Disturbances of precision grip in Huntington's disease

Fellows, S J; Schwarz, Michael; Schaffrath, C; Dömges, Frank; Noth, Johannes

doi:10.1016/s0304-3940(97)00264-4

Cited by 34 publications

(19 citation statements)

References 14 publications

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“…Alterations in R6/2 mice were also observed in grip strength measurements, consistent with those observed in human HD (Fellows et al, 1997;Gordon et al, 2000). This recapitulation of the behavioral alteration observed in human HD demonstrates the utility of the grip strength test as a marker to assess the benefit of potential therapeutics for HD in the R6/2 mouse.…”

Section: Discussionsupporting

confidence: 78%

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

Stack

Kubilus

Smith

et al. 2005

J of Comparative Neurology

219

215

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Genetic murine models play an important role in the study of human neurological disorders by providing accurate and experimentally accessible systems to study pathogenesis and to test potential therapeutic treatments. One of the most widely employed models of Huntington's disease (HD) is the R6/2 transgenic mouse. To characterize this model further, we have performed behavioral and neuropathological analyses that provide a foundation for the use of R6/2 mice in preclinical therapeutic trials. Behavioral analyses of the R6/2 mouse reveal age-related impairments in dystonic movements, motor performance, grip strength, and body weight that progressively worsen until death. Significant neuropathological sequela, identified as increasing marked reductions in brain weight, are present from 30 days, whereas decreased brain volume is present from 60 days and decreased neostriatal volume and striatal neuron area, with a concomitant reduction in striatal neuron number, are present at 90 days of age. Huntingtin-positive aggregates are present at postnatal day 1 and increase in number and size with age. Our findings suggest that the R6/2 HD model exhibits a progressive HD-like behavioral and neuropathological phenotype that more closely corresponds to human HD than previously believed, providing further assurance that the R6/2 mouse is an appropriate model for testing potential therapies for HD.

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Section: Discussionsupporting

confidence: 78%

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

Stack

Kubilus

Smith

et al. 2005

J of Comparative Neurology

219

215

View full text Add to dashboard Cite

show abstract

“…COP displacements are aVected by the loss of sensory information (Fitzpatrick et al 1994;Horak et al 1990;Paulus et al 1984). Impaired proprioception has often been proposed to explain various movement deWcits in basal ganglia disorders (Fellows et al 1997;Klockgether and Dichgans 1994;Meyer et al 1992;Poizner et al 1998). However, several studies suggest that there are no sensory deWcits in TS (Abbruzzese and Berardelli 2003).…”

Section: Discussionmentioning

confidence: 99%

Postural control anomalies in children with Tourette syndrome

et al. 2007

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The goal of the present study was to determine whether postural control is aVected in Gilles-de-la-Tourette syndrome (TS). Center of pressure (COP) displacements were recorded in children with TS and unaVected siblings in three conditions using a force platform: (1) Eyes-Open, (2) Eyes-Closed, (3) One-Leg standing with eyes open. The COP range and velocity were higher in children with TS than in unaVected siblings in all conditions. These diVerences could not be attributed to age, present tic severity, comorbidities (hyperactivity and compulsions) or medication. The data suggest that sub-clinical postural control anomalies are present in TS.

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“…31 Although PD and HD are both disorders of the BG, their clinical and neurophysiological manifestations differ. For instance, whereas long latency reflexes (LLR) and somatosensory-evoked potentials (SEP) are largely ab-sent in HD, [32][33][34][35] LLR and SEP are present but abnormal in PD. 9,36,37 Despite these differences, it is interesting to note that both our study and that of Smith and colleagues 31 found the patient groups to be deficient in adaptive control.…”

Section: Discussionmentioning

confidence: 99%

Deficits in rapid adjustments of movements according to task constraints in Parkinson's disease

et al. 2004

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The role of the basal ganglia in the adaptive control of movement was investigated by unexpectedly perturbing movements in 8 patients with Parkinson's disease (PD) tested off medication and in 6 aged-matched healthy subjects. Subjects performed two movement components simultaneously and without visual feedback: touching the nose with the finger while leaning the trunk forward. Subjects wore a harness connected to an electromagnet, which was attached to a wall. The trunk movement was mechanically blocked in randomly selected trials by engaging the electromagnet. While healthy subjects performed the task equally well in both conditions, PD subjects' hand movements significantly deteriorated in trunk-perturbed compared to trunk-free trials. Deteriorated hand movements were characterized by segmented hand paths, unsmooth velocity profiles, and prolonged movement times. This finding indicated that the relatively local trunk perturbation had a global effect on the hand movement of PD subjects, necessitating them to reinitiate, after some delay, their arm movement in perturbed trials. Thus, the basal ganglia may be a critical node in brain networks mediating the flexibility of responses to altered motor states.

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Disturbances of precision grip in Huntington's disease

Cited by 34 publications

References 14 publications

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

Postural control anomalies in children with Tourette syndrome

Deficits in rapid adjustments of movements according to task constraints in Parkinson's disease

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