Cognitive and Neuropsychiatric Impairment in Dystonia

Abstract

Purpose of Review To review recent literature evaluating psychiatric and cognitive symptoms in dystonia, the two non-motor symptom groups most frequently evaluated in dystonia research and recognised in clinical practice. Recent Findings Recent work has embedded clinical recognition of psychiatric symptoms in dystonia, with depressive and anxiety-related symptoms routinely observed to be the most common. Less explored symptoms, such as self-harm, suicidal ideation, and substance abuse, represent newer areas of investigation, with initial work suggesting higher rates than the background population. Investigation of cognitive function has provided less consistent results, both within individual dystonia subtypes and across the spectrum of dystonias, partly reflecting the heterogeneity in approaches to assessment. However, recent work indicates impairments of higher cognitive function, e.g. social cognition, and disrupted visual and auditory sensory processing. Summary Dystonia demonstrates psychiatric and cognitive symptom heterogeneity, with further work needed to recognise endophenotypes and improve diagnostic accuracy, symptom recognition, and management.

The Impact of Non-Motor Symptoms on Quality of Life in Cervical Dystonia

“…The NMSs need a lot of attention from a clinical point of view, as they strongly influence the QoL and play a fundamental role in the perception of the patient, both of himself and of his life in general [10]. In addition, NMSs are an interesting starting point for research thanks to studies already carried out that classify a part of the symptoms analyzed as a primary component of dystonia [45].…”

Longitudinal predictors of health-related quality of life in isolated dystonia

“…Nonmotor symptoms (NMS), i.e. depression, generalized anxiety disorder (GAD), social anxiety disorder (SAD), self-esteem and acceptance of illness often concomitate the motor manifestations in isolated dystonia, occur more frequently than in healthy controls [ 1 – 7 ] and impact on the health-related quality of life (HR-QoL) [ 8 – 12 ]. The previous studies on HR-QoL in dystonia have been cross-sectional [ 8 – 14 ] and do not allow conclusions on predictors of future QoL.…”

Clinimetrics of the Italian version of the Montreal Cognitive Assessment (MoCA) in adult-onset idiopathic focal dystonia

“…The non-motor phenotype of patients with adult-onset idiopathic focal dystonia (AOIFD) (Albanese et al 2013 ) has been recently acknowledged to be possibly featured by cognitive deficits of a dysexecutive-inattentive and amnestic nature (Aita et al 2022 , Bailey et al 2022 ) which are thought to be accounted for by an involvement of both frontal-striatal networks (Kuyper et al 2011 ) and collicular, thalamic and middle-temporal structures (Rafee et al 2021 ). Consistently, and even though the functional entailments of such dysfunctions in this population are to this day mostly unknown (Monaghan et al 2021 ), cognitive screening has been preventively recommended in AOIFD patients (Yang et al 2017 ), especially to the aim of monitoring their cognitive status within the context of either surgical of pharmacological treatments (Jahanshahi 2011 , Jahanshahi 2017 ).…”

“…However, no consensus has been reached to this day as to which cognitive screener(s) might be suitable for use in this population–since available studies on cognition in AOIFD employed such tests solely to semiological aims, whilst neglecting the assessment of their clinimetrics and feasibility (Bailey et al 2022 ). However, in this respect, promising evidence has been delivered on the psychometric soundness and cross-sectional feasibility of the Montreal Cognitive Assessment (MoCA) (Nasreddine et al 2005 ) in patients with a genetic dystonia-parkinsonim syndrome (Aliling et al 2019 )–which present with neural alterations and thus cognitive features similar to AOIFD patients (Jamora et al 2014 ).…”