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  • Updated 05.09.2020
  • Released 04.26.1996
  • Expires For CME 05.09.2023

Acute inflammatory demyelinating polyradiculoneuropathy

Introduction

Overview

Acute inflammatory demyelinating polyradiculoneuropathy is the most frequent pattern of Guillain-Barré syndrome encountered in North America and Europe. In this review, the author explains the clinical features, criteria for diagnosis, advances in the pathogenesis, and treatment of acute inflammatory demyelinating polyradiculoneuropathy. In addition, variants of Guillain-Barré syndrome and related disorders are briefly discussed. This update includes some studies on biological markers in this disorder.

Key points

• Acute inflammatory demyelinating polyradiculoneuropathy is the most frequent pattern of Guillain-Barré syndrome encountered in North America and Europe.

• Molecular mimicry and ganglioside antibodies play an important role in the pathogenesis of some variants of Guillain-Barré syndrome (eg, acute motor axonal neuropathy).

• Intravenous immunoglobulins or plasma exchange are proven effective therapy for Guillain-Barré syndrome.

• Treatment approach to patients with treatment-related fluctuations or suboptimal clinical response after the initial therapy is an unsettled issue.

Historical note and terminology

Acute inflammatory demyelinating polyradiculoneuropathy is an inflammatory disease of the peripheral nervous system characterized by lymphocytic and macrophagic infiltration with destruction of myelin. The condition is often designated as the Landry-Guillain-Barré-Strohl syndrome or Guillain-Barré syndrome (GBS) in recognition of the descriptions provided by these authors. An acute, ascending, predominantly motor paralysis with respiratory failure and death was first reported by Landry in 1859 (46). In 1916 G Guillain and JA Barré, then French army neurologists, along with A Strohl reported 2 patients with acute polyradiculoneuritis and introduced the concept of "albuminocytologic dissociation" in the cerebrospinal fluid as a laboratory marker to distinguish this disorder from other neuropathies and from poliomyelitis (27). An approximate translation of the opening sentence in their report is:

We bring to attention in the present note a clinical syndrome that we have observed in 2 individuals, a syndrome characterized by motor difficulty, abolition of deep tendon reflexes with preservation of cutaneous reflexes, paresthesias without objective sensory loss, pain on deep palpation of large muscles, minor modifications in electrical reactions of nerve and muscle, and increased albumin in the cerebrospinal fluid with most notably, absence of cellular reaction (albuminocytologic dissociation) (27; 04).

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