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Mechanisms of postural instability in hereditary spastic paraplegia

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Abstract

Hereditary spastic paraplegia (HSP) is characterized by progressive lower extremity spasticity and weakness, due to retrograde axonal degeneration of the corticospinal tract and posterior spinal columns. HSP patients fall frequently. We hypothesized that delayed postural responses contribute to their balance impairments. To distinguish between a delay in afferent and efferent signals, we combined postural responses with a startling acoustic stimulus (SAS). The SAS triggers a postural response directly, bypassing afferent proprioceptive input. We performed two experiments. First, 18 HSP patients and nine healthy controls stood on a balance platform and were instructed to counteract forward and backward balance perturbations, without taking a step or grabbing a handrail. Second, 12 HSP patients and nine controls received backward perturbations, while a SAS accompanied onset of platform motion in 25 % of trials. HSP patients were less successful than controls in maintaining balance following backward and forward perturbations. Furthermore, latencies of postural responses were significantly delayed in HSP-patients, by 34 ms in gastrocnemius following forward, and by 38 ms in tibialis anterior following backward perturbations. A SAS accelerated postural responses in all participants, but more so in HSP patients whose latencies were normalized. Our results suggest that delayed postural responses in HSP patients contribute to their balance problems. Combining balance perturbations with a SAS restored normal latencies, suggesting that conduction of efferent signals (presumably by the reticulospinal tract) is normal. We therefore suggest that the delayed postural responses in HSP are caused by slowed conduction time via the posterior spinal columns.

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Acknowledgments

This research was funded by a Radboud University Nijmegen Medical Centre Research Grant to JN, and a Netherlands Organization for Scientific Research Veni Research Grant (916.10.106) to VW. BRB was supported by a research grant from the Netherlands Organization for Scientific Research (016.076.352). We thank Roland Loeffen for technical assistance.

Conflicts of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

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Authors and Affiliations

  1. Department of Rehabilitation, Nijmegen Centre for Evidence Based Practice, Radboud University Medical Centre, Nijmegen, The Netherlands

    Jorik Nonnekes, Mark de Niet, Alexander C. Geurts & Vivian Weerdesteyn

  2. Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands

    Lars B. Oude Nijhuis, Susanne T. de Bot, Bart P. C. van de Warrenburg & Bastiaan R. Bloem

  3. Sint Maartenskliniek Research, Development and Education, Nijmegen, The Netherlands

    Alexander C. Geurts & Vivian Weerdesteyn

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  1. Jorik Nonnekes
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  2. Mark de Niet
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  3. Lars B. Oude Nijhuis
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  4. Susanne T. de Bot
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  5. Bart P. C. van de Warrenburg
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  6. Bastiaan R. Bloem
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  7. Alexander C. Geurts
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  8. Vivian Weerdesteyn
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Correspondence to Jorik Nonnekes.

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Nonnekes, J., de Niet, M., Nijhuis, L.B.O. et al. Mechanisms of postural instability in hereditary spastic paraplegia. J Neurol 260, 2387–2395 (2013). https://doi.org/10.1007/s00415-013-7002-3

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  • DOI: https://doi.org/10.1007/s00415-013-7002-3

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