I.P.Pavlov Saint-Petersburg State Medical University
A number of severe progressive neuropathies are based on the autoimmune
pathogenesis, which is considered essential in myasthenia gravis, myasthenic Lambert-
polyneuropathy, chronic inflammatory demyelinating polyneuropathy, multifocal motor
neuropathy, multiple sclerosis, inflammatory myopathies, syndrome of muscular
hypertonicity, autoimmune neuromyotonia, paraneoplastic cerebellar degeneration and
neuropathy, neuropathies associated with systemic vasculitis and viral infection. In
these disorders autoantibodies affect glial cells, myelin, axon, calcium channels, muscle
[Dalakas M.C., 1995].
Demyelinating diseases are widespread in the population with an unstoppable trend
to "rejuvenation", with rapid incapacitation and uniquely poor prognosis. Demyelination
is due to release of control of T-suppressors with the abolition of immune tolerance to
myelin basic protein (MBP). Antibodies against MBP stimulate complement which
increases the permeability of the blood-brain barrier to components of the immune
system. Antibodies to MBP, specifically affecting the oligodendrocytes and myelin, have
myelinolitic and myelinotoxic effects. During the incubation of these antibodies with
allogenic MBP a pronounced proteolytic effect is revealed [Vostrikova I.L. and others,
which is the data confirming the presence of common antigenic determinants between
the encephalitogenic region of MBP, and several viruses (measles, rubella, Epstein-Barr
virus, cytomegalovirus, herpes simplex). Obviously, as a result of a viral infection there
is the initial startup of autoimmune disorders, leading to failure of tolerance to MBP and
development of severe demyelinating disease. An important role in the pathogenesis of
autoimmune diseases is played by cytokines, in particular TNF-
which strengthen the
and thereby promoting demyelination
[Körner H. et al., 1997].
In multiple sclerosis, encephalomyelitis, neuromyelitis optica we can observe
the destruction of myelin in the white matter of the brain and spinal cord, the myelin
sheath of nerve trunks. Myelin acts as a sort of insulator, allowing the neuro-electrical
impulses spread in certain directions. Its elimination leads to multiple "short circuits" in
the process, which determines a particular clinical picture of the disease depends on the
level of "circuit".
Efferent therapy, mainly plasmapheresis, removing from an organism such
demyelinating agents, promotes if not recovery of the destroyed, then at least a slowing
of the progression of these diseases, the stabilization of state [Keegan M. et al., 2002;
S. et al., 2009;
., 2010; McDaneld L.M. et al., 2010; Gwathmey K. et al.,
each) there are signs of regression paralysis, impaired recovery of sensitivity, increase
muscle strength. Repeated annual courses suspend the progression of the disease and
improve quality of life for patients.
Let us examine some of these diseases in more detail.
Multiple sclerosis - demyelinating disease of the central nervous system. It is an
autoimmune inflammatory disease with diverse clinical manifestations, often leading to
severe damage of motor activity, paralysis, vision loss, disorders of the pelvic organs.
Although the etiology and pathogenesis are unknown, a number of features support the
hypothesis about the role of as yet unidentified infectious agent that triggers an immune
response against the distorted own nervous tissue, especially in genetically susceptible
individuals. Although the antibody titers detected increase to various viruses (influenza,
herpes simplex virus, Epstein-Barr virus, papilloma virus) in plasma and cerebrospinal
fluid, but there is no clear guidance for the detection of RNA or viral antigens in the
brain tissue itself. However, there are indications of the possible role of retroviruses in
multiple sclerosis [Allain J.-P., 1998]. There is a chance of a process of molecular
mimicry with the antigenic structure of individual proteins of viruses similar to the
proteins of the brain tissue. In this case, a possible autoantigen is influenced by the
excited viral antigens of T-lymphocytes, a myelin-oligodendrocyte-glycoprotein, breach
of which may underlie the pathogenesis of multiple sclerosis [Bernard C.C.A. et al.,
T-lymphocytes, which penetrate into microglia and activate secretion and release of
myelotoxic factors with direct damage to myelin in oligodendrocytes. Autoantibodies to
MBP join in the processes of demyelination in the later stages of the development of
multiple sclerosis [Vostrikova I.L. and others, 2006]. Activation of microglia cells also
leads to the production of proinflammatory cytokines, chemokines, which in turn drives
lymphocytes. In these processes there is also a release of "tumor necrosis factor", nitric
oxide and free oxygen radicals, interleukins 1 and 12. Cytokines are detected and
cerebro-spinal fluid. The content of IL12 can grow well in advance (4-6 weeks) to the
exacerbation of the disease [van Boxel-Dezaire A.H.H. et al., 1999].
A number of patients with multiple sclerosis exhibit antinuclear autoantibodies
characteristic of systemic lupus erythematosus which rather points to a systemic
disease [Fukazawa T., 1997].
Demyelination process is not irreversible, as there is some "nerve growth factor"
contributing to the restoration of myelin and nerve cell regeneration. However, the
presence of autoantibodies against this factor in multiple sclerosis weakens the
Currently there are no known reliable methods of treatment that may inhibit the
progression of the process. Based on the postulate of autoimmune nature of multiple