Pathology of Guillain-Barré Syndrome

Abstract

Descriptions of myelin breakdown and inflammatory changes in peripheral nerves of patients with acute “multiple neuritis” or Landry’s ascending paralysis began to appear during the late nineteenth century, long before Guillain, Barré and Strohl’s paper on acute radiculoneuritis with albumino-cytological dissociation was published in 1916. However, the inflammatory changes were regarded as inconstant, late and secondary in an influential review and study of 50 personal cases by Haymaker and Kernohan in 1949. Twenty years later the modern history of Guillain-Barré syndrome began with the landmark study of Asbury et al. (1969) who emphasised the early appearance of lymphocytic infiltration and drew the important analogy with EAN. Subsequent electron microscopic studies, notably by Prineas (1981), have indicated a consistent association between macrophage infiltration and demyelination, similar to that noted in EAN (see Chapter 3). This chapter reviews these landmark studies in the light of our present understanding of ways in which demyelination may be induced by immunological means (see Chapter 2). In this review it is important to remember the alternative possibilities that demyelination neuropathy may be induced either by T cell-mediated responses to P₂ protein or by antibody-mediated responses to galactocerebroside. Another strand running through the interpretation of the pathology of GBS is that its clinical course is heterogeneous. While most patients make satisfactory recoveries, a few, often those with an explosive onset, develop wasting, persistent weakness and disability, and have electrophysiological evidence of axonal degeneration rather than demyelination.