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  • Updated 08.18.2020
  • Released 04.23.1996
  • Expires For CME 08.18.2023

Sarcoid neuropathy

Introduction

Overview

The author reviews current concepts of sarcoidosis-associated neuropathies. Clinical features, diagnostic considerations, and management strategies are discussed. In particular, the recent literature on sarcoidosis-associated small fiber neuropathy and possible new target-directed treatment options are highlighted. Concepts regarding the use of immunomodulatory agents are also highlighted.

Key points

• Sarcoidosis can involve the peripheral nervous system in a variety of ways.

• Patients can have a motor, sensory, sensorimotor, and/or autonomic neuropathy; the neuropathy may be primarily axonal or demyelinating.

• Patients can have involvement of isolated peripheral nerves, such as an ulnar neuropathy, or generalized involvement.

• Sarcoidosis is associated with virtually all peripheral neuropathy subtypes, eg, large fiber sensorimotor, pure motor, etc.

• Small fiber sensory neuropathy is the most common peripheral nerve manifestation and is seen in up to a third of patients with systemic disease.

• Small fiber sensory neuropathy is commonly associated with autonomic dysfunction.

• Intraepidermal nerve fiber density analysis, autonomic testing, and more recently, corneal confocal microscopy, provide objective confirmation for the diagnosis of small fiber neuropathy.

• Sarcoidosis-associated neuropathy may respond to immunosuppressive medications.

Historical note and terminology

Sarcoidosis was first described by Sir Jonathan Hutchinson more than a century ago (30; 12). The term "sarcoidosis" is derived from "sarkoid," used by Caesar Boeck at the turn of the twentieth century (10). Neurologic manifestations of sarcoidosis were reviewed as early as 1935 by Levin (37). Peripheral nerve involvement with sarcoidosis was first recorded by Winkler and Mazza (74; 40).

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