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Hutchinson M, Isa T, Molloy A, et al. Cervical dystonia: a disorder of the midbrain network for covert attentional orienting. Front Neurol. 2014;5:54doi: 10.3389/fneur.2014.00054.
Hutchinson, M., Isa, T., Molloy, A., Kimmich, O., Williams, L., Molloy, F., Moore, H., Healy, D. G., Lynch, T., Walsh, C., Butler, J., Reilly, R. B., Walsh, R., & O'Riordan, S. (2014). Cervical dystonia: a disorder of the midbrain network for covert attentional orienting. Frontiers in neurology, 554. https://doi.org/10.3389/fneur.2014.00054
Hutchinson, Michael, et al. "Cervical dystonia: a disorder of the midbrain network for covert attentional orienting." Frontiers in neurology vol. 5 (2014): 54. doi: https://doi.org/10.3389/fneur.2014.00054
Hutchinson M, Isa T, Molloy A, Kimmich O, Williams L, Molloy F, Moore H, Healy DG, Lynch T, Walsh C, Butler J, Reilly RB, Walsh R, O'Riordan S. Cervical dystonia: a disorder of the midbrain network for covert attentional orienting. Front Neurol. 2014 Apr 28;5:54. doi: 10.3389/fneur.2014.00054. eCollection 2014. PMID: 24803911; PMCID: PMC4009446.
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Front Neurol. 2014 Apr 28;5:54. doi: 10.3389/fneur.2014.00054. eCollection 2014.

Cervical dystonia: a disorder of the midbrain network for covert attentional orienting.

Frontiers in neurology

Michael Hutchinson, Tadashi Isa, Anna Molloy, Okka Kimmich, Laura Williams, Fiona Molloy, Helena Moore, Daniel G Healy, Tim Lynch, Cathal Walsh, John Butler, Richard B Reilly, Richard Walsh, Sean O'Riordan

Affiliations

  1. Department of Neurology, St. Vincent's University Hospital , Dublin , Ireland ; School of Medicine and Medical Science, University College Dublin , Dublin , Ireland.
  2. Department of Developmental Physiology, National Institute for Physiological Sciences , Okazaki , Japan.
  3. Department of Neurophysiology, Beaumont Hospital , Dublin , Ireland.
  4. Cork University Hospital , Cork , Ireland.
  5. Department of Neurology, Beaumont Hospital , Dublin , Ireland.
  6. Dublin Neurological Institute, Mater Misericordiae Hospital , Dublin , Ireland.
  7. Department of Statistics, Trinity College Dublin , Dublin , Ireland.
  8. Trinity Centre for Bioengineering, Trinity College Dublin , Dublin , Ireland.
  9. Department of Neurology, The Adelaide and Meath Hospital , Dublin , Ireland.

PMID: 24803911 PMCID: PMC4009446 DOI: 10.3389/fneur.2014.00054

Abstract

While the pathogenesis of cervical dystonia remains unknown, recent animal and clinical experimental studies have indicated its probable mechanisms. Abnormal temporal discrimination is a mediational endophenotype of cervical dystonia and informs new concepts of disease pathogenesis. Our hypothesis is that both abnormal temporal discrimination and cervical dystonia are due to a disorder of the midbrain network for covert attentional orienting caused by reduced gamma-aminobutyric acid (GABA) inhibition, resulting, in turn, from as yet undetermined, genetic mutations. Such disinhibition is (a) subclinically manifested by abnormal temporal discrimination due to prolonged duration firing of the visual sensory neurons in the superficial laminae of the superior colliculus and (b) clinically manifested by cervical dystonia due to disinhibited burst activity of the cephalomotor neurons of the intermediate and deep laminae of the superior colliculus. Abnormal temporal discrimination in unaffected first-degree relatives of patients with cervical dystonia represents a subclinical manifestation of defective GABA activity both within the superior colliculus and from the substantia nigra pars reticulata. A number of experiments are required to prove or disprove this hypothesis.

Keywords: GABA; cervical dystonia; covert attention; superior colliculus; temporal discrimination

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