Abstract

The aim of this review was to analyse the pathophysiology of axonal degeneration in Guillain#x2013;Barr#xE9; syndrome (GBS) with emphasis on early stages (#x2264; 10 days after onset). An overview of experimental autoimmune neuritis (EAN) models is provided. Originally GBS and acute inflammatory demyelinating polyneuropathy were equated, presence of axonal degeneration being attributed to a #x201C;bystander#x201D; effect. Afterwards, primary axonal GBS forms were reported, designated as acute motor axonal neuropathy/acute motor#x2013;sensory axonal neuropathy. Revision of the first pathological description of axonal GBS indicates the coexistence of active axonal degeneration and demyelination in spinal roots, and pure Wallerian-like degeneration in peripheral nerve trunks. Nerve conduction studies are essential for syndrome subtyping, though their sensitivity is scanty in early GBS. Serum markers of axonal degeneration include increased levels of neurofilament light chain and presence of anti-ganglioside reactivity.