I17 Sensory Processing In Huntington's Disease

International Journal of Psychophysiology, 1988

Scalp-recorded sensory evoked potentials (EPs) elicited by left and right median nerve stimulation (LSEP and RSEP), checkerboard pattern flash (VEP) and acoustic click (AEP) were obtained in 22 individuals 'at risk' (AR) for Huntington's disease and 22 hospitalized neurotic patients matched for age, gender and intelligence. EPs of AR subjects were generally similar to those of the neurotic comparison group, in terms of overall configuration; however, mean amplitudes were significantly lower for ARs. While the general amplitude reduction for ARs was seen in all three modalities, the somatosensory modality yielded the most abnormal findings.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques, 2018

Recent advances in neurophysiological techniques have contributed to our understanding of the pathophysiology of Huntington's disease (HD). Studies of the motor cortical excitability and central motor pathways have shown variable results. Our aims were to evaluate the cortical excitability changes in HD using transcranial magnetic stimulation (TMS) and correlate the changes with cognitive impairment. The study included 32 HD patients and 30 age- and gender-matched controls. The demographic and clinical profiles of the patients were recorded. All subjects were evaluated by TMS and resting motor threshold (RMT), central motor conduction time (CMCT), silent period (SP), short-interval intracortical inhibition (SICI), and intracortical facilitation were determined. A battery of neuropsychological tests was administered to all subjects. The mean age of the patients was 42.1±14.1 years, and that of controls 39.4±12.4 years (p=0.61). There was no significant difference in RMT and CMCT ...

Trends in Cognitive Sciences, 1998

Interest in the information-processing capabilities of anatomically-defined segregated corticostriatal circuits (see ) has spawned a number of conceptual and computational models from workers in a variety of disparate disciplines, ranging from robotics to neuropsychology 1-3 . Insights into the functions of the basal ganglia in human subjects can be achieved through the study of behavioural and cognitive symptoms resulting from neurodegenerative diseases such as Parkinson's disease (PD) and Huntington's disease (HD), which afflict these structures. The striking movement abnormalities caused by PD and HD have supported the view of the basal ganglia as structures important in movement control. However, it is evident that both of these disorders encompass more than simply motor deficits, with impairments in the cognitive and psychiatric domains increasingly becoming major foci of research. PD and HD affect basal ganglia function in very different ways; PD mainly through the degeneration of nigro-striatal dopaminergic circuitry, beginning in the putamen, and HD through the degeneration of the striatum itself, probably beginning in the caudate nucleus. These separate forms of pathology and their associations with distinctive motor symptoms, akinesia in PD and chorea in HD, suggest the possibility of qualitative differences in otherwise overlapping cognitive deficits, that might provide additional clues about the functioning of distinct parts of the striatal circuitry.

Journal of Neurophysiology, 2008

The basal ganglia are assumed to be of importance in action/response selection, but results regarding the importance are contradictive. We investigate these processes in relation to attentional processing using ERPs in Huntington's disease (HD), an autosomal genetic disorder expressed by degeneration of the basal ganglia, using a Flanker Task. A symptomatic HD-group, a presymptomatic HD-group (pHD) and healthy controls were examined.

Journal of Neuroscience, 2008

Cognitive functions are thought to deteriorate globally in late stages of various neurodegenerative disorders. Here we describe that this general assumption is not justified and fails in Huntington's disease (HD). Presymptomatic gene mutation carriers (pHDs) and healthy controls performed worse compared with symptomatic HDs in an auditory signal detection task. During task performance, behavioral data and event-related potentials (ERPs) [i.e., MMN (mismatch negativity), P3a, and RON (reorienting negativity)] were recorded. Not only behavioral performance but also neurophysiological correlates of auditory sensory memory and attentional reorientation indicate enhanced performance occurring primal in late stages of a neurodegenerative disorder. Increased activity of the NMDA-receptor system, an assumed pathogenic mechanism in HD, might facilitate signal propagation at striatal level that enables more efficient task execution through a winner-take-all process. The results challenge the view that late stage neurodegeneration is necessarily related to a global decline in cognitive abilities in HD. In contrast, selectively enhanced cognitive functioning can emerge together with otherwise impaired cognitive functioning.

Journal of Neuroscience, 2011

Cognitive decline precedes motor symptoms in Huntington disease (HD). A transgenic rat model for HD carrying only 51 CAG repeats recapitulates the late-onset HD phenotype. Here, we assessed prefrontostriatal function in this model through both behavioral and electrophysiological assays. Behavioral examination consisted in a temporal bisection task within a supra-second range (2 vs.8 s), which is thought to involve prefrontostriatal networks. In two independent experiments, the behavioral analysis revealed poorer temporal sensitivity as early as 4 months of age, well before detection of overt motor deficits. At a later symptomatic age, animals were impaired in their temporal discriminative behavior. In vivo recording of field potentials in the dorsomedial striatum evoked by stimulation of the prelimbic cortex were studied in 4-to 5-month-old rats. Input/output curves, paired-pulse function, and plasticity induced by theta-burst stimulation (TBS) were assessed. Results showed an altered plasticity, with higher paired-pulse facilitation, enhanced short-term depression, as well as stronger long-term potentiation after TBS in homozygous transgenic rats. Results from the heterozygous animals mostly fell between wild-type and homozygous transgenic rats. Our results suggest that normal plasticity in prefrontostriatal circuits may be necessary for reliable and precise timing behavior. Furthermore, the present study provides the first behavioral and electrophysiological evidence of a presymptomatic alteration of prefrontostriatal processing in an animal model for Huntington disease and suggests that supra-second timing may be the earliest cognitive dysfunction in HD.

Античная древность и средние века. Т. 52, 2024

COSTUME OF THE POPULATION OF THE CIMMERIAN BOSPOROS IN THE HUNNIC PERIOD The purpose of this work is to determine the main features and characteristics of the costume of the sedentary population of the Cimmerian Bosporos in the Hunnic Period (last third of the fourth to mid-fifth century). The elements of men’s costume comprise mostly of metal parts of belt sets and shoes. Women’s costume from the period features several traditions: Pontic, Germanic, and Ponto- Germanic. The Pontic tradition is typical of the presence of elements of women’s attire (earrings, necklaces, bracelets, metal appliques) with the absence of brooches, usual for the Germans. The costume of the Germanic tradition shows two paired brooches placed on the shoulders or in the pectoral area. The Ponto- Germanic costume includes both Germanic brooches and the elements of the Pontic costume. The main artifacts determining the specificity of the costume of the Cimmerian Bosporos from the Hunnic Period are some types of belt and shoe sets, brooches featuring the local tradition, metal appliques, metal wide-opening beads, necklaces with cone-shaped and lunular pendants, crescent- shaped or three- partite earrings, metal mirrors with a central loop, and bracelets with zoomorphic endings. The Bosporan costume from the Hunnic Period also contains other elements, which distribution area covers vast territories, so they cannot be considered typically Bosporan. Among these there are fly brooches, earrings with a multifaceted pendant on one ending, bracelets with widened ends, buckles with elongated tongues, and so on. Simultaneously, the artefacts typical of the Pontic / Bosporan costume also widely distributed in Europe during the Hunnic Period. All these factors supplied the Bosporan costume om the Great Migration Period with fully international character, typical of the sedentary population both on the territory of the Roman Empire and in the adjacent regions of the Barbaricum.

DISFUNCIONALIDAD DE LOS ESTADOS NACIONALES SUDAMERICANOS, 2024

El libro analiza la disfuncionalidad de los Estados nacionales sudamericanos para garantizar el control territorial, la seguridad y la defensa de los países. En cuanto a la metodología seguida, cada capítulo aborda los divesos problemas, limitaciones y dificultades que los países de la región presentan para el pleno control de sus territorios nacionales, señalando derroteros a seguir para corregir esta problemática.

We introduce for the first time the appurtenance equation and inclusion equation, which help in understanding the operations with neutrosophic numbers within the frame of neutrosophic statistics. The way of solving them resembles the equations whose coefficients are sets (not single numbers).

Revista Foral 2014 (16.ª Feira Medieval de Alhos Vedros, 7 a 10-6-2024), 2024

A família de Luís Vaz de Camões é ainda, em larga medida, um grande mistério! Agora que se comemoram os 500 anos do seu nascimento, resumimos neste pequeno ensaio alguns dados conhecidos sobre as origens familiares e inserção social do Príncipe dos Poetas, , tendo por base os dados coligidos pelos seus biógrafos do séc. XVII, como Manuel Correia, Severim de Faria, Faria e Sousa e o menos conhecido Franco Barreto, revistos e acrescentados por uma das mais notáveis camonistas do século XX, a arquivista Maria Clara Pereira da Costa, que entre 1979 e 1984 nos deu a conhecer vários documentos inéditos sobre a família de Luís de Camões, incluindo um seu primo afastado, Rodrigo Álvares de Camões, que em Lisboa foi contemporâneo e viveu perto do poeta no Bairro de Santana, mas também residiu em Almada e tinha uma importante propriedade na Margem do Sul do Tejo, a Quinta da Verderena Pequena, hoje na freguesia de Santo André (Barreiro), mas então pertencente ao termo de Alhos Vedros (Moita). * O texto tem duas revisões em relação à edição que foi publicada na Revista Foral, respeitantes às ligações a Almada e Caparica de Rodrigo de Álvares Camões e Manuel Severim de Faria, familiares de Camões, e de João de Ornelas, proprietário no séc. XV da Quinta da Verderena Pequena que depois veio a pertencer a Rodrigo Álvares. Publicada na Revista Foral 2014 (16.ª Feira Medieval de Alhos Vedros, 7 a 10-6-2024)