ICD-11 classes
08 Diseases of the nervous system
Other disorders of the nervous system
8E4A — Paraneoplastic or autoimmune disorders of the nervous system
8E4A.0 — Paraneoplastic or autoimmune disorders of the central...

ICD-11 8E4A.0 — Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord

Paraneoplastic and autoimmune disorders of the central nervous system, brain and spinal cord nervous system result from a targeted immune attack on neurons or glial cells in the central (e.g. encephalopathy, ataxia, myelopathy, myelitis) nervous system. 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 central 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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 8E4A.0 contains 4 clarifying diagnoses:

1. 8A43 — Neuromyelitis optica
   It contains 9 clarifying diagnoses.
2. 9B71.4 — Paraneoplastic retinopathy
   It contains 3 clarifying diagnoses.
3. 9B71.5 — Autoimmune retinopathy
4. 8A42 — Acute disseminated encephalomyelitis
   It contains 3 clarifying diagnoses.