Axonal Guillain-Barre syndrome: a critical review

Axonal Guillain-Barre Syndrome (GBS) was first described by Feasby et al. i n 1986, challenging the existent notion of GBS being a primarily demyelinat ing disease. The severe course and slow recovery commonly seen in these pat ients was ascribed to axonal degeneration. Other authors challenged this cl aim on several grounds. Amidst these controversies, epidemics of a similar illness were reported from China, which were given the acronym AMAN, having exclusive motor involvement in contrast to the cases already described in which both motor and sensory involvement were present (AMSAN). Pathological ly, Wallerian degeneration, minimal lymphocytic response, absent demyelinat ion or inflammation and periaxonal macrophages are prominent features. Ultr astructural studies have revealed node of Ranvier to be the prime target of immune attack. A frequent occurrence of antecedent Campylobacter jejuni in fection and a strong association between elevated titres of IgG GM1 and axo nal GBS on a background of preceding C. jejuni infection has been observed and molecular mimicry between lipopolysaccharides of C. jejuni and neural e pitopes has been proposed as a mechanism of injury. Clinically axonal varia nt is similar to AIDP, but a more severe course, with frequent respiratory involvement, ventilator dependence and significant residue may be seen. Dia gnosis is essentially electrophysiological. Treatment is similar to AIDP, p referential benefit of either IVIG or plasmapheresis needs to be further ev aluated. A critical review of existing literature in axonal GBS is presente d.