Axonal Guillain-Barré syndrome: a critical review.
Axonal Guillain-Barré Syndrome (GBS) was first described by Feasby et al. in 1986, challenging the existent notion of GBS being a primarily demyelinating disease. The severe course and slow recovery commonly seen in these patients was ascribed to axonal degeneration. Other authors challenged this claim 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). Pathologically, Wallerian degeneration, minimal lymphocytic response, absent demyelination or inflammation and periaxonal macrophages are prominent features. Ultrastructural studies have revealed node of Ranvier to be the prime target of immune attack. A frequent occurrence of antecedent Campylobacter jejuni infection and a strong association between elevated titres of IgG GM1 and axonal GBS on a background of preceding C. jejunii infection has been observed and molecular mimicry between lipopolysaccharides of C. jejuni and neural epitopes has been proposed as a mechanism of injury. Clinically axonal variant is similar to AIDP, but a more severe course, with frequent respiratory involvement, ventilator dependence and significant residue may be seen. Diagnosis is essentially electrophysiological. Treatment is similar to AIDP, preferential benefit of either IVIG or plasmapheresis needs to be further evaluated. A critical review of existing literature in axonal GBS is presented.
Chowdhury
D
D
Department of Neurology, G.B. Pant Hospital, New Delhi, India. debashc@bol.net.in
Arora
A
A
D001369:Axons / Q000473:pathology*; D002169:Campylobacter Infections / Q000150:complications*; D016123:Campylobacter jejuni / Q000472:pathogenicity*; D002681:China / Q000453:epidemiology; D004196:Disease Outbreaks; D020275:Guillain-Barre Syndrome / Q000276:immunology / Q000382:microbiology / Q000503:physiopathology*; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D011379:Prognosis
D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous