Axonal Guillain-Barré syndrome: a critical review

Abstract

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.

Complete genome sequence and analysis of Wolinella succinogenes

“…Only recently Campylobacter jejuni was recognized as the causative agent of serious illnesses such as Guillain-Barré syndrome (3) and human gastroenteritis (4). The most prominent representative of the Helicobacteraceae is Helicobacter pylori, which has been demonstrated to cause ulcers and gastric cancer in humans (3). These organisms belong together with Wolinella succinogenes to the epsilon subclass of the proteobacteria, as has been demonstrated by 16S rRNA analysis (5,6).…”

Differences in Membrane Properties in Simulated Cases of Demyelinating Neuropathies: Internodal Focal Demyelinations with Conduction Block

“…The demyelinations are associated with a corresponding loosening of the myelin lamellae away from the axolemma in the given internodal segments. The other membrane parameter values for the human motor fibre are the same as described earlier [18,20] 3. We would like to remind the reader that the channel types are also in accordance with the directly investigated ion channels in human axons [22,23].…”

“…In some of these disorders such as Charcot-Marie-Tooth disease type 1A (CMT1A) [1,2], the demyelination uniformly affects the nerve, whereas in other disorders such as Guillain-Barré syndrome (GBS) and multifocal motor neuropathy (MMN) the demyelinative process affects the nerve focally. GBS is classified into acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) by pathological and electrodiagnostic criteria [3][4][5]. A critical diagnostic feature in the MMN is the demonstration of conduction block in multiple peripheral nerves on electrophysiological investigations [6,7].…”

Comparison of efficacy and outcome of intravenous immunoglobulin therapy versus plasmapheresis in patients with Gullian Barre syndrome

“…11 Lawn ND et al in their study have found that bulbar dysfunction and upper limb paralysis were significant predictors of requirement of mechanical ventilation. 12 In patients with GBS definitive treatment should be initiated as soon after diagnosis as possible.…”