Nota Clínica

Two new cases of sporadic Creutzfeldt-Jakob disease in Madrid, Spain

M.E. Toribio-Díaz, E. García-Di Ruggiero, F. Pérez-Parra [REV NEUROL 2011;53:281-286] PMID: 21796606 DOI: https://doi.org/10.33588/rn.5305.2011228

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Volumen 53 | Number 05 | Nº of views of the article 8.888 | Nº of PDF downloads 899 | Article publication date 01/09/2011

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ABSTRACT

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. Keywords14-3-3 protein Creutzfeldt-Jakob disease Heidenhain variant Periodic complexes Prionopathy CategoriesInfecciones

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Nota Clínica

Two new cases of sporadic Creutzfeldt-Jakob disease in Madrid, Spain