Intact ability to learn internal models of arm dynamics in Huntington's disease but not cerebellar degeneration
Open accessAbstract
Two different compensatory mechanisms are engaged when the nervous system senses errors during a reaching movement. First, on-line feedback control mechanisms produce in-flight corrections to reduce errors in the on-going movement. Second, these errors modify the internal model with which the motor plan is transformed into motor commands for the subsequent movements. What are the neural mechanisms of these compensatory systems? In a previous study, we reported that while on-line error correction was disturbed in patients with Huntington's disease (HD), it was largely intact in patients with cerebellar degeneration. Here we altered dynamics of reaching and studied the effect of error in one trial on the motor commands that initiated the subsequent trial. We observed that in patients with cerebellar degeneration, motor commands changed from trial-to-trial by an amount that was comparable to control subjects. However, these changes were random and were uninformed by the error in the preceding trial. In contrast, the change in motor commands of HD patients was strongly related to the error in the preceding trial. This error-dependent change had a sensitivity that was comparable to healthy controls. As a result, HD patients exhibited no significant deficits in adapting to novel arm dynamics, whereas cerebellar subjects were profoundly impaired. These results demonstrate a double dissociation between on-line and trial-to-trial error correction suggesting that these compensatory mechanisms have distinct neural bases that can be differentially affected by disease.
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The cerebellum provides a substrate for motor adaptation through the updating of a forward model, but the striatum does not.
“while the cerebellum seems to provide a substrate for motor adaptation through the updating of a forward model, it is not the case for the striatum (Smith and Shadmehr, 2005; Graybiel, 2008; Seidler et al., 2013).”
From The Forward Model: A Unifying Theory for the Role of the Cerebellum in Motor Control and Sense of Agency (2021) · cites this paper as Smith and Shadmehr, 2005
Patients with cerebellar lesions are impaired in visuomotor rotation tasks.
“First, patients with cerebellar lesions are impaired in visuomotor rotation tasks (Weiner et al., 1983; Martin et al., 1996; Smith and Shadmehr, 2005; Tseng et al., 2007; Rabe et al., 2009; Werner et al., 2010; Schlerf et al., 2013; Bernard and Seidler, 2013; Burciu et al., 2014).”
From The Forward Model: A Unifying Theory for the Role of the Cerebellum in Motor Control and Sense of Agency (2021) · cites this paper as Smith and Shadmehr, 2005
Adaptation is dependent on the cerebellum.
“Numerous studies suggest that adaptation is dependent on the cerebellum (Martin et al., 1996a, b; Smith and Shadmehr, 2005; Tseng et al., 2007), a structure unaffected in Parkinson's disease (PD), and therefore initial learning in patients with PD would be expected to proceed as in controls – as indeed was recently demonstrated (Bedard and Sanes, 2011; Marinelli et al., 2009).”
From Rethinking motor learning and savings in adaptation paradigms: model-free memory for successful actions combines with internal models (2011) · cites this paper as Smith and Shadmehr, 2005
Adaptation is an automatic, obligate, perhaps reward-indifferent, cerebellar-based learning process.
“It appears, therefore, that adaptation is “automatic” – it is an obligate, perhaps reward-indifferent (Mazzoni and Krakauer, 2006), cerebellar-based (Martin et al., 1996a, b; Smith and Shadmehr, 2005; Tseng et al., 2007) learning process that will attempt to”
From Rethinking motor learning and savings in adaptation paradigms: model-free memory for successful actions combines with internal models (2011) · cites this paper as Smith and Shadmehr, 2005
Error-based learning is likely to be cerebellar-dependent.
“Error-based learning is likely to be cerebellar-dependent (Martin et al., 1996a, b; Smith and Shadmehr, 2005; Tseng et al., 2007).”
From Rethinking motor learning and savings in adaptation paradigms: model-free memory for successful actions combines with internal models (2011) · cites this paper as Smith and Shadmehr, 2005
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- Authors
- Smith, M. A., & Shadmehr, R.
- Affiliations
- Johns Hopkins University (US)
- Venue
- Journal of Neurophysiology
- Publisher
- American Physiological Society
- Published
- 2005-05
- Volume
- 93
- Issue
- 5
- Pages
- 2809-2821
- ISSN
- 0022-3077 (ISSN-L)
- 1522-1598
- OpenAlex
- W1988596442
- Cited by
- 497
- References
- 66
- Citations by year
- 2026: 9 · 2025: 18 · 2024: 12 · 2023: 12 · 2022: 14 · 2021: 27 · 2020: 27 · 2019: 29 · 2018: 24 · 2017: 30 · 2016: 24 · 2015: 32 · 2014: 34 · 2013: 36 · 2012: 33
- FWCI
- 10.87
- Type
- journal article
- Language
- en
- Open access
- green
- nrs.harvard.edu
- Licence
- cc-by
- Locations
- Journal of Neurophysiology (publishedVersion)
- PubMed (publishedVersion)
- Digital Access to Scholarship at Harvard (DASH) (Harvard University) (submittedVersion, cc-by)
- Funders
- Hereditary Disease Foundation
- National Institute of Neurological Disorders and Stroke (NS16375, P01NS016375, NS 37422, R01NS037422)
- Topics
- Life Sciences › Neuroscience › Cognitive Neuroscience › Motor Control and Adaptation
- Health Sciences › Medicine › Neurology › Neurological disorders and treatments
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Keywords
- Huntington's disease, cerebellar degeneration, internal models, arm dynamics, motor adaptation, error correction, motor command, reaching movements, double dissociation
- MeSH terms
- Adult, Arm, Female, Humans, Huntington Disease, Learning, Male, Middle Aged, Models, Neurological, Motor Activity, Psychomotor Performance, Spinocerebellar Degenerations, Task Performance and Analysis
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