Assessment of simple movements reflects impairment in Huntington's disease

Saft, Carsten; Andrich, Jürgen; Meisel, Nina-Marie; Przuntek, H.; Müller, Thomas

doi:10.1002/mds.20939

Cited by 32 publications

(28 citation statements)

References 14 publications

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“…Secondly, motor deficits can be detected early in the disease process, even in presymptomatic gene-positive subjects [8,9,19], raising the possibility that an objective marker might be of use even in early disease. And finally, motor deficits progress in line with other clinical measures of disease stage [17,20,21], and correlate with the loss of striatal D2 binding on raclopride 11C PET [18]. Such observations suggest that objective measures of motor function may indeed change over time, allowing us to follow disease progression.…”

Section: Introductionmentioning

confidence: 59%

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Hand tapping: A simple, reproducible, objective marker of motor dysfunction in Huntington’s disease

Michell

Goodman

Silva³

et al. 2008

J Neurol

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Huntington's disease (HD) is a severe neurodegenerative condition in which the impairment in voluntary movement is related to functional disability. Clinical assessment of motor deficit currently relies largely on subjective rating scales without objective measurement. We have developed a quick and easy-to-use hand tapping device that enables measurement of (a) the number of taps in 30 seconds, (b) variability in tapping rhythm and (c) fatigue over the testing period. Initial cross-sectional testing of 178 consecutive HD clinic patients using an early model of the device showed that the total number of taps in 30 seconds correlated with the motor UHDRS (Spearmann's rho, r(s) = -0.81, p < 0.0001) and independence scores (r(s) = 0.78, p = 0.01). Longitudinal data from a small cohort followed over 10 years reveals a correlation between total number of taps in 30 seconds and motor UHDRS over time (rs = -0.49, p < 0.001), and suggests the technique may provide an objective measure of disease progression. Further tests on 15 HD patients and 9 controls were repeated three times in a single day using an updated device. The HD group made significantly fewer taps in 30 seconds (median HD = 79, control = 104, p = 0.009) and had greater variability of inter-tap interval (mean interdecile range HD = 148, control = 56, p = 0.016) compared to controls. Both the total number of taps and variability of inter-tap interval correlated with motor UHDRS. Of vital importance for any potential marker of disease progression is that these tapping parameters were reproducible with repeated measurement. Given that hand tapping parameters differ between HD and control populations, they correlate with motor UHDRS over time and are reproducible, we propose that assessment of hand tapping represents a useful objective adjunct to the clinical assessment of HD patients.

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Section: Introductionmentioning

confidence: 59%

“…A small number of studies have addressed the quantitative assessment of motor phenotype in HD [3,4,7,8,15,17,20,21]. Of particular note, Garcia Ruiz and colleagues [7] found a difference between HD and control populations using the four motor tests suggested in the CAPIT [10].…”

Section: Introductionmentioning

confidence: 99%

Hand tapping: A simple, reproducible, objective marker of motor dysfunction in Huntington’s disease

Michell

Goodman

Silva³

et al. 2008

J Neurol

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“…Suitable items (6, finger taps; 7, pronate/supinate hands; 8, luria, fisthand-palm test; 9, rigidity arms; 11, maximal dystonia b/c (right/left upper extremity); 12, maximal chorea d/e (right/left upper extremity)) of the motor part of the UHDRS were added to the UHDRS total score. 2 …”

Section: Unified Huntington's Disease Rating Scalementioning

confidence: 99%

“…In contrast with more complex alternate finger tapping tasks, this apparatus asks for repetitive performance of simple movements. 2 Results of this motor test support assessment of clinical symptoms in addition to clinical rating scales-that is, the Unified Huntington's Disease Rating Scale (UHDRS). 2 3 This scale estimates behavioural, cognitive and motor dysfunction, but may have the physician's subjective impression.…”

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confidence: 92%

Assessment of simple movements and progression of Huntington's disease

Andrich

Saft²,

Ostholt³

et al. 2006

Journal of Neurology, Neurosurgery & Psychiatry

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Instrumental measurement of simple motion sequences reflects impairment in patients with Huntington's disease (HD). The objectives were to study the progress of symptoms of HD and tapping results in 42 patients with HD, without symptomatic drug treatment over 3 years. Assessment moments were at baseline, and at years 1, 2 and 3. Unified Huntington's Disease Rating Scale (UHDRS) total score and UHDRS arm score significantly increased. Motor test outcomes considerably worsened. Instrumental test results significantly correlated with both UHDRS scores at each assessment. Assessment of simple movement sequences is an additional simple method to follow impairment in patients with HD in addition to clinical rating. H untington's disease (HD) is a neurodegenerative, autosomal, dominantly inherited disorder, which manifests in early to middle adulthood and is associated with CAG repeat expansion in the first exon of a gene on chromosome 4. Characteristic clinical symptoms of HD are impaired cognition and behaviour, involuntary movements and bradykinesia, all of which influence initiation and execution of movement even in the early stages.1 This results in a reduced performance of instrumental motor tests, which assess simple movementsthat is, in patients with HD in comparison with healthy subjects.2 Results of this motor task are not specific, but this kind of motion assessment does not require the services of an experienced clinician. In contrast with more complex alternate finger tapping tasks, this apparatus asks for repetitive performance of simple movements.2 Results of this motor test support assessment of clinical symptoms in addition to clinical rating scales-that is, the Unified Huntington's Disease Rating Scale (UHDRS).2 3 This scale estimates behavioural, cognitive and motor dysfunction, but may have the physician's subjective impression.3 The objectives were to study the progress of symptoms in HD and tapping results in patients with HD, without symptomatic drug treatment over 3 years. Subjects and methods Patients with HDIn all, 21 female and 21 male patients with HD (mean (SD) age 48.79 (8.65) years; CAG 45.05 (2.95); age at onset of motor signs 40.74 (9.12) years; age at onset of psychiatric signs 39.1 (8.44) years) were enrolled. Patients with HD who had other medical conditions, which may affect the outcomes of the performed assessments, were excluded. DesignMolecular analysis of the CAG expansion was performed as described elsewhere. 4 We scored patients with HD with the UHDRS immediately before or after the motor test performance at each assessment moment at baseline (1), and at years 1, 2 and 3. The patients should receive no symptomatic drug treatment-that is, for involuntary movements, during the observation period. All participating doctors and technicians were blinded to each other. Unified Huntington's Disease Rating ScaleAll patients with HD were scored with the UHDRS. Suitable items (6, finger taps; 7, pronate/supinate hands; 8, luria, fisthand-palm test; 9, rigidity arms; 11, maximal dystonia...

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“…Both simple as well as complex (simultaneous and sequential) movements of the arm and hand reveal explicit deficits of timing at different stages of the disease. Simple timed motor tests such as finger dexterity, movement between two points, and walking tests, all demonstrate deterioration over time in manifest HD [9,30,35]. Hand-tapping parameters differ between HD and control populations and show high reproducibly and correlate with motor Unified Huntington's disease rating scale (UHDRS) changes over time in manifest cases of the disease [28].…”

Section: Introductionmentioning

confidence: 99%

Huntington’s disease: changes in saccades and hand-tapping over 3 years

Antoniades

Mason

et al. 2010

J Neurol

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Saccades and hand-tapping are both potential biomarkers in patients with Huntington's disease (HD). While it is well known that patients with manifest Huntington's disease display abnormalities in both of these tasks, less is known about how these abnormalities progress over time, or to what extent premanifest patients are affected. This study was designed to address these issues. We examined premanifest and manifest Huntington's cohorts, together with a group of controls, over a 3-year period. Data were collected using a portable head-mounted saccadometer and a computerised hand-tapping device. Both premanifest and manifest Huntington's disease patients display significant and systematic changes from year to year in the parameters describing saccadic latency, while controls remain unchanged. By contrast, although hand-tapping was abnormal in HD patients, annual changes were much smaller. Measuring the rate of progression of saccadic abnormalities in manifest HD patients may provide a way to track disease progression, and thus help to evaluate novel therapies to modify the disease. The clear-cut progression in saccadic abnormalities in the premanifest group may prove useful in the future as a predictor of time to disease onset.

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Assessment of simple movements reflects impairment in Huntington's disease

Cited by 32 publications

References 14 publications

Hand tapping: A simple, reproducible, objective marker of motor dysfunction in Huntington’s disease

Hand tapping: A simple, reproducible, objective marker of motor dysfunction in Huntington’s disease

Assessment of simple movements and progression of Huntington's disease

Huntington’s disease: changes in saccades and hand-tapping over 3 years

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