Models of Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIPD) are autoimmune-mediated inflammatory diseases of the peripheral nervous system (PNS). The umbrella term of GBS is several variants with distinct clinical and pathological features. The most frequent from of GBS in the western countries is the acute inflammatory demyelinating polyneurophaty (AIPD), characterized by acute ascending paralysis, hyporeflexia with variable sensory involvement and histopathologically by mononuclear infiltrates and segmental demyelination. Chronic inflammatory demyelinating polyneuropathy (CIPD) can be relapsing-remitting or progressive. Experimental Autoimmune Neuritis (EAN) is a widely accepted model of GBS and is induced by immunization with peripheral myelin antigens. Disease develops after around 2 weeks with ataxia and weakness. Disease is normally monophasic.

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P2 induced EAN in rats

EAN is a model of acute inflammatory demyelinating polyneurophaty (AIPD), a subform of Guillain-Barré syndrome (GBS). AIPD is characterized by acute ascending paralysis, hyporeflexia with variable sensory involvement and histopathologically by mononuclear infiltrates and segmental demyelination. EAN is induced with peripheral myelin antigens (for example P2 peptide) in combination with adjuvant and results in a monophasic disease. P2-induced EAN in Lewis rats results in a severe and demyelinating monophasic neuropathy with weakness and ataxia. The disease is CD4+ T cell mediated but also macrophages and B cells play an important role. In this model, disease is accompanied by demyelination of the sciatic nerve as can be assessed by histology at termination. In addition, anti-P2 antibody responses can be followed in serum throughout the experiment. The CD4+ T cell dependency of this model allows for adoptive T cell transfer following ex vivo stimulation of autoreactive T cells.

EAN development in Lewis rats after P2/CFA immunisation

Lewis rats were injected s.c. with P2/CFA day 0. Cyclosporine was administered s.c. daily starting day 0.