ORIGINAL ARTICLES

FAMILIAL GUILLAIN-BARRE SYNDROME

Since the eradication of poliomyelitis in the western hemisphere, Guillain-Barré syndrome (GBS) has become the most common cause of acute neuromuscular paralysis in the developed world. GBS is an acute disease of peripheral nerves that is characterized by the stripping away of myelin in a segmental fashion, resulting in rapidly ascending paralysis that can lead to respiratory muscle compromise and death. It has long been recognized that frequently, GBS is preceded by an acute infectious illness. Indeed, the renowned late 19th- and early 20th-century physician, Sir William Osler, called the syndrome “acute post-infectious polyneuritis” in 1892.⁵⁷ Evidence is mounting, however, that the most important trigger of GBS is not vaccines or viruses but infection with the common diarrhea-producing bacteria, Campylobacter jejuni. This article reviews the evidence that Campylobacter infections may cause GBS and discusses the pathogenesis of Campylobacter- induced GBS.

HLA typing for class I and II antigens was performed on 89 caucasoid patients with Guillain-Barré syndrome and compared with controls. No significant association, between HLA type and susceptibility to disease, ptognosis at 12 months or antecedent event was discovered. HLA antigen DR2 was more frequent among patients with the most profound muscle weakness.