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Advances in the understanding of sensory neuronopathies
  1. Steve Vucic
  1. Westmead Hospital, Westmead, NSW 2145, Australia
  1. Correspondence to Steve Vucic, Westmead Hospital, Westmead, NSW 2145, Australia; steve.vucic{at}sydney.edu.au

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Evolving clinical phenotypes and causes linked to sensory neuronopathy.

Sensory neuronopathies (or ganglionopathies) represent a rare group of peripheral nerve disorders characterised by degeneration or dysfunction of dorsal root ganglia (DRG) or trigeminal sensory neurons.1 2 Large sensory fibres are typically affected, resulting in loss of proprioception and pseudoathetoid posturing of limbs, along with loss of vibration, touch and deep tendon reflects. In addition, severe gait ataxia and a positive Romberg’s sign are accompanying clinical features that result in marked disability. While motor neurons are preserved, ‘apparent’ clinical weakness secondary to loss of proprioception is often evident. Less frequently, small DRGs are only affected leading to burning, paraesthesia and stabbing pains, along with impaired pinprick and temperature sensations. In contrast to axonal neuropathies, sensory …

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Footnotes

  • Correction notice This article has been corrected since it appeared Online First. Name 'Chaniz-Laguna' has been corrected to 'Echaniz-Laguna' throughout.

  • Contributors I was commissioned to write the editorial.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Commissioned; internally peer reviewed.

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