Executive cognitive deficits in primary dystonia

Abstract

Primary dystonia is a disorder of movement for which no consistent pathophysiology has been identified; in the absence of evidence to the contrary, it is assumed to be cognitively benign. We have studied a clinically heterogeneous group of 14 patients with primary dystonia on a battery of neuropsychological tests. Despite well-preserved speed of information processing, language, spatial, memory and general intellectual skills relative to normal controls, we have identified a constellation of attentional-executive cognitive deficits on the Cambridge Neuropsychological Test Automated Battery (CANTAB). Specifically, patients demonstrated significant difficulties negotiating the extra-dimensional set-shifting phase of the IED task. The implications of these findings for the pathophysiology of primary dystonia are discussed. This is, to the best of our knowledge, the first report of a significant cognitive deficit in patients with primary dystonia.

Relationship of Cognitive Function to Motor Symptoms and Mood Disorders in Patients With Isolated Dystonia

“…These figures are very similar to those reported previously (van Tricht et al, 2012;Ostrem et al, 2011;Scott et al, 2003;Jahanashahi et al, 2003;Bugalho et al, 2008;Kleiner-Fisman et al, 2007;Kuyper et al, 2011).…”

“…Our patient sample of 38 is larger than most previously published studies (Ostrem et al, 2011;Taylor et al, 1991;Scott et al, 2003;Jahanashahi et al, 2003;Balas et al, 2006;Kleiner-Fisman et al, 2007;Vidalihet et al, 2005). Studies with similar size limitations and those with larger samples (up to n = 51) have also reported scattered impairments on tests of executive functioning (van Tricht et al, 2012;Bugalho et al, 2008;Romano et al, 2012).…”

“…Some studies have suggested preserved cognition in dystonia on the basis of a brief assessment battery only (Taylor et al, 1991). A more comprehensive study by Scott et al (2003) found intact performance on a range of standard measures of cognitive and executive functioning, but also selective impairment on one test of executive functioning from the CANTAB (Intra/Extra Dimensional Set-Shifting Task). Other investigations have also revealed scattered impairments, mostly on tests of executive functions.…”

Nonmotor symptoms in primary adult‐onset cervical dystonia and blepharospasm

“…Attention deficit was reported by recent studies of patients with different types of dystonia, including primary generalized dystonia and focal dystonia (Allam, Frank, Pereira, & Tomaz, 2007; Scott et al., 2003). Attention deficit may indicate the disruption of the dorsolateral‐prefrontal loop, which involves the striatum, thalamus and prefrontal cortex, regions showing altered functional activity and microstructural abnormalities in dystonia (Yang et al., 2013; Zoons, Booij, Nederveen, Dijk, & Tijssen, 2011).…”

Executive functioning in patients with blepharospasm in comparison with patients with hemifacial spasm

“…It has been also difficult to determine the specific impact of perceptual, mnemonic and executive cognitive deficits on focal dystonia 12,13 . Executive functions are mental processes involved in the performance of goal-directed behavior whether expressed through a mental or a motor act 14 .…”