The ataxic neuropathies

J Neurol. 2021 Oct;268(10):3675-3689. doi: 10.1007/s00415-020-09994-y. Epub 2020 Jun 15.


Ataxia is a frequent symptom in neurological cases with many causes. Sensory ataxia (due to involvement of the proprioceptive pathways) is observed in conditions affecting the central nervous system (spinal cord disorder) and the peripheral nervous system (peripheral neuropathy). The latter correspond to what we refer to as 'ataxic neuropathies'. Ataxic neuropathies represent a wide and heterogeneous spectrum of disorders that may affect dorsal root nerves, dorsal root ganglia, nerve trunks, distal nerve endings or all of them together. The identification of a predominant sensory ataxia in a case of peripheral neuropathy should raise the possibility of some specific etiologies. We propose here to present the main causes of ataxic neuropathies, which are identified with diagnostic workflows that are dictated by the topography of the likely sites of lesions in the proprioceptive pathway together with the timing of their occurrence (acute, subacute, or chronic).

Keywords: Ataxia; Dorsal ganglia; Immune-mediated; Neuronopathy; Paraneoplastic; Sensory; Sjögren.

Publication types

  • Review

MeSH terms

  • Ataxia / diagnosis
  • Ganglia, Spinal
  • Humans
  • Peripheral Nervous System Diseases* / complications
  • Peripheral Nervous System Diseases* / diagnosis
  • Spinal Nerve Roots