Sensory neuronopathies

Muscle Nerve. 2016 Jan;53(1):8-19. doi: 10.1002/mus.24943. Epub 2015 Nov 23.

Abstract

The sensory neuronopathies (or ganglionopathies) are a small subcategory of neuropathies characterized by primary degeneration of the dorsal root ganglia and trigeminal ganglion sensory neurons, resulting in a distinctive clinical presentation. Patients typically have subacute onset of asymmetric, non-length-dependent sensory impairment and early ataxia. The etiologies of acquired sensory neuronopathies are rather limited. Early identification is imperative, as they may herald an underlying malignancy or an autoimmune condition such as Sjögren syndrome. This review addresses the various causes of acquired sensory neuronopathies, the recommended diagnostic approach, and treatment options. Finally, I will briefly discuss a select few hereditary and degenerative sensory neuronopathies, which, in contrast to the acquired disorders, are slowly progressive and are usually associated with additional neurological symptoms.

Keywords: Sjögren syndrome; dorsal root ganglion; paraneoplastic neuropathy; sensory ganglionopathy; sensory neuronopathy.

Publication types

  • Review

MeSH terms

  • Action Potentials / physiology
  • Afferent Pathways / physiopathology*
  • Humans
  • Neural Conduction / physiology
  • Peripheral Nervous System Diseases / pathology*
  • Sensory Receptor Cells / physiology*