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

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

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Article numberArticle 54
Pages (from-to)54
JournalFrontiers in Neurology
Volume5 APR
DOIs
Publication statusPublished - 2014
Externally publishedYes

Keywords

  • Cervical dystonia
  • Covert attention
  • GABA
  • Superior colliculus
  • Temporal discrimination

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