Paraneoplastic or autoimmune disorders of the nervous system

Definition

Paraneoplastic and autoimmune disorders of the nervous system result from a targeted immune attack on neurons or glial cells in the central (e.g. encephalopathy, ataxia, myelitis) or peripheral nervous systems (peripheral or autonomic neuropathies, neuromuscular junction disorders or myopathy). In the paraneoplastic context, this attack is a consequence of a potentially effective tumour immune response initiated by onco-neural antigens derived from a systemic cancer. In the non-paraneoplastic context termed autoimmune the etiology remains elusive though increasing evidence indicates a preceding infectious trigger in at least some cases. These disorders are commonly multifocal causing injury and symptoms arising from involvement at many levels of the nervous system. A personal or family history of autoimmunity is often found. Accompanying neural and non-organ specific (thyroid peroxidase [TPO] antibodies) autoantibodies may be found. The neural autoantibody profile may be predictive of a specific cancer type and may be associated with a particular neurological phenotype. Exclusion of alternative etiologies (e.g. infections) is important. Response to immunotherapy may support the diagnosis.

Subcategories (4)