Sporadic Creutzfeldt-Jakob disease with glial PrP nuclear and perinuclear immunoreactivity.

Proteinase K-resistant prion protein (PrP ) nuclear and perinuclear immunoreactivity in oligodendrocytes of the frontal cortex is found in one case of otherwise typical sporadic Creutzfeldt-Jakob disease (sCJD) type VV2a. The PrP nature of the inclusions is validated with several anti-PrP antibodies directed to amino acids 130-160 (12F10), 109-112 (3F4), 97-102 (8G8) and the octarepeat region (amino acids 59-89: SAF32). Cellular identification and subcellular localization were evaluated with double- and triple-labeling immunofluorescence and confocal microscopy using antibodies against PrP, glial markers, and histone H3.

Geographical analysis of the sporadic Creutzfeldt-Jakob disease distribution in the autonomous community of the Basque Country for the period 1995-2008.

Background: The Basque Country, in Spain, shows one of the highest sporadic Creutzfeldt-Jakob disease (sCJD) incidence rates in Europe. The purpose is to analyse a possible focus of unidentified external or environmental factors which could trigger the high incidence rates of sCJD in the Basque Country.

Methods: We estimated the relative risk and the posterior relative risk distribution of sCJD cases for each town of the Basque Country and for the period 1995-2008.

[Where does Parkinson's disease start?].

Parkinson's disease concept is classically restricted to a rigid-akinetic syndrome due to a nigro-striatal dopaminergic depletion. This concept ignored the widespread extension of neuropathologic findings across the CNS. Moreover detailed clinical studies showed that patients suffer a constellation of other symptoms and signs such as anosmia, constipation, cardiovascular disautonomia, neuropsychologic and neuropsychiatric disorders, dementia and sleep changes.

Risk factors for dementia in the epidemiological study of Munguialde County (Basque Country-Spain).

Background: Prevalence of degenerative dementias and dementias associated with cerebrovascular disease is increasing. Dementia is one of the most significant public health problem. In recent years, the role of vascular risk factors (hypertension, diabetes mellitus and hypercholesterolemia) and depression has been evaluated.

Prevalence of neuropsychiatric symptoms in elderly patients with dementia in Mungialde County (Basque Country, Spain).

Background: Determination of the prevalence of neuropsychiatric symptoms in elderly patients with dementia in Mungialde.

Methods: 108 subjects with dementia, who participated in a door-to-door epidemiological study, were included. The 12-item Spanish version of the Neuropsychiatric Inventory was used to evaluate these symptoms.

Polymorphism in the cholesterol 24S-hydroxylase gene (CYP46A1) associated with the APOEpsilon3 allele increases the risk of Alzheimer's disease and of mild cognitive impairment progressing to Alzheimer's disease.

Background: Late-onset Alzheimer's disease (LOAD) is associated with changes in certain proteins, such as ApoE and Cyp46A1, of the elimination route for cerebral cholesterol. The main lipoprotein involved in its transport is ApoE whose Epsilon4 allele is the least efficient. However, the presence or absence of this allele does not determine the development of LOAD, which implies the existence of other susceptibility factors associated with the disease, such as the CYP46A1 gene that encodes the enzyme cholesterol 24S-hydroxylase.

Human T-cell leukemia virus type I infection in various recipients of transplants from the same donor.

Background: The human T-cell lymphotrophic virus (HTLV) causes adult T-cell leukemia-lymphoma, tropical spastic paraparesis-HTLV type I, and associated myelopathy.

Methods: An analysis was performed of serum samples from a multiorgan donor and the five recipients. Also studied was the donor's family and the partner of one of the renal recipients.