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SUMMARY The pandemic caused by the COVID-19 has influenced peoples' lifestyles. Home-confinement scenario might impair physical activity practice, resulting in new challenges for maintaining health during the pandemic of the COVID-19. The aim of this study was to present the current context of COVID-19 pandemic, its impact on the practice of physical activity, and the strategies available to remain active during home-confinement according to international recommendations. The narrative review was conducted based on studies that analyzed themes related to physical activity and COVID-19. Virtual Health Library (VHL), CINAHL, Cochrane, PsycINFO, PubMed, ScienceDirect, Scientific Electronic Library Online (SciELO), Scopus, SPORTDiscus, and Web of Science databases were searched for relevant papers. Although an increased number of experimental studies are still necessary, people should devote more time to physical activity during social isolation. Guidelines were adjusted by international entities in order to encourage people to remain active, through practice regular physical activity, using alternative strategies such as fitness program applications, exergames, online exercise classes, and even chores. Reduction of prolonged sedentary behavior could contribute to maintain health and improve quality of life during the COVID-19 pandemic.
La pandemia provocada por la Covid-19 ha influido en los estilos de vida de las personas. El escenario de confinamiento domiciliario podría perjudicar la práctica de actividad física, generando nuevos desafíos para el mantenimiento de la salud durante la pandemia de la Covid-19. El objetivo de este estudio fue presentar el contexto actual de la pandemia de Covid-19, su impacto en la práctica de actividad física y las estrategias disponibles para mantenerse activo durante el confinamiento domiciliario según las recomendaciones internacionales. La revisión narrativa se realizó con base en estudios que analizaron temas relacionados con la actividad física y la Covid-19. Se realizaron búsquedas de artículos relevantes en las bases de datos Virtual Health Library (BVS), CINAHL, Cochrane, PsycINFO, PubMed, ScienceDirect, Scientific Electronic Library Online (SciELO), Scopus, SPORTDiscus y Web of Science. Aunque todavía es necesario un mayor número de estudios experimentales, las personas deberían dedicar más tiempo a la actividad física durante el aislamiento social. Las pautas fueron ajustadas por entidades internacionales para alentar a las personas a mantenerse activas, mediante la práctica de actividad física regular, utilizando estrategias alternativas como aplicaciones de programas de acondicionamiento físico, exergames, clases de ejercicios en línea e incluso tareas domésticas. La reducción del sedentarismo prolongado podría contribuir a mantener la salud y mejorar la calidad de vida durante la pandemia de Covid-19.
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Humanos , Aislamiento Social , Ejercicio Físico , Salud , Infecciones por Coronavirus , RevisiónRESUMEN
Prion diseases or transmissible spongiform encephalopathies (TSEs) are human and animal diseases naturally or experimentally transmissible with a long incubation period and a fatal course without remission. The nature of the transmissible agent remains debated but the absence of a structure evoking a conventional microorganism led Stanley B. Prusiner to hypothesize that it could be an infectious protein (proteinaceous infectious particle or prion). The prion would be the abnormal form of a normal protein, cellular PrP (PrPc) which will change its spatial conformation and be converted into scrapie prion protein (PrPsc) with properties of partial resistance to proteases, aggregation and insolubility in detergents. No inflammatory or immune response are detected in TSEs which are characterized by brain damage combining spongiosis, neuronal loss, astrocytic gliosis, and deposits of PrPsc that may appear as amyloid plaques. Although the link between the accumulation of PrPsc and the appearance of lesions remains debated, the presence of PrPsc is constant during TSE and necessary for a definitive diagnosis. Even if they remain rare diseases (2 cases per million), the identification of kuru, at the end of the 1950s, of iatrogenic cases in the course of the 1970s and of the variant of Creutzfeldt-Jakob disease (CJD) in the mid-1990s explain the interest in these diseases but also the fears they can raise for public health. They remain an exciting research model because they belong both to the group of neurodegenerative diseases with protein accumulation (sporadic CJD), to the group of communicable diseases (iatrogenic CJD, variant of CJD) but also to the group of genetic diseases with a transmission Mendelian dominant (genetic CJD, Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia).
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Síndrome de Creutzfeldt-Jakob , Enfermedad de Gerstmann-Straussler-Scheinker , Kuru , Enfermedades por Prión , Animales , Síndrome de Creutzfeldt-Jakob/diagnóstico , Humanos , Enfermedades por Prión/diagnósticoRESUMEN
Protein misfolding cyclic amplification assay (PMCA) and real-time quaking-induced conversion (RT-QuIC) are two amplification techniques based on the ability of PrPsc to induce a conformational change in PrP allowing the detection of minute amounts of PrPsc in body fluids or tissues. PMCA and RT-QuIC have different ability to amplify PrPsc from sporadic, variant and genetic forms of Creutzfeldt-Jakob disease (CJD). PMCA readily amplifies PrPsc from variant CJD (vCJD) tissue while RT-QuIC easily amplifies PrPsc from sporadic CJD (sCJD) patient tissues. In terms of diagnosis, this implies the possibility of distinguishing vCJD from sCJD and explains the wider use of RT-QuIC given the respective frequencies of vCJD and sCJD. The sensitivity values of RT-QuIC for the diagnosis of sCJD are comparable or higher than those of the other tests (EEG, MRI, detection of 14-3-3 or tau proteins in cerebrospinal fluid) but with a specificity close to 100%. These new diagnostic methods could also be useful for the diagnosis of other neurodegenerative diseases.
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Síndrome de Creutzfeldt-Jakob/diagnóstico , Síndrome de Creutzfeldt-Jakob/microbiología , Enfermedades por Prión/diagnóstico , Enfermedades por Prión/microbiología , Humanos , Técnicas de Diagnóstico Molecular/métodosRESUMEN
Spinal cord stimulation (SCS) is an effective surgical therapy used for the treatment of chronic neuropathic pain. Tonic SCS is safe and improve not only gait disorders, motor symptoms, but also quality of life in Parkinson patients even with dopa-resistant symptoms with or without associated deep brain stimulation.
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Trastornos Neurológicos de la Marcha/terapia , Enfermedad de Parkinson/terapia , Calidad de Vida , Estimulación de la Médula Espinal , Caminata , Anciano , Trastornos Neurológicos de la Marcha/etiología , Humanos , Masculino , Persona de Mediana Edad , Enfermedad de Parkinson/complicaciones , Estudios Prospectivos , Resultado del Tratamiento , Caminata/fisiologíaRESUMEN
The development of neuropsychological assessment methods using virtual reality (VR) is a valid and promising option for the detection of cognitive impairment in the older people, focusing on activities composed of tasks of multiple demands. This study verified the association of age, schooling, and general cognitive status on the performance of neurologically healthy older adults in ECO-VR, a VR task of multiple demands for neuropsychological assessment. A total of 111 older adults answered a sociodemographic questionnaire, the Mini Mental State Examination, the Vocabulary subtest of the Wechsler Intelligence Scale for Adults (third edition), and the ECO-VR. Correlation analyses, multiple linear regression, and comparisons between groups (effects by age and schooling groups) were used to evaluate the results. The ECO-VR total score was significantly associated with age, years of education, MMSE, and Vocabulary subtest. The linear regression models identified that age was the main predictor for total score and rule breaking of ECO-VR. According to the univariate analysis, it was identified the main effect of age group and schooling group in the total ECO-VR score, but there was no interaction effect. The results are discussed in order to understand the role of sociodemographic characteristics in the performance of older adults in a VR task of multiple demands. It was also verified the possibility use of VR for neuropsychological assessment of older adults.
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The accumulation of a specific protein in aggregated form is a common phenomenon in human neurodegenerative diseases. In Parkinson's disease, this protein is α-synuclein which is a neuronal protein of 143 amino acids. With a monomeric conformation in solution, it also has a natural capacity to aggregate into amyloid structures (dimers, oligomers, fibrils and Lewy bodies or neurites). It therefore fulfils the characteristics of a prion protein (different conformations, seeding and spreading). In vitro and in vivo experimental evidence in transgenic and wild animals indicates a prion-like propagation of Parkinson's disease. The sequential and predictive distribution of α-synuclein demonstrated by Braak et al. and its correlation with non-motor signs are consistent with the prion-like progression. Although the triggering factor causing the misfolding and aggregation of the target protein is unknown, Parkinson's disease is a highly relevant model for the study of these mechanisms and also to test specific treatments targeting the assemblies of α-synuclein and propagation from pre-motor phase of the disease. Despite this prion-like progression, there is currently no argument indicating a risk of human transmission of Parkinson's disease.
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Enfermedad de Parkinson/etiología , Enfermedades por Prión , Humanos , Priones/metabolismo , Sinucleínas/metabolismoRESUMEN
France, involved for a long time in the epidemiological surveillance of transmissible spongiform encephalopathy (TSE), created a national network of surveillance in 1991, because of the description of the first cases of Creutzfeldt-Jakob disease (CJD) linked to a treatment by growth hormone of human origin and the observation of cases of cats infected with the agent of the bovine spongiform encephalopathy in the United Kingdom (UK). The French surveillance network is integrated into the European network of surveillance since its creation in 1993. As in other countries, sporadic CJD is the most frequent form of TSE in France with an annual mortality rate of 1.44 per million. Genetic forms are most often associated with a mutation at codon 200. Among the cases of iatrogenic CJD, 13 cases of CJD after duramater grafts were observed and 119 related to treatment with growth hormone. France is the country worst affected in Europe and the world by this latter form, before the USA and UK. Since 1996, 27 cases of variant of CJD (vCJD) has been observed, making France the second country in the world most affected after the UK. No cases of transfusion-associated vCJD have been observed.
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Síndrome de Creutzfeldt-Jakob/epidemiología , Vigilancia de la Población , Animales , Enfermedades Asintomáticas , Portador Sano , Enfermedades de los Gatos/epidemiología , Enfermedades de los Gatos/transmisión , Gatos , Bovinos , Codón/genética , Síndrome de Creutzfeldt-Jakob/genética , Síndrome de Creutzfeldt-Jakob/transmisión , Contaminación de Medicamentos , Duramadre/trasplante , Encefalopatía Espongiforme Bovina/epidemiología , Contaminación de Alimentos , Francia/epidemiología , Salud Global , Hormona de Crecimiento Humana/efectos adversos , Hormona de Crecimiento Humana/aislamiento & purificación , Humanos , Enfermedad Iatrogénica , Carne/efectos adversos , Complicaciones Posoperatorias/epidemiología , Prevalencia , Enfermedades por Prión/transmisión , Enfermedades por Prión/veterinaria , Proteínas Priónicas , Priones/genética , Reacción a la Transfusión , Reino Unido/epidemiología , ZoonosisRESUMEN
In 2009, a pathologist with sporadic Creutzfeldt-Jakob Disease (sCJD) was reported to the Spanish registry. This case prompted a request for information on health-related occupation in sCJD cases from countries participating in the European Creutzfeldt Jakob Disease Surveillance network (EuroCJD). Responses from registries in 21 countries revealed that of 8,321 registered cases, 65 physicians or dentists, two of whom were pathologists, and another 137 healthcare workers had been identified with sCJD. Five countries reported 15 physicians and 68 other health professionals among 2,968 controls or non-cases, suggesting no relative excess of sCJD among healthcare professionals. A literature review revealed: (i) 12 case or small case-series reports of 66 health professionals with sCJD, and (ii) five analytical studies on health-related occupation and sCJD, where statistically significant findings were solely observed for persons working at physicians' offices (odds ratio: 4.6 (95 CI: 1.2-17.6)). We conclude that a wide spectrum of medical specialities and health professions are represented in sCJD cases and that the data analysed do not support any overall increased occupational risk for health professionals. Nevertheless, there may be a specific risk in some professions associated with direct contact with high human-infectivity tissue.
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Síndrome de Creutzfeldt-Jakob/epidemiología , Empleos en Salud , Personal de Salud , Síndrome de Creutzfeldt-Jakob/transmisión , Notificación de Enfermedades/estadística & datos numéricos , Europa (Continente) , Femenino , Humanos , Masculino , Patología , Vigilancia de la Población , Proteínas PrPSc/genética , Sistema de Registros , RiesgoRESUMEN
AIMS: Neuronal death is a major neuropathological hallmark in prion diseases. The association between the accumulation of the disease-related prion protein (PrP(Sc) ) and neuronal loss varies within the wide spectrum of prion diseases and their experimental models. In this study, we investigated the relationships between neuronal loss and PrP(Sc) deposition in the cerebellum from cases of the six subtypes of sporadic Creutzfeldt-Jakob disease (sCJD; n=100) that can be determined according to the M129V polymorphism of the human prion protein gene (PRNP) and PrP(Sc) molecular types. METHODS: The numerical density of neurones was estimated with a computer-assisted image analysis system and the accumulation of PrP(Sc) deposits was scored. RESULTS: The scores of PrP(Sc) immunoreactive deposits of the punctate type (synaptic type) were correlated with neurone counts - the higher the score the higher the neuronal loss - in all sCJD subtypes. Large 5- to 50-µm-wide deposits (focal type) were found in sCJD-MV2 and sCJD-VV2 subtypes, and occasionally in a few cases of the other studied groups. By contrast, the highest scores for 5- to 50-µm-wide deposits observed in sCJD-MV2 subtype were not associated with higher neuronal loss. In addition, these scores were inversely correlated with neuronal counts in the sCJD-VV2 subtype. CONCLUSIONS: These results support a putative pathogenic role for small PrP(Sc) deposits common to the various sCJD subtypes. Furthermore, the observation of a lower loss of neurones associated with PrP(Sc) type-2 large deposits is consistent with a possible 'protective' role of aggregated deposits in both sCJD-MV2 and sCJD-VV2 subtypes.
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Cerebelo/patología , Síndrome de Creutzfeldt-Jakob/patología , Neuronas/patología , Proteínas PrPSc/metabolismo , Recuento de Células , Muerte Celular/fisiología , Cerebelo/metabolismo , Síndrome de Creutzfeldt-Jakob/metabolismo , Humanos , Interpretación de Imagen Asistida por Computador , Immunoblotting , Inmunohistoquímica , Neuronas/metabolismoRESUMEN
BACKGROUND AND PURPOSE: The physiopathologic bases underlying the signal intensity changes and reduced diffusibility observed in prion diseases (TSEs) are still poorly understood. We evaluated the interest of MRS combined with DWI both as a diagnostic tool and a way to understand the mechanism underlying signal intensity and ADC changes in this setting. MATERIALS AND METHODS: We designed a prospective study of multimodal MR imaging in patients with suspected TSEs. Forty-five patients with a suspicion of TSE and 11 age-matched healthy volunteers were included. The MR imaging protocol included T1, FLAIR, and DWI sequences. MRS was performed on the cerebellum, pulvinar, right lenticular nucleus, and frontal cortex. MR images were assessed visually, and ADC values were calculated. RESULTS: Among the 45 suspected cases, 31 fulfilled the criteria for probable or definite TSEs (19 sCJDs, 3 iCJDs, 2 vCJDs, and 7 genetic TSEs); and 14 were classified as AltDs. High signals in the cortex and/or basal ganglia were observed in 26/31 patients with TSEs on FLAIR and 29/31 patients on DWI. In the basal ganglia, high DWI signals corresponded to a decreased ADC. Metabolic alterations, increased mIns, and decreased NAA were observed in all patients with TSEs. ADC values and metabolic changes were not correlated; this finding suggests that neuronal stress (vacuolization), neuronal loss, and astrogliosis do not alone explain the decrease of ADC. CONCLUSIONS: MRS combined with other MR imaging is of interest in the diagnosis of TSE and provides useful information for understanding physiopathologic processes underlying prion diseases.
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Imagen de Difusión por Resonancia Magnética/métodos , Espectroscopía de Resonancia Magnética/métodos , Enfermedades por Prión/metabolismo , Enfermedades por Prión/patología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Encefalopatías/metabolismo , Encefalopatías/patología , Encefalopatías/fisiopatología , Cerebelo/metabolismo , Cerebelo/patología , Cuerpo Estriado/metabolismo , Cuerpo Estriado/patología , Lóbulo Frontal/metabolismo , Lóbulo Frontal/patología , Humanos , Persona de Mediana Edad , Enfermedades por Prión/fisiopatología , Estudios Prospectivos , Pulvinar/metabolismo , Pulvinar/patología , Sensibilidad y EspecificidadRESUMEN
BACKGROUND: Nonconvulsive status epilepticus (NCSE) in patients with confusion may be difficult to distinguish from nonepileptic (metabolic/toxic, postanoxic, and spongiform) encephalopathies. This study aimed to describe the misleading presentation of patients with sporadic Creutzfeldt-Jakob disease (sCJD) who were initially diagnosed with a refractory NCSE (rNCSE). METHODS: We retrospectively reviewed the clinical characteristics, EEG records, brain MRI scans, 14-3-3 protein detection in CSF, genotype of the prion protein gene, and neuropathologic data of patients referred to our neurologic intensive care unit (NICU) with this presentation. RESULTS: Ten patients with a final diagnosis of definite (n = 7) or probable (n = 3) sCJD were referred to our NICU with an initial diagnosis of rNCSE. Reanalysis of the EEG ruled out ictal rhythmic activities, but showed diffuse, periodic, or semiperiodic sharp-wave complexes (PSWC) with short period. PSWC were briefly attenuated by auditory (n = 5) or painful (n = 3) stimuli and by IV injection of antiepileptic drugs (n = 5) but without clinical improvement. In addition, PSWC showed fluctuations according to the vigilance level (n = 5). Brain MRI showed hyperintensities in basal ganglia (n = 9/10) and in cortical areas (n = 7/10). 14-3-3 Protein was detected in CSF (n = 10). Only 2 sCJD subtypes were found (MM1 5/7, MV1 2/7). CONCLUSIONS: This series of patients suggests that sporadic Creutzfeldt-Jakob disease should be considered as a differential diagnosis, rather than as a cause, of apparent refractory nonconvulsive status epilepticus. Criteria for nonconvulsive status epilepticus diagnosis should rely on careful examination of both EEG parameters and clinical state so that aggressive, unnecessary treatments can be avoided.
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Síndrome de Creutzfeldt-Jakob/diagnóstico , Síndrome de Creutzfeldt-Jakob/fisiopatología , Estado Epiléptico/diagnóstico , Estado Epiléptico/fisiopatología , Adulto , Anciano , Anciano de 80 o más Años , Anticonvulsivantes/uso terapéutico , Síndrome de Creutzfeldt-Jakob/tratamiento farmacológico , Diagnóstico Diferencial , Electroencefalografía/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Sistema de Registros , Estudios Retrospectivos , Estado Epiléptico/tratamiento farmacológicoAsunto(s)
Síndrome de Creutzfeldt-Jakob/diagnóstico , Encéfalo/patología , Codón , Síndrome de Creutzfeldt-Jakob/genética , Síndrome de Creutzfeldt-Jakob/patología , Heterocigoto , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Proteínas Priónicas , Priones/genéticaRESUMEN
Several molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt-Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt-Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Patients with sporadic Creutzfeldt-Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as 'suspected sporadic Creutzfeldt-Jakob disease' but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt-Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt-Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic resonance imaging was positive in 83% of cases. In all definite cases, the amended criteria would cover the vast majority of suspected cases, being positive in 98%. Cerebral cortical signal increase and high signal in caudate nucleus and putamen on fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging are useful in the diagnosis of sporadic Creutzfeldt-Jakob disease. We propose an amendment to the clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease to include findings from magnetic resonance imaging scans.
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Síndrome de Creutzfeldt-Jakob/diagnóstico , Proteínas 14-3-3/análisis , Adulto , Anciano , Anciano de 80 o más Años , Antiinflamatorios/uso terapéutico , Biomarcadores/análisis , Corteza Cerebral/patología , Codón/genética , Síndrome de Creutzfeldt-Jakob/líquido cefalorraquídeo , Síndrome de Creutzfeldt-Jakob/genética , Electroencefalografía , Reacciones Falso Positivas , Femenino , Genotipo , Humanos , Cooperación Internacional , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Estándares de ReferenciaRESUMEN
BACKGROUND: With respect to sporadic Creutzfeldt-Jakob disease (sCJD), six molecular subtypes (MM1, MM2, MV1, MV2, VV1, and VV2) have been described, which vary with respect to age at disease onset, disease duration, early symptoms, and neuropathology. MRI signal alterations were reported to correlate with distinct Creutzfeldt-Jakob disease (CJD) subtypes. This multicenter, international study aimed to describe the brain MRI findings associated with each of the sCJD molecular subtypes. METHODS: Pathologically confirmed sCJD cases with codon 129 genotype (MM, MV, and VV), PrP(Sc) type, and fluid-attenuated inversion recovery (FLAIR) or diffusion-weighted imaging (DWI) were collected in seven countries. All MRI scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus, and cerebellum. RESULTS: MRI scans were evaluated in 211 CJD patients (98 MM1, 23 MM2, 19 MV1, 30 MV2, 9 VV1, and 32 VV2). Basal ganglia hyperintensities occurred most frequently in MV2, VV2, and MM1 subtypes (79, 77, and 70%). Wide cerebral cortical signal increase was most common in VV1, MM2, and MV1 subtypes (86, 77, and 77%). Thalamic hyperintensities occurred most often in VV2 (45%) and MV2 (43%). The most consistent finding across most subtypes was high signal in basal ganglia, with these abnormalities found in 63% (FLAIR) and 71% (DWI). CONCLUSION: Cortical signal increase and hyperintensities in the basal ganglia and thalamus are detected by MRI across all molecular sporadic Creutzfeldt-Jakob disease subtypes. Our findings argue that characteristic MRI lesion patterns may occur for each molecular subtype.
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Encéfalo/patología , Síndrome de Creutzfeldt-Jakob/patología , Imagen por Resonancia Magnética/métodos , Ganglios Basales/anatomía & histología , Ganglios Basales/patología , Ganglios Basales/fisiopatología , Encéfalo/anatomía & histología , Encéfalo/fisiopatología , Corteza Cerebral/anatomía & histología , Corteza Cerebral/patología , Corteza Cerebral/fisiopatología , Codón , Síndrome de Creutzfeldt-Jakob/clasificación , Síndrome de Creutzfeldt-Jakob/genética , Análisis Mutacional de ADN , Imagen de Difusión por Resonancia Magnética/métodos , Progresión de la Enfermedad , Pruebas Genéticas , Genotipo , Humanos , Fibras Nerviosas Mielínicas/patología , Variaciones Dependientes del Observador , Oportunidad Relativa , Proteínas PrPSc/genética , Valor Predictivo de las Pruebas , Sensibilidad y Especificidad , Tálamo/anatomía & histología , Tálamo/patología , Tálamo/fisiopatologíaRESUMEN
INTRODUCTION: Transmissible spongiform encephalopathies (TSE) have been under epidemiological surveillance in France and in Europe since the early 1990s. The observation of iatrogenic Creutzfeldt-Jakob disease (CJD), the outbreak of bovine spongiform encephalopathy (ESB) and its probable transmission to many species gave rise to the surveillance which remains warranted by the emergence of a variant of CJD (vCJD), in 1996. STATE OF ART: In France, epidemiological surveillance is coordinated by the InVS which receives input from cases notifications addressed to INSERM Unit 708 directly by clinicians or more often following requests for 14-3-3 detection in CSF. All suspected cases are followed up until a final diagnosis is established. Thanks to the effectiveness of the French network of neuropathology, autopsies are performed in more than half of patients who die with a diagnosis of suspected CJD. Diagnostic criteria allow comparison of the incidence of the different forms of the disease in all countries with a system of surveillance. Sporadic CJD is the most frequent form of the disease with more than 80% of the cases. Its origin remains unknown. To date, cases of iatrogenic CJD referred to the French surveillance network have been caused by dura mater grafts or human growth hormone treatments administrated in the 1980s. Ten percent of TSE are of genetic origin with an autosomic dominant transmission of a mutation or an insertion located on the PRNP gene. The most recent form of the disease is vCJD which is a new form, first described in the United Kingdom in 1994. PROSPECT AND CONCLUSION: Active epidemiological surveillance remains a timely issue, particularly in France, because of the development of new cases of iatrogenic CJD after human growth hormone treatment. It is of importance in France and worldwide because of the emergence of post-transfusional cases of vCJD and the possible appearance of vCJD in persons with valine-valine or methionine-valine genotypes at codon 129.
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Síndrome de Creutzfeldt-Jakob/epidemiología , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Animales , Bovinos , Síndrome de Creutzfeldt-Jakob/clasificación , Síndrome de Creutzfeldt-Jakob/genética , Síndrome de Creutzfeldt-Jakob/mortalidad , Femenino , Francia/epidemiología , Geografía , Humanos , Enfermedad Iatrogénica/epidemiología , Masculino , Persona de Mediana Edad , Enfermedades por Prión/epidemiologíaRESUMEN
We assessed the prevalence of Wernicke encephalopathy (WE) in all 657 cases suspected of Creutzfeldt-Jakob (CJD) referred from 2001 to 2006 to the French Neuropathology Network of CJD. Clinical, biological and imaging data were reviewed when the diagnosis of WE was made at autopsy. No CJD was found in five cases suspected of sporadic CJD. In these five cases, myoclonus had been observed in four, CSF 14-3-3 protein in two. In 14 other cases, WE was combined with CJD, 13 of which were sporadic. These belonged mainly to the molecular variants of sporadic CJD associated with a long duration of disease. This stresses the necessity of remaining alert to the diagnosis of WE when CJD is suspected.
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Encéfalo/patología , Síndrome de Creutzfeldt-Jakob/epidemiología , Síndrome de Creutzfeldt-Jakob/patología , Encefalopatía de Wernicke/epidemiología , Encefalopatía de Wernicke/patología , Proteínas 14-3-3/líquido cefalorraquídeo , Adulto , Anciano , Anciano de 80 o más Años , Síndrome de Creutzfeldt-Jakob/diagnóstico , Diagnóstico Diferencial , Humanos , Persona de Mediana Edad , Mioclonía/epidemiología , Mioclonía/patología , Prevalencia , Sistema de Registros , Factores de Tiempo , Encefalopatía de Wernicke/diagnóstico , Adulto JovenAsunto(s)
Codón/genética , Síndrome de Creutzfeldt-Jakob/diagnóstico , Síndrome de Creutzfeldt-Jakob/genética , Imagen de Difusión por Resonancia Magnética , Genotipo , Priones/genética , Ganglios Basales/patología , Núcleo Caudado/patología , Corteza Cerebral/patología , Cuerpo Estriado/patología , Humanos , Metionina/genética , Polimorfismo Genético , Pulvinar/patología , Estudios Retrospectivos , Valina/genéticaRESUMEN
INTRODUCTION: Spouses play a major role as care givers for their partners with Parkinson's disease. This de facto part of family nursing turns out to be so demanding that they often feel isolated. While spouses may have access to financial and technical aids, but no specific psychological support is available to assist them in coping with the difficulties they have to face. Supporting and educating spouses thus appears today to be a real need. METHODS: Wishing to create an appropriate support program responding to the needs and expectancies of spouses of Parkinson's disease, we conducted a study designed to measure the effects of Parkinson's disease on spouses' quality-of-life and identify the priority needs in terms of information and support. This study included the spouses of 14 patients who participated in semi-directive individual interviews and a focus group. RESULTS: The data collected shows that spouses experience great disarray when faced with the disease. Their perception of Parkinson's disease has a strong anxiogenic effects. Caring for their spouse on a day to day basis creates a permanent atmosphere of stress with an insecure feeling generating tensions and major frustrations. Most of the spouses do not allow themselves any break and are overwhelmed with ambivalent feelings. They experience a kind of hostility towards their spouse and at the same time feel guilty for their attitude and also for their helplessness. The disease also leads to an impoverishment of the couples' social network, due to reduced autonomy and fear of other people's way of looking at them. CONCLUSION: Our study confirms the usefulness of organizing an educational support program for these spouses who often feel very lonely and helpless when confronted with their partner's disease.
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Cuidadores/psicología , Enfermedad de Parkinson/psicología , Calidad de Vida , Adulto , Anciano , Anciano de 80 o más Años , Ansiedad/psicología , Femenino , Grupos Focales , Humanos , Entrevista Psicológica , Masculino , Persona de Mediana Edad , Apoyo SocialRESUMEN
Prosaccades and antisaccades were investigated in three groups of patients with parkinsonian syndromes, Parkinson's disease, corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP), and in a control group. Saccade tasks were performed in single-task blocks (i.e. either blocks of prosaccades or blocks of antisaccades) and in mixed-task blocks (i.e. in blocks of randomly interleaved pro- and antisaccades). Saccade latencies and directional errors (misdirected saccades) were analysed in each subject, and we concentrated more specifically on the comparison of error rates in single tasks and in repeated trials of mixed tasks (i.e. mixing costs). The performance of each group in single tasks was largely consistent with previous studies, with normal antisaccade error rates in Parkinson's disease and CBD patients and increased antisaccade error rates in PSP patients. In contrast, a double dissociation was observed in mixed tasks. Parkinson's disease and CBD patients showed a marked increase in prosaccade and antisaccade error rates in repeated trials of mixed tasks, illustrated by increased mixing costs, whereas PSP patients showed similar error rates in single and repeated trials of mixed tasks, i.e. normal mixing costs. These results demonstrate that: (i) antisaccade performances may be differentially affected in mixed tasks and single tasks; (ii) the region of the dorsolateral prefrontal cortex which is crucial for reflexive saccade inhibition does not seem to be involved in the additional processes required in mixed-task conditions; (iii) the study of interleaved pro- and antisaccades may increase the accuracy of the differential diagnosis between these parkinsonian syndromes.