Jeremy H. Rees

Campylobacter jejuni Infection and Guillain–Barré Syndrome

BACKGROUND Although infection with Campylobacter jejuni is recognized as a common antecedent of the Guillain-Barré syndrome, the clinical and epidemiologic features of this association are not well understood. METHODS We performed a prospective case-control study in a cohort of patients with Guillain-Barré syndrome (96 patients) or Miller Fisher syndrome (7 patients) who were admitted to hospitals throughout England and Wales between November 1992 and April 1994. Bacteriologic and serologic techniques were used to diagnose preceding C. jejuni infection. RESULTS There was evidence of recent C. jejuni infection in 26 percent of the patients with Guillain-Barré or Miller Fisher syndrome, as compared with 2 percent of household controls and 1 percent of age-matched hospital controls (P < 0.001). Of the 27 patients with C. jejuni infection, 19 (70 percent) reported having had a diarrheal illness within 12 weeks before the onset of the neurologic illness. No specific serotypes were associated with Guillain-Barré syndrome. C. jejuni infection was slightly more common in men (P = 0.14) and was more likely to be associated with a pure motor syndrome and a slower recovery (P = 0.03). The patients with preceding C. jejuni infection were more likely to have acute axonal neuropathy or axonal degeneration in association with acute inflammatory demyelinating polyradiculoneuropathy, and they had greater disability after one year (P = 0.02). C. jejuni infection was significantly associated with a poor outcome even after correction for other factors associated with a poor prognosis. CONCLUSIONS Infection with C. jejuni often precedes the Guillain-Barré syndrome and is associated with axonal degeneration, slow recovery, and severe residual disability.

Clinical and Epidemiologic Features of Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is defined clinically as a peripheral neuropathy causing limb weakness that progresses for up to 4 weeks before reaching a plateau. The symptoms may be caused by inflammatory demyelination, axonal degeneration, or both. GBS occurs throughout the world, with a median incidence of 1.3 cases/100,000 population (range, 0.4-4.0). Males are more commonly affected than females, and there are peaks in young adults and the elderly. There is no clear seasonal association in Western countries, although this may be because the most frequent antecedent events, respiratory and enteric infections, have opposite seasonality. The most frequently identified cause of GBS is Campylobacter jejuni infection, which has been identified in up to 41% of patients and is associated with more severe disease and prolonged disability. Summer epidemics of GBS occur among children and young adults in Northern China and are particularly likely to be associated with C. jejuni infection.

Guillain-Barre syndrome

Guillain-Barre syndrome has now become recognized as a clinical syndrome that may be due to several pathological entities, consisting of an acute inflammatory demyelinating polyradiculoneuropathy as well as an acute motor axonal neuropathy. Campylobacter jejuni infection is a common preceding event and, together with anti-ganglioside GM1 antibodies, is associated with axonal damage and a poor outcome. The mechanism by which such antibodies damage axons is not clear. The Miller Fisher syndrome is very closely associated with antibodies to ganglioside CQ1b that may be important in pathogenesis. Treatment of Guillain-Barre syndrome with intravenous immunoglobulin appears to be as effective as plasma exchange in one controlled trial. Two small series have reported a high incidence of early relapses following intravenous immunoglobulin, and its efficacy is being reexamined in a further controlled trial.