Peripheral nervous system neuroimmunology seen by a neuro-pathologist

Area of Science:

• Neurology
• Immunology
• Pathology

Background:

• Dysimmune neuropathies are characterized by microscopical lesions often described before immunological studies.
• Immune system dysregulation plays a role in peripheral nerve damage.

Purpose of the Study:

• To describe immune mechanisms in primitive autoimmune inflammatory peripheral polyneuropathies (Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy) and polyneuropathies linked to monoclonal dysglobulinemia.
• To review the variability of nerve lesions in dysimmune neuropathies.

Main Methods:

• Review of neuropathological and immunological studies.
• Case-by-case pathological examination (nerve biopsy) for diagnosis and mechanism identification.

Main Results:

• Humoral and cellular immune reactions against Schwann cells and/or axonal antigens are implicated in primitive dysimmune neuropathies.
• Monoclonal dysglobulinemia can directly harm peripheral nerves in associated polyneuropathies.

Conclusions:

• Immune tolerance failure underlies primitive dysimmune neuropathies.
• Understanding pathological lesions and mechanisms in monoclonal dysglobulinemia-associated polyneuropathies is crucial for guiding specific treatments.