Recent Advances in Structural Basis for Molecular Mimicry in Inflammatory Autoimmune Demyelinating Polyneuropathy - Molecules: Nucleation, Aggregation and Crystallization - page 306

Introduction
Molecular mimicry is one mechanism by which infectious agents may trigger
an immune response against auto-antigens. Four criteria must be satisfied to
conclude that a disease is triggered by molecular mimicry (Ang et al ., 2004):
(i) the establishment of an epidemiological association between the infec-
tious agent and the immune-mediated disease, (ii) the identification of T-cells
or antibodies directed against the patient's target antigens, (iii) the identifi-
cation of microbial mimics of the target antigen, and (iv) reproduction of the
disease in an animal model. This review provides an update on the molecu-
lar mimicry of the acute inflammatory autoimmune demyelinating and
axonal forms of Guillain-Barré syndrome (GBS), Miller Fisher syndrome
(MFS), and chronic inflammatory demyelinating polyneuropathy (CIDP).
Guillain-Barré syndrome
GBS is a representative paralytic syndrome characterized by autoimmune
response induced inflammation in the peripheral nervous system (PNS),
with an annual incidence of two cases per 1 000 000 of the population.
GBS is defined as a recognizable clinical entity characterized by rapidly
evolving symmetrical limb weakness, loss of tendon reflexes, the absence
of mild sensory signs and autonomic dysfunctions. Being the most fre-
quent cause of acute flaccid paralysis initiated by a range of quite distinct
immunopathological events, GBS has been identified as being triggered
by many kinds of agents: Campylobacter jejuni ( C. jejuni , in 13-39%
of cases), cytomegalovirus (CMV, 5-22%), Haemophilus influenzae
( H. influenzae , 1-8%), Epstein-Barr virus (EBV, 1-13%), and Mycoplasma
pneumoniae ( M. pneumoniae , 5%) (Godschalk et al ., 2007; Jacobs et al .,
1998; Koga et al ., 2005). All of these pathogens have carbohydrate epi-
topes which are common with glycoproteins of peripheral nerve tissue.
The most frequent pattern of GBS encountered in Europe and North
America is that originally described as acute inflammatory demyelinating
polyradiculoneuropathy (AIDP), with demyelination and a variable degree of
lymphocytic infiltration. In some other developing areas in the world, in
China and in Japan (Kuwabara et al ., 2003), the axonal pattern of GBS, acute