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J Neuropsychol. 2017 Mar 11. doi: 10.1111/jnp.12120. [Epub ahead of print]

Action-semantic and syntactic deficits in subjects at risk for Huntington's disease.

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Laboratory of Experimental Psychology and Neuroscience (LPEN), Institute of Cognitive and Translational Neuroscience (INCYT), INECO Foundation, Favaloro University, Buenos Aires, Argentina.
National Scientific and Technical Research Council (CONICET), Buenos Aires, Argentina.
Faculty of Education, National University of Cuyo (UNCuyo), Mendoza, Argentina.
Neuroscience Group, Faculty of Medicine, University of Antioquia (UDEA), Medellín, Colombia.
Group of Neuropsychology and Conduct (GRUNECO), Faculty of Medicine, University of Antioquia (UDEA), Medellín, Colombia.
Psychological Studies Department, Icesi University, Cali, Colombia.
Autonomous University of the Caribbean, Barranquilla, Colombia.
Departamento de Lingüística y Literatura, Facultad de Humanidades, Universidad de Santiago de Chile, Chile.
Center for Social and Cognitive Neuroscience (CSCN), School of Psychology, Universidad Adolfo Ibáñez, Santiago de Chile, Chile.
Centre of Excellence in Cognition and its Disorders, Australian Research Council (ACR), Sydney, New South Wales, Australia.


Frontostriatal networks play critical roles in grounding action semantics and syntactic skills. Indeed, their atrophy distinctively disrupts both domains, as observed in patients with Huntington's disease (HD) and Parkinson's disease, even during early disease stages. However, frontostriatal degeneration in these conditions may begin up to 15 years before the onset of clinical symptoms, opening avenues for pre-clinical detection via sensitive tasks. Such a mission is particularly critical in HD, given that patients' children have 50% chances of inheriting the disease. Against this background, we assessed whether deficits in the above-mentioned domains emerge in subjects at risk to develop HD. We administered tasks tapping action semantics, object semantics, and two forms of syntactic processing to 18 patients with HD, 19 asymptomatic first-degree relatives, and sociodemographically matched controls for each group. The patients evinced significant deficits in all tasks, but only those in the two target domains were independent of overall cognitive state. More crucially, relative to controls, the asymptomatic relatives were selectively impaired in action semantics and in the more complex syntactic task, with both patterns emerging irrespective of the subjects' overall cognitive state. Our findings highlight the relevance of these dysfunctions as potential prodromal biomarkers of HD. Moreover, they offer theoretical insights into the differential contributions of frontostriatal hubs to both domains while paving the way for innovations in diagnostic procedures.


Huntington's disease; action semantics; asymptomatic first-degree relatives; pre-clinical detection; syntax


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