About two-thirds of Guillain-Barré Syndrome (GBS) patients have a history of a preceding infection, usually an upper respiratory tract infection or gastroenteritis. A number of microorganisms, both bacterial and viral, have been implicated in GBS, and possible associations with Campylobacter jejuni, (C. jejuni), cytomegalovirus (CMV), Epstein-Barr (EB) virus and mycoplasma have been demonstrated in case-control studies. The frequency of C. jejuni infection is much higher in Japan than that in the USA or Europe. In contrast, GBS in Japan is associated less frequently with CMV infection than in western countries.

Here I present a representative case of GBS, in whom neuropathic symptoms appeared following C. jejuni infection. The patient was an 82 year-old female. She had severe diarrhea about one week before the onset. Weakness of her upper extremities appeared and rapidly progressed to severe flaccid quadriparesis within a few days, and she needed ventilator support after admission. Characteristically she did not show any sensory deficits, although marked atrophy of the limbs appeared in the early stage. The laboratory examinations revealed elevated protein content with normal cell numbers in the cerebrospinal fluid (CSF). An electromyographic (EMG) study performed just after the onset showed inexcusability of nerves, but reservation potentials were found in many muscles one month later. Stool culture yielded C. jejuna, and antiGMI and GDlb IgG ganglioside antibodies were elevated at the onset and decreased thereafter. Her recovery was poor despite extensive treatment with plasma exchange, and she remained in a wheelchair one year after the onset of her illness. We diagnosed this case as axonal form of GBS following C. jejuna infection.

Jacobs et al. (Ann Neurol 1996;40:181-187) described the clinical characteristics of patients having GBS associated with jejune infection. About 40 % of GBS patients with jejune infection had diarrhea, and they had a more severe maximal weakness with less severe parenthesis and sensory deficits, as found in our presented patient. In our previous study, approximately 50 % of the patients with the axonal form, which is frequently encountered in Japan, had exhibited diarrhea, and in some cases C. jejuna was cultured as a putative causative agent. Our patient also showed increased anti-GM1 and GDlb IgG ganglioside antibody taters at the onset. According to the work of Hao et al. (J Neuroimmunol 1998; 81:116-126), there is a significant positive correlation between evidence of C. jejuni infection and the presence of anti-ganglioside antibodies, particularly anti-GM1 and GDlb antibody titers, in Japanese GBS patients.

Molecular mimicry of GMI by several strains of jejune provides a possible pathogenetic link between C. jejuna infection and anti-GM1 antibody. The lipopolysaccharides of C. jejuna contain ganglioside epitomes, including GM1, similar to those in peripheral nerves. After C. jejuna infection, the immune response elicited to epitomes in C. jejuna would trigger antibody production and these antibodies, directed to C. jejuna, would be cross-reactive with ganglioside in the myelin sheath or axolemma, causing the peripheral nerve injury. However as only a small proportion of persons with C. jejuni-induced diarrhea subsequently develop GBS, we speculate that other factors, such as involvement of specific C. jejuni strains or host susceptibility also play an important role in the pathogenesis of GBS.

In summary C. jejuna infection is the predominant antecedent infection in GBS. The frequency is especially high in Japan, and this may be consistent with the high frequency of the axonal form of GBS in Japanese patients. Clinically C. jejuna infection- associated GBS generally progresses to maximum disability within one week and is a severe and pure motor variant with a poor prognosis. Ventilator support is often needed. C. jejuna infection is often associated with the presence of antibodies against GM1, which may target and injure the peripheral nerves. So, if it were possible to prevent C. jejuna-induced GBS, we could markedly reduce the numbers of severely disabled survivors of GBS.