada. Caso 1: mujer con pérdida de fuerza progresiva en las extremidades, cambio en su comportamiento con labilidad emotiva y tendencia al aislamiento. Valorada inicialmente como una paciente psiquiátrica, en los meses posteriores presentó una situación de inmovilidad absoluta con tendencia al mutismo. El fallecimiento se produjo a los seis meses del inicio de los síntomas. Caso 2: varón con cuadro progresivo de alteración de la visión en forma de hemianopsia homónima derecha, dificultad para la emisión del lenguaje, mareo e inestabilidad. Progresivamente manifestó un síndrome cerebeloso con mioclonías generalizadas hasta su fallecimiento, 10 semanas después del inicio de los síntomas. En ambos casos el electroencefalograma mostró un patrón típico y la proteína 14-3-3 fue positiva en el estudio de líquido cefalorraquídeo. Conclusión. Nos ha llamado la atención una incidencia tan elevada en tan corto período y en un área sanitaria de apenas 170.000 habitantes (AU)


Introduction. Creutzfeldt-Jakob disease is a transmissible spongiform encephalopathy, in which there is typically an accumulation of an altered isoform of the prion protein in the brain. The sporadic form is characterised by presenting a rapidly progressive dementia, with signs and symptoms of pyramidal, extrapyramidal, cerebellar and cortical involvement, as well as the presence of myoclonias. Diagnosis is based on the clinical findings in combination with the typical results of an electroencephalogram, cerebrospinal fluid study or neuroimaging. Case reports. We report two cases detected between the years 2008 and 2010 in the Hospital del Henares, in Coslada, Madrid. Case 1: a female with progressive loss of strength in her limbs, change in her behaviour with mood swings and a tendency towards seeking to isolate herself. Although initially considered a psychiatric patient, in the months that followed she became absolutely immobile with a tendency towards mutism. Death occurred six months after the onset of symptoms. Case 2: a male with a progressive picture of altered vision in the form of right homonymous hemianopia, difficulty in verbally expressing language, dizziness and instability. The patient gradually developed a cerebellar syndrome with generalised myoclonias until his death, 10 weeks after the onset of symptoms. In both cases, the electroencephalogram showed a typical pattern and 14-3-3 protein was positive in the cerebrospinal fluid study. Conclusions. Such a high incidence over such a short period of time and in a health district of barely 170,000 inhabitants seems rather surprising (AU)


Assuntos
Humanos , Masculino , Feminino , Síndrome de Creutzfeldt-Jakob/diagnóstico , Eletroencefalografia , Doenças Priônicas/diagnóstico , Proteínas 14-3-3/líquido cefalorraquidiano
2.
Rev Neurol ; 53(5): 281-6, 2011 Sep 01.
Artigo em Espanhol | MEDLINE | ID: mdl-21796606

RESUMO

INTRODUCTION: Creutzfeldt-Jakob disease is a transmissible spongiform encephalopathy, in which there is typically an accumulation of an altered isoform of the prion protein in the brain. The sporadic form is characterised by presenting a rapidly progressive dementia, with signs and symptoms of pyramidal, extrapyramidal, cerebellar and cortical involvement, as well as the presence of myoclonias. Diagnosis is based on the clinical findings in combination with the typical results of an electroencephalogram, cerebrospinal fluid study or neuroimaging. CASE REPORTS: We report two cases detected between the years 2008 and 2010 in the Hospital del Henares, in Coslada, Madrid. Case 1: a female with progressive loss of strength in her limbs, change in her behaviour with mood swings and a tendency towards seeking to isolate herself. Although initially considered a psychiatric patient, in the months that followed she became absolutely immobile with a tendency towards mutism. Death occurred six months after the onset of symptoms. Case 2: a male with a progressive picture of altered vision in the form of right homonymous hemianopia, difficulty in verbally expressing language, dizziness and instability. The patient gradually developed a cerebellar syndrome with generalised myoclonias until his death, 10 weeks after the onset of symptoms. In both cases, the electroencephalogram showed a typical pattern and 14-3-3 protein was positive in the cerebrospinal fluid study. CONCLUSIONS: Such a high incidence over such a short period of time and in a health district of barely 170,000 inhabitants seems rather surprising.


Assuntos
Síndrome de Creutzfeldt-Jakob/diagnóstico , Proteínas 14-3-3/líquido cefalorraquidiano , Idoso , Idoso de 80 Anos ou mais , Síndrome de Creutzfeldt-Jakob/epidemiologia , Síndrome de Creutzfeldt-Jakob/patologia , Síndrome de Creutzfeldt-Jakob/fisiopatologia , Eletroencefalografia , Evolução Fatal , Feminino , Humanos , Masculino , Espanha/epidemiologia
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