Motor–Language Coupling in Huntington’s Disease Families

Abstract

Traditionally, Huntington’s disease (HD) has been known as a movement disorder, characterized by motor, psychiatric, and cognitive impairments. Recent studies have shown that motor and action–language processes are neurally associated. The cognitive mechanisms underlying this interaction have been investigated through the action compatibility effect (ACE) paradigm, which induces a contextual coupling of ongoing motor actions and verbal processing. The present study is the first to use the ACE paradigm to evaluate action–word processing in HD patients (HDP) and their families. Specifically, we tested three groups: HDP, healthy first-degree relatives (HDR), and non-relative healthy controls. The results showed that ACE was abolished in HDP as well as HDR, but not in controls. Furthermore, we found that the processing deficits were primarily linguistic, given that they did not correlate executive function measurements. Our overall results underscore the role of cortico-basal ganglia circuits in action–word processing and indicate that the ACE task is a sensitive and robust early biomarker of HD and familial vulnerability.

Syntax, action verbs, action semantics, and object semantics in Parkinson's disease: Dissociability, progression, and executive influences

“…Similarly, our finding that processing of action-related information in PD is impaired even at a strictly semantic level replicates previous results by Ibáñez et al (2013). The crucial implication of the BG in these domains is further highlighted by reports of similar impairments in other neurodegenerative motor disorders (Bak et al, 2006;Cotelli et al, 2006;Daniele et al, 2013;Kargieman et al, 2014;Silveri & Ciccarelli, 2007).…”

“…As García and Ibáñez (2014) argue, effective diagnosis might be achieved through early detection of linguistic alterations even before other domains are affected, paving the way for timely application of cognitive stimulation programs. Also, in light of convergent results from other clinical populations (Bak et al, 2006;Cotelli et al, 2006;Daniele et al, 2013;Kargieman et al, 2014;Silveri & Ciccarelli, 2007), this prospective assessment model could be extended to other neurodegenerative diseases involving comparable language affectation (especially in the domain of action verbs) following BG damage (e.g., progressive supranuclear palsy, Huntington's disease, cortico-basal degeneration).…”

“…We assessed linguistic (syntactic processing, action-verb production) and semantic (actionaction and object-object association) skills using tasks which have proven sensitive to subtle impairments in neurodegenerative motor disorders, including PD (Cardona et al, 2013;Colman, Koerts, Stowe, Leenders, & Bastiaanse, 2011;Cotelli et al, 2007;Ibáñez et al, 2013;Lieberman et al, 1990), Huntington's disease (Kargieman et al, 2014;Saldert, Fors, Stroberg, & Hartelius, 2010), and motor neuron disease (Bak & Hodges, 2004;Cobble, 1998). All tasks were administered in Spanish, the participants' mother tongue.…”

“…The role of BG in syntactic processing has been proposed to reflect their specialization for the acquisition and execution of hierarchical and sequential motor/cognitive routines (Ullman, 2001), their role in EFs (Grossman, 1999;Grossman, Lee, Morris, Stern, & Hurtig, 2002;Lee et al, 2003), and their involvement in cue-guided predictions (Kotz, Schwartze, & Schmidt-Kassow, 2009). Similarly, the crucial engagement of frontobasal structures during action-verb processing is highlighted by selective semantic and lexical deficits observed in multiple neurodegenerative motor diseases, such as motor neuron disease (Bak, 2013;Bak & Hodges, 2004;Bak, O'Donovan, Xuereb, Boniface, & Hodges, 2001;Bak et al, 2006;Hodges, & Bak, 1997), amyotrophic lateral sclerosis (Bak & Hodges, 2004;Neary, Snowden, & Mann, 2000), progressive supranuclear palsy (Bak et al, 2001;Bak et al, 2006), corticobasal degeneration (Cotelli et al, 2006;Silveri & Ciccarelli, 2007), and Huntingtonʼs disease (Kargieman et al, 2014).…”

Dementia in Latin America: An Emergent Silent Tsunami

“…Some research groups in Latin America are already addressing relevant clinical issues regarding Alzheimer's disease (Parra et al, 2009, 2010, 2015; Pietto et al, 2016) and other dementias (Cardona et al, 2013; Ibáñez et al, 2013; Baez et al, 2014a,b, 2015, 2016a,b,c; Kargieman et al, 2014; Garcia-Cordero et al, 2015, 2016; Melloni et al, 2015, 2016; Santamaria-Garcia et al, 2016; Sedeno et al, 2016). The urgent need now is to develop and implement health-care strategies and national plans that meet the needs of individuals with dementia and their families.…”