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  • Updated 10.14.2021
  • Released 03.15.2001
  • Expires For CME 10.14.2024

Chronic idiopathic axonal polyneuropathy

Introduction

Overview

Idiopathic sensory and sensorimotor polyneuropathies typically present in middle to late adulthood as distal symmetric polyneuropathy, also named chronic idiopathic axonal polyneuropathy. A large number of disorders cause distal polyneuropathy, and a full diagnostic evaluation must be completed before categorizing a neuropathy as idiopathic. An expanding number of conditions and risk factors are associated with neuropathy, including metabolic syndrome impaired glucose tolerance, celiac antibodies, and monoclonal gammopathies. Skin biopsy for assessment of intraepidermal nerve fiber density is a valid and reliable method of confirming the diagnosis of small fiber sensory neuropathy. Neuropathic pain is a common presenting symptom and is often the focus of medical management.

Key points

• Despite being a common neurologic condition with apparent minimal associated disability, from the patient’s point of view, idiopathic axonal sensory and motor polyneuropathy does lead to impairment of activities of daily living, reduced quality of life, and is associated with other comorbidities that actually shorten lifespan and have significant socioeconomic impact (77).

• There is currently no known disease-modifying treatment.

Historical note and terminology

The term polyneuropathy is frequently used interchangeably with the term neuropathy to imply the same conditions. Chronic idiopathic axonal polyneuropathy is a useful operational term. The approach to this condition has changed over time, moving from improved recognition of various causes to isolation of an idiopathic disorder and more recently to identification of modifiable risk factors (83).

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