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Paraneoplastic or autoimmune disorders of the nervous system

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

sections/codes in this section (8E4A-8E4A)

• Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord  (8E4A.0)
• Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous system  (8E4A.1)
• Paraneoplastic or autoimmune neuromuscular transmission disorders  (8E4A.2)
• Paraneoplastic or autoimmune disorders of the muscle  (8E4A.3)
• Other specified paraneoplastic or autoimmune disorders of the nervous system  (8E4A.Y)
• Paraneoplastic or autoimmune disorders of the nervous system, unspecified  (8E4A.Z)

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