Creutzfeldt-Jakob Disease: an under-Recognized Cause of Dementia

Department of Gerontology, Geriatrics and Physiatry, Catholic University of the Sacred Heart, Rome, Italy, Department of Pathology, National Institute for Infectious Diseases, Istituto di Ricovero e Cura A Carattere Scientifico Lazzaro Spallanzani, Rome, Italy, Department of Neurological Science, University of Bologna, Bologna, Italy, Institut du Vieillissement, Université de Toulouse, Toulouse, France
Journal of the American Geriatrics Society (Impact Factor: 4.57). 01/2012; 60(1):156-7. DOI: 10.1111/j.1532-5415.2011.03756.x
Source: PubMed

ABSTRACT Export Date: 23 March 2012, Source: Scopus, CODEN: JAGSA, doi: 10.1111/j.1532-5415.2011.03756.x, PubMed ID: 22239296, Language of Original Document: English, Correspondence Address: Cerullo, F.; Department of Gerontology, Geriatrics and Physiatry, Catholic University of the Sacred Heart, Rome, Italy, Chemicals/CAS: amyloid beta protein, 109770-29-8, References: Glatzel, M., Stoeck, K., Seeger, H., Human prion diseases: Molecular and clinical aspects (2005) Arch Neurol, 62, pp. 545-552;

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    ABSTRACT: We report here an autopsy case of a 64-year-old female with slowly progressive dementia and parkinsonism in a 4-year-long clinical course. Post-mortem examination revealed a severely atrophic brain with spongiform degeneration, neuronal loss and gliosis in the gray matter. Many prion protein plaque deposits were present in the occipital lobe, amygdala and cerebellum. Additionally, Lewy bodies were observed in the brainstem. Prion protein gene analysis of the patient revealed polymorphism at the codon-129 valine heterozygote. This genotype is known to sometimes accompany a missense mutation of the gene in uncommon hereditary prion diseases, but no mutation was found in the open reading frame. Thus, it might be suggested that this case showed simultaneously the features of both sporadic Creutzfeldt-Jakob disease (CJD) with codon-129 valine and Parkinson's disease. However, the predisposing factors for contracting both diseases simultaneously remain to be determined, because the incidence of Parkinson's disease accompanied by CJD is very low.
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