Handwriting Movement Abnormalities in Symptomatic and Premanifest Huntington's Disease

Abstract

Background Kinematic measures of handwriting movements are sensitive to mild subclinical motor abnormalities stemming from a wide range of disorders involving the basal ganglia including Huntington's disease (HD). Prior research has not investigated handwriting movements in at‐risk individuals in the premanifest stage of HD. Objectives The purpose of this study was to examine whether handwriting movement abnormalities are present prior to clinically manifest chorea in HD. Methods A total of 38 symptomatic HD, 30 gene‐positive premanifest, and 25 healthy control participants completed handwriting tasks consisting of circles, loops, sentences, and spirals with a noninking pen on a digitizing tablet. Multiple measures of pen stroke kinematics and pressure were measured along with the cognitive and motor status of each participant. Burden of pathology and CAG × age product scores were obtained from each participant with HD. Results Participants with HD exhibited significantly longer and more variable stroke durations, decreased handwriting smoothness, and increased and more variable pen pressures when compared with the healthy controls. We found significant positive associations between stroke duration and both burden of pathology and CAG × age product. Results from a discriminant function analysis revealed a 7‐factor model that distinguished premanifest from healthy controls with 85% accuracy. Factors in the model included greater variability in stroke amplitude, velocity and pen pressure, higher levels of pen pressure, longer stroke durations, and lower velocities for combinations of handwritten circles, sentences, and spirals. Conclusions These findings support the clinical utility of dynamic measures of handwriting kinematics as a potential early behavioral biomarker in HD.

A Smart Ink Pen for the Ecological Assessment of Age-Related Changes in Writing and Tremor Features

“…In this framework, it is clear that a technology that allows the combined study of handwriting and tremor features perfectly suits the current needs of the neurological research field. Indeed, the importance of studying handwriting is not restricted to Parkinson's Disease, but can be extended to a variety of other neurological disorders, including Dyskinesia [44], Huntington's Disease [4], and Multiple Sclerosis [45], not only to support the diagnosis process, but also to quantify the severity of clinical signs over time and to monitor and manage the risks associated with medications [44]. In addition, a technology that allows quantitative, simple, and ecologically valid evaluation of handwriting finds potential and interesting applications also in the youngest population, since handwriting and text production skills assume a central role in the children's development process.…”

“…For this reason, the analysis of this everyday activity has been leveraged for assessing different conditions. As for neurology, kinematic analysis of handwriting has been used as a clinical tool highly sensitive to even subtle dysfunctions, particularly useful in Parkinson's disease (PD) [2], Dystonia [3], and Huntington's disease [4] evaluation. Given its fine motor nature, handwriting analysis turned out to be a very useful tool also for the investigation of tremor [5].…”

hiPSCs for predictive modelling of neurodegenerative diseases: dreaming the possible

“…The main tool for assessing disease stage is the Unified Huntington’s Disease Rating Scale (UHDRS) 102 , which has several components that score motor, cognitive and functional abilities 103 . Also under development, and in early use, are rater-independent tests such as the Q-Motor assessment, which is a precalibrated, automated system that provides standardized measurements of the patient’s motor abilities to avoid rater bias 104 , 105 . On the molecular side, large efforts have been directed towards identifying fluid biomarkers, but so far only cerebrospinal fluid and plasma levels of neurofilament seem to be reliable indicators of HD progression 106 .…”

Online Handwriting, Signature and Touch Dynamics: Tasks and Potential Applications in the Field of Security and Health

“…These tasks are useful to support diagnosis of e.g. Parkinson's disease [86,85], Huntington's disease [7], essential tremor [63,80,70,27], developmental dysgraphia [31], fatigue [34], or brachial dystonia [81]. See Figure 6.…”

“…This kind of task is especially challenging for those people affected by cognitive impairment, such as dementia, mild cognitive impairment, etc. This is the case of the house drawing Archimedean spiral, meanders and straight lines -Parkinson's disease [86,85] -Huntington's disease [7] -essential tremor [63,80,70,27] -developmental dysgraphia [31] -fatigue [34] -brachial dystonia [81] Single or overlapped circles -Huntington's disease [7] -schizophrenia [9] Spring task -fatigue [34] -developmental dysgraphia [31,103] -schizophrenia [9,14] -bipolar disorder [14] -Parkinson's disease [93] -Huntington's disease [7] Rainbow task -developmental dysgraphia [31,67] Saw task -developmental dysgraphia [31] Drawing Tasks Security (user identification and verification)…”