Objective
To see if the distribution patterns of phosphorylated 43-kDa TAR DNA-binding protein (pTDP-43) intraneuronal inclusions in amyotrophic lateral sclerosis (ALS) permit recognition of neuropathological stages.
Methods
pTDP-43 immunohistochemistry was performed on 70 μm sections from ALS autopsy cases (N=76) classified by clinical phenotype and genetic background.
Results
ALS cases with the lowest burden of pTDP-43 pathology were characterized by lesions in the agranular motor cortex, brainstem motor nuclei of cranial nerves XII-X, VII, V, and spinal cord α-motoneurons (stage 1). Increasing burdens of pathology showed involvement of the prefrontal neocortex (middle frontal gyrus), brainstem reticular formation, precerebellar nuclei, and the red nucleus (stage 2). In stage 3, pTDP-43 pathology involved the prefrontal (gyrus rectus and orbital gyri) and then postcentral neocortex and striatum. Cases with the greatest burden of pTDP-43 lesions showed pTDP-43 inclusions in anteromedial portions of the temporal lobe, including the hippocampus (stage 4). At all stages, these lesions were accompanied by pTDP-43 oligodendroglial aggregates. Ten cases with C9orf72 repeat expansion displayed the same sequential spreading pattern as non-expansion cases but a greater regional burden of lesions, indicating a more fulminant dissemination of pTDP-43 pathology.
Interpretation
pTDP-43 pathology in ALS possibly disseminates in a sequential pattern that permits recognition of four neuropathological stages consistent with the hypothesis that pTDP-43 pathology is propagated along axonal pathways. Moreover, the fact that pTDP-43 pathology develops in the prefrontal cortex as part of an ongoing disease process could account for the development of executive cognitive deficits in ALS.
Neurodegenerative diseases (NDs) are defined by the accumulation of abnormal protein deposits in the central nervous system (CNS), and only neuropathological examination enables a definitive diagnosis. Brain banks and their associated scientific programs have shaped the actual knowledge of NDs, identifying and characterizing the CNS deposits that define new diseases, formulating staging schemes, and establishing correlations between neuropathological changes and clinical features. However, brain banks have evolved to accommodate the banking of biofluids as well as DNA and RNA samples. Moreover, the value of biobanks is greatly enhanced if they link all the multidimensional clinical and laboratory information of each case, which is accomplished, optimally, using systematic and standardized operating procedures, and in the framework of multidisciplinary teams with the support of a flexible and user-friendly database system that facilitates the sharing of information of all the teams in the network. We describe a biobanking system that is a platform for discovery research at the Center for Neurodegenerative Disease Research at the University of Pennsylvania.
Political consumerism has recently generated academic interest among political participation researchers. While some scholars underscore political consumerism as an emerging civic and political engagement, others discredit its democratic potentials. Drawing on two national survey samples in 2002 that measured both boycotting and ‘buycotting’, this study examines political consumers' demographic backgrounds, socio‐political attitudes, issue orientations and their status of civic and political participation. Findings suggest that: (1) boycotters and buycotters should be distinguished because their demographic backgrounds are substantially different; (2) political consumers possess post‐material values and are mainly interested in lifestyle‐oriented social issues; and (3) political consumers are more active political participants. Theoretical implications of the findings and an agenda for future studies are also discussed.
With the advent of social network sites (SNSs), people can efficiently maintain preexisting social relationships and make online friendships without offline encounters. While such technological features of SNSs hold a variety of potential for individual and collective benefits, some scholars warn that use of SNSs might lead to socially negative consequences, such as social isolation, erosion of social cohesion, or SNS addiction. This study distinguishes types of SNS relationships, and investigates their relationships with social isolation, interpersonal trust, and SNS addiction. We classify SNS relationships into two types: (a) social relationships based on reciprocity between a user and his/her friends, and (b) parasocial relationships in which an ordinary user is aware of activities of a celebrity (e.g., famous actors, athletes, and others) but not vice versa. Based on achievements in studies of media effect and social psychology, we constructed a set of hypotheses, and tested them using a subsample of SNS users drawn from representative survey data in South Korea. We found that dependency on parasocial relationships is positively related with loneliness but negatively correlated with interpersonal distrust, while dependency on social relationship is negatively correlated with loneliness but positively related with trust. However, more dependency on both social and parasocial relationships are positively related with SNS addiction. Implications based on findings are also discussed.
Although there has been much speculation regarding the strengths and weaknesses of face-to-face versus online deliberative settings, no studies have systematically compared the two. Drawing on a national sample of Americans who reported deliberating faceto-face and/or online, we examine these two deliberative settings with regard to the participants, the motivations, the process, and the effects. Our findings, although tentative, suggest that the two settings are distinct in several important ways. Relative to face-to-face deliberation, online deliberation over-represents young, male, and white users, attracts more ideological moderates, generates more negative emotions, and is less likely to result in consensus and political action. At the same time, online deliberators perceived online settings as more politically and racially diverse. Implications for understanding the democratic potential of different forms of deliberation are discussed.
Relying on Gudykunst's cultural variability in communication (CVC) framework and culture‐specific facial expressions of emotion, we examined how people's use of emoticons varies cross‐culturally. By merging emoticon usage patterns on Twitter with Hofstede's national culture scores and national indicators across 78 countries, this study found that people within individualistic cultures favor horizontal and mouth‐oriented emoticons like :), while those within collectivistic cultures favor vertical and eye‐oriented emoticons like ^_^. Our study serves to demonstrate how recent big data‐driven approaches can be used to test research hypotheses in cross‐cultural communication effectively from the methodological triangulation perspective. Implications and limitations regarding the findings of this study are also discussed.
Public health officials (PHOs) are responsible for providing trustworthy information during a public health crisis; however, there is little research on how the public behaves when their expectations for such information are violated. Drawing on media dependency theory and source credibility research as our primary theoretical framework, we tested how credibility of information from PHOs is associated with people's reliance on a particular communication channel in the context of the 2015 Middle East Respiratory Syndrome (MERS) outbreak in South Korea. Using nationally representative data (N = 1036) collected during the MERS outbreak, we found that less credible information from PHOs led to more frequent use of online news, interpersonal networks, and social media for acquiring MERS-related information. However, credibility of information from PHOs was not associated with the use of television news or print newspapers. The theoretical and practical implications of our results on communication channels usage are discussed.
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