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The role of sensory information in the pathophysiology of focal dystonias

Nature Reviews Neurology volume 15, pages 224–233 (2019)Cite this article

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Abstract

Over the past few decades, abnormalities in sensory functions, such as tactile, proprioceptive and nociceptive processing, have been increasingly recognized in patients with focal dystonias. In this Review, we ask whether sensory system abnormalities are specific to particular types of dystonia, whether a causal link exists between sensory alterations and dystonic motor activity and how mechanisms underlying the sensory abnormalities fit in with the proposed ‘network model’ of dystonia. We suggest that alterations in the various sensory modalities participate at three different levels in the pathophysiological cascade that leads to dystonia: a background level that predisposes individuals to dystonia, a disease-related level that is evident only when dystonia becomes manifest and a causative level that triggers dystonia. We conclude that it is crucial to study sensory as well as motor pathophysiology to fully understand focal dystonias.

Key points

  • Patients with different forms of focal dystonia, including blepharospasm, cervical dystonia and focal hand dystonia (FHD), share similar sensory abnormalities.

  • The mechanisms that underlie somatosensory temporal discrimination abnormalities make individuals susceptible to various forms of focal dystonia but are not directly linked to the emergence of motor symptoms.

  • Alterations in spatial discrimination reflect the remodelling of receptive fields in the cortex, which might result from dystonic motor manifestations.

  • Changes in proprioceptive afferent processing might have a direct causal role in FHD.

  • To fully understand focal dystonias, it is crucial to study both sensory and motor pathophysiology.

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Fig. 1: The network model of dystonia.
Fig. 2: Tactile and sensorimotor processing.

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Review criteria

PubMed was searched for full-text papers (original studies, randomized controlled trials and reviews) published in English from January 1990 to June 2018. The search terms used to perform the query were “sensory abnormalities and focal dystonia”, “somatosensory and focal dystonia”, “proprioceptive and focal dystonia”, “temporal discrimination, dystonia”, “spatial discrimination, dystonia”, “non-motor symptoms and dystonia”, “pain and focal dystonia” and “thermal abnormalities and focal dystonia”, individually and in combination. The search disclosed 216 articles. We then excluded case reports, meta-analyses and studies dealing with dystonia as a part of other movement disorders. Reference lists of identified articles were also searched for relevant papers.

Reviewer information

Nature Reviews Neurology thanks M. Fiorio, A. Sadnicka and another anonymous reviewer for their contribution to the peer review of this work.

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Authors and Affiliations

  1. IRCCS Neuromed, Pozzilli, Italy

    Antonella Conte, Giovanni Fabbrini & Alfredo Berardelli

  2. Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy

    Antonella Conte, Giovanni Fabbrini & Alfredo Berardelli

  3. Department of Basic Medical Sciences, Neurosciences and Sensory Organs, University of Bari Aldo Moro, Bari, Italy

    Giovanni Defazio

  4. Department of Medical Sciences and Public Health, University of Cagliari, Monserrato, Sardinia, Italy

    Giovanni Defazio

  5. Human Motor Control Section, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD, USA

    Mark Hallett

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Contributions

A.C. researched data for the article, and all authors made substantial contributions to discussions of the content. A.B., A.C. and M.H. wrote the article. A.B., G.D., G.F. and M.H. reviewed the manuscript before submission.

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Correspondence to Alfredo Berardelli.

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Glossary

Homotopic and associative cerebral plasticity

A time-dependent form of plasticity in humans that relies on associative stimulation of two neural substrates in a temporally coordinated manner.

Thermal sensory limen test

A test for thermal sensation that belongs to the quantitative sensory testing battery. This test identifies the difference threshold for alternating cool and warm stimuli.

N2–P2 LEP complex

A cortical evoked potential recorded from the scalp at the vertex after laser-induced stimulation of the nociceptive fibres.

Centre-surround inhibition

The suppression of activity of neighbouring cells by a central group of neurons.

N20 somatosensory evoked potential component

The N20 component of the evoked potential recorded from the parietal cortex after median nerve electrical stimulation at the wrist.

Cross-modal temporal discrimination

The temporal discrimination of two consecutive stimuli of different sensory modalities.

Aristotle’s illusion

A tactile illusion that is created when two fingers of one hand are crossed and a small object is pressed into the cleft between the tips of the crossed fingers. The sensation is that of touching two objects rather than one.

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Conte, A., Defazio, G., Hallett, M. et al. The role of sensory information in the pathophysiology of focal dystonias. Nat Rev Neurol 15, 224–233 (2019). https://doi.org/10.1038/s41582-019-0137-9

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