Department of Neurology, Nerve-Muscle Unit, CHU Bordeaux (Pellegrin Hospital), University of Bordeaux, 1 place Amélie Raba-Léon, 33076, Bordeaux, France.
AOC National Reference Center for Neuromuscular Disorders, CHU Bordeaux (Pellegrin Hospital), University of Bordeaux, 1 place Amélie Raba-Léon, 33076, Bordeaux, France.
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).
共济失调是许多病因导致的神经科常见症状。感觉性共济失调（由于本体感觉通路受累）见于影响中枢神经系统（脊髓疾病）和周围神经系统（周围神经病）的疾病。后者对应我们所说的“共济失调性神经病”。共济失调性神经病是广泛而多样的疾病谱，可能影响背根神经、背根神经节、神经干、远端神经末梢或全部。在周围神经病中发现主要的感觉性共济失调应提示某些特定病因的可能性。在这里，我们提出了导致共济失调性神经病的主要病因，这些病因可通过诊断工作流程来确定，该流程由本体感觉通路中可能的病变部位的分布以及病变发生的时间（急性、亚急性或慢性）来决定。
