Guillain-Barre syndrome

As in so many areas of scientific inquiry, the path to a mechanistic understanding of the pathobiology of Guillain-Barre syndrome (GBS) begins with phenomenology. A group of phenotypes fall under of the rubric of GBS, some with a very similar appearance and others quite disparate. The question of whether they represent a common pathogenic process with diverse clinical manifestations, or are distinct disease entities, awaits a definitive answer. Guillain, Barre, and Stroll initiated the debate in the early 20th century, insisting that their eponymous disorder was comparatively benign and, therefore, different from that described by Landry some 60 years earlier. The archetypal descriptor “ascending paralysis” remains applicable in characterizing the clinical features of most GBS subcategories. The term GBS, however, has taken on more generic connotations and has been largely supplanted by a more complex nosology based on the neurobiology of the disease. Contemporary electrophysiologic techniques have played a large role in the subclassification of GBS by permitting characterization of the pathologic processes affecting peripheral nerves, and the selective subpopulations of axons involved in these GBS variants. There is general consensus regarding the taxonomy of these GBS species with the most common entities including acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor and sensory axonal neuropathy (AMSAN), and acute motor axonal neuropathy (AMAN), all of which are phenomenologically more similar than not, along with Fisher syndrome, which is characterized by its strikingly distinctive features. While GBS has traditionally been conceptualized as an autoimmune disorder …