Infections & Guillain Barré Syndrome
Prof. Dr. F.G.A. Van Der Meché
Erasmus Medical Center Rotterdam, The Netherlands

Campylobacter Jejuni infections are now well known antecedent infections of the Guillain Barré syndrome. It has been found all around the world. In China it is associated with a primary axonal form as has been nicely elucidated by the joint Chinese-American research efforts. In the western world the pure motor form of GBS is associated with C. Jejuni infection. It is not easy to determine whether in these patients there is a pure axonal disorder or that the myelin is influenced also. Due to our optimal health care efforts there are hardly any autopsy studies available; therefore it will be very difficulty to confirm the phenomena found in china in the western world.
GBS preceded by C. Jejuni infection has a more severe course. In our studies however this seems primarily the case in patients treated with plasma exchange. Patients with C. Jejuni infection treated with immunoglobulins did relatively much better. Although this observation need to be consolidated in other studies, it favors further the use of immunoglobulins as a primary treatment, especially now also the international study coordinated by Prof. Hughes in London, UK, showed equal efficacy of immunoglobulins compared to plasma exchange. In the Netherlands a short media hype occurred at the end of 1996, when also the media discovered the association between C. Jejuni and GBS. They did so after the publication by the Dutch Consumers Organization, that chicken was much more often infected with Campylobacter than had been anticipated. It could be reassured however that we had not seen an increase of GBS over the recent years and that cooking the chicken well prevents infection.
Cytomegalovirus is the second most frequently occurring infection before the onset of GBS. We published results on the clinical aspects in association with CMV. It happens to be associated very strongly with more severe sensory deficit. Also the pattern of weakness is clarly different compared for instance with C. Jejuni associated GBS; in CMV related patients the weakness is more often starting in the more proximal muscles, the muscles close to the shoulders and the hips. This also includes the respiratory muscles and indeed these patients need more often artificial ventilation. Recovery is generally rather good after a more severe clinical course in the beginning. This may be due to the fact that CMV infection occurs in general more often in young adults and a younger age has seen shown to be a favorable prognostic factor in all prognostic studies.
In addition we have found an association between CMV related GBS and antibodies directed against the peripheral nerve ganglioside GM2 in 20% of the CMV patients. This is also found in smaller series by others. As in the case of C. Jejuni, molecular mimicry between structures on the infectious agent and the peripheral nerve may play a role in the development of GBS.
At present the multicenter study investigating the role of high dose methylprednisolone as an additional treatment besides immunoglobulins is still going on. In the meantime over 35 centers are involved including center in Germany and Spain. Centers in the USA and Belgium are in the process of getting involved. At this time, the study is half way completed. The first results are to be expected at the end of 1998.