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Copyright of Acta Neurológica Colombiana is the property of Colombian Association of Neurology / Asociacion Colombiana de Neurologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Araucaria is the property of Araucaria-Revista Iberoamericana de Filosofia, Politica y Humanidades and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The Creutzfeldt-Jakob disease is a neurodegenerative, neuroselective and fatal entity, that is not usually reported in Mexico. We present a 40-year-old male patient who presents the onset of this illness, with short-term memory disorder, depressive episodes and emotional lability with a tendency to irritability. He also presents space disorientation, decreased strength of the left and lateral hemibody drive in the ipsilateral walk, and insomnia, for which he is admitted to the hospital during 40 days for diagnostic approach. Several studies were carried out during his hospital stay, the most relevant for the diagnosis: a magnetic resonance which presented "cortical ribboning" and hyperintensities in the nuclei of the base, both diagnosis highly suggested the pathology. The positive results to protein 14-3-3 reaffirmed the diagnosis. After 15 months of the onset of neurological symptoms, the patient presented symptoms of pneumonia, which lead to the hospitalization. During his stay, he presented a pulmonary abscess and rapid progressive dementia. The patient died in the hospital by a pulmonary sepsis 18 months after the onset of symptoms. No necropsy was performed, following the current standards for the disease management. [ABSTRACT FROM AUTHOR]

Copyright of Galicia Clínica is the property of Sociedad Gallega de Medicina Interna and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Prionpathies represent a group of neurodegenerative diseases of familiar, sporadic or acquired origin, heterogeneous symptoms and fatal outcome. The psychiatric symptoms can be present at the beginning and/or during the clinical evolution, with the presence or not of neurological disorders. Patients with these sympthoms are referred to psychiatric units, both at hospital level and as outpatients, to study a psychiatric disorder. In the described clinical case, the patieht was admitted several times at emergency units, rated by neurologists and psychiatrists leading to different psychiatric disorders diagnoses. However spongiform encephalopathy was diagnosed only during the last month of the disease. [ABSTRACT FROM AUTHOR]

Creutzfeldt-Jakob disease (CJD) is a fatal neurological disease caused by pathological isoform of the human prion protein. Clinical features of six cases of the sporadic form of CJD with definitive diagnosis by histopathology, and five cases with probable diagnosis were reported in patients treated at the Peruvian National Institute of Neurological Sciences. The average age of onset in definite cases was 55.8 years and in probable cases was 59.6, mostly males. The average disease duration was 8.8 months. Atypical EEG was found in 50% of definite cases and in 80% of probable. The 14-3-3 protein in cerebrospinal fluid was positive in a probable case, and typical MRI findings were observed in two probable cases. All cases studied had a typical clinical course of the disease, and it is considered as the first report of CJD in Peru. [ABSTRACT FROM AUTHOR]

Update the knowledge about particles called prions, handling precautions and recommendations of tissue in patients with suspected infection, in addition to a systematization of the item has slopes of great medical interest. We reviewed studies of the last 10 years systematically with keywords that included, prion, orCreutzfeldtdakob spongiform encephalopathy, dementia and brain biopsy progression fast. In Mesh Ime&line), human papers were included in English and Spanish domains. We perlormed a screening of the work that made mention of experience (more than onol and that related to the handling precautions and tissues of patients with spongiform encephalopathy. Consistent with the literature, patients with probable prion disease should be treated with great caution, first to see if there is possibility brain biopsy to confirm the diagnosis, and after his death according to European recommendations mainly in the preparation of cremation. The sporadic appearance of disease is spontaneous, the prion protein remains a major source of molecular research has generated two novel awards. Not everything has been linked to disease just as there are approaches that knowledge of the foundations of learning is linked to prions according to faculty experts like Professor Eric R. Kandel. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disease caused by prions that affect the central nervous system. It's invariably fatal. It can be classified as sporadic, familial and iatrogenic. It's clinical features are subacute dementia, associated with motor and visual symptoms, as well as myoclonus. Electroencephalogram (EEG) shows pseudoperiodic spicular activity. Magnetic Resonance Imaging (MRI) presents with hyperintense signals in both striated corpus and some cerebral cortex areas. Cerebrospinal fluid (CSF) shows an increased level of 14-3-3 protein. Finally, histopathological study of brain states spongiform encephalopathy. CASE REPORT: We report two cases of CJD. Two women who started at ages 44 and 67 years old with abnormal gait, cognitive impairment and myoclonus, with rapid progression of symptoms to absolute dependence. EEG was compatible with the disease and imaging tests showed hyperintense signals in striatum and / or some areas of the cortex. Both patients coursed with infections during their hospitalization period and both underwent gastrostomy due to the presence of a severe swallowing disorder. DISCUSSION: The diagnosis of CJD is suspected based on the clinical findings and it is supported by characteristic features on MRI, EEG and CSF analysis. If the familial form of CJD is suspected, a genetic study issuggested however, in Herminda Martin Clinical Hospital (HCHM) this is not available. CJD is an invariably fatal disease and it doesn't have any treatment. This makes difficult to choose between executing tests and interventions, and having an expectant management. [ABSTRACT FROM AUTHOR]

Prion diseases are a group of fatal neurodegenerative disorders that occur in inherited, acquired and sporadic forms, a characteristic that makes them unique. Herein, we review the literature to update the current knowledge of these diseases, with particular emphasis on the inherited forms. The PRNP gene is located on chromosome 20 and contains two exons and one intron. It encodes the prion protein (PrPc) that comprises 253 aminoacids and exhibits a polymorphism on the codon 129 (valine or metionine) that can modify the disease phenotype. The hallmark of prion diseases is the misfolding of the prion protein observed in the brain of affected individuals (prion-only hypothesis). Creutzfeldt- Jackob disease (CJD) can be sporadic or iatrogenic and there is a new variant. Dominantly inherited forms include familial CJD, Gerstmann-Straussler-Scheinker disease, and fatal familial insomnia that arise from germline mutations in the PRNP gene. There are three types of pathogenic PRNP mutations: point mutations leading to an aminoacid substitution or premature stop codon, and insertion of additional octapeptide repeats. Predictive diagnosis is possible in all inherited prion diseases, resulting in ethical issues. Even though prion diseases have been known for several years they are an enigma in biology, thus a better understanding of the underlying mechanisms that generate them is needed. [ABSTRACT FROM AUTHOR]

Prions are the etiologic agents of spongiform encephalopaties in mammals and humans. Protein aggregations present in these pathological entities are the same that other neurodegenerative diseases like Alzheimer's disease. However recent studies suggest that under specific conditions prions could participate in many biological processes. In fungi prions act as genetic elements for phenotypic inheritance under adverse environment; an Aplysia model shows that prion-like proteins in other organisms like CPEB could have functions in memory formation. Can prion participate in biological processes really? Here, we review some details of prion disease, prion as genetic elements and the relevance of their ability for save information on memory formation. [ABSTRACT FROM AUTHOR]

Introduction: At present time, it is known that prion diseases or transmissible spongiform encephalopaties (TSE), represent a group of neurodegenerative pathologies of the central nervous system, that affect humans as well as animals. The least rare of these is Creutzfeldt-Jakob disease (CJD). In 1998 the first case of Gerstman-Sträussler-Scheinker (GSS) of familiar type was reported (a case by million families). The bovine spongiform encephalopaty (BSE) - better known as "crazy cows disease" - is attributed to the consumption of contaminated products with affected cows meat. The prion disease can appear like a genetic disorder, infectious or sporadic, which involve modifications of the prion protein (PrP), a normal component of the mammal cells. Recently it has been suggested that cellular prion protein (PrPc) acts like a metaloprotein, and has an intrinsic tendency to adopt some structural characteristics of the Scrapie prion protein (PrPsc). [ABSTRACT FROM AUTHOR]

The transmissible spongiform encephalopathies (TSE) diseases, sometimes referred to as prion diseases, are a group of rare, incurable, transmissible neurodegenerative, and fatal diseases, affecting a variety of mammals. Over the last 20 years, they have gone from a fascinating but relatively difficult to understand group of diseases to one that is a major economic problem in agricultural, cattle farming and as well as a threat to human health, to due to prions are infectious for cattle, wildlife animals or both and human beings. TSE include kuru and Creutzfeldt-Jakob disease (CJD) in humans, scrapie in sheep, transmissible mink encephalopathy (TME), and chronic wasting disease (CWD) in mule deer and elk, while in cattle the head-line grabbing ‘mad cow’ disease, bovine spongiform encephalopathy (BSE), immediately comes to mind. CWD can be transmitted to susceptible animals indirectly, from environments contaminated by excreta or decomposed carcasses. Last five years, the known distribution of CWD across North America has expanded; increasing concerns over the impact of this disease on populations of free-ranging cervids and the viability of game farming industries. In Mexico, there is no evidence of CWD of any specie of wildlife or captivity deer. This TSE is considered exotic and it should take pertinent measures to avoid its entrance to Mexico. [ABSTRACT FROM AUTHOR]

Copyright of Galicia Clínica is the property of Sociedad Gallega de Medicina Interna and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)