8E4A.0ICD-11 MMS 2026-01
Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord
Description
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.
Index terms
- Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord
- Paraneoplastic encephalitis
- Paraneoplastic encephalitis, neural autoantibody positive
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- Paraneoplastic encephalitis, neural autoantibody negative
- Autoimmune encephalitis
- Hashimoto encephalitis
- non-vasculitic autoimmune meningoencephalitis
- Autoimmune encephalitis, neural autoantibody positive
- Autoimmune encephalitis, neural autoantibody negative
- Paraneoplastic encephalopathy
- Autoimmune encephalopathy
- Hashimoto’s encephalopathy
- SREAT - [steroid-responsive-encephalopathy-associated with autoimmune thyroiditis]
- autoimmune dementia
- immunotherapy responsive encephalopathy
- Paraneoplastic limbic encephalitis
- Paraneoplastic limbic encephalitis, neural autoantibody positive
- Paraneoplastic limbic encephalitis, neural autoantibody negative
- Autoimmune limbic encephalitis
- Autoimmune limbic encephalitis, Neural autoantibody positive
- Autoimmune limbic encephalitis, Neural autoantibody negative
- Paraneoplastic brainstem encephalitis
- Paraneoplastic rhomboencephalitis
- paraneoplastic brain stem syndrome
- Paraneoplastic brainstem encephalitis, neural autoantibody positive
- Paraneoplastic brainstem encephalitis, neural autoantibody negative
- Autoimmune brainstem encephalitis
- autoimmune rhomboencephalitis
- Autoimmune brainstem encephalitis, neural autoantibody positive
- Autoimmune brainstem encephalitis, neural autoantibody negative
- Paraneoplastic cerebellar degeneration
- paraneoplastic ataxia
- Paraneoplastic cerebellar degeneration, neural autoantibody positive
- Paraneoplastic cerebellar degeneration, neural autoantibody negative
- Autoimmune cerebellar degeneration or cerebellitis
- autoimmune ataxia
- Autoimmune cerebellar degeneration or cerebellitis, neural autoantibody positive
- Autoimmune cerebellar degeneration or cerebellitis, neural autoantibody negative
- Paraneoplastic encephalomyelitis
- Paraneoplastic encephalomyelitis, neural autoantibody positive
- Paraneoplastic encephalomyelitis, neural autoantibody negative
- Paraneoplastic movement disorder
- Paraneoplastic movement disorder, autoantibody positive
- Paraneoplastic movement disorder, autoantibody negative
- Paraneoplastic chorea
- Paraneoplastic dyskinesia
- Autoimmune movement disorder
- paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections [PANDAS]
- Autoimmune movement disorder, autoantibody positive
- Autoimmune movement disorder, autoantibody negative
- Autoimmune chorea
- Autoimmune dyskinesia
- Autoimmune dystonia
- Autoimmune myoclonus
- Paraneoplastic myelopathy
- Paraneoplastic myelitis
- Paraneoplastic myelopathy, neural autoantibody positive
- Paraneoplastic myelopathy, neural autoantibody negative
- Autoimmune myelopathy
- autoimmune myelitis
- Autoimmune myelopathy, neural autoantibody positive
- Autoimmune myelopathy, neural autoantibody negative
- Stiff person syndrome
- Moersch-Woltman syndrome
- stiff man syndrome
- Stiff person syndrome classic form
- Stiff person syndrome classic form, glutamic acid decarboxylase-autoantibody positive
- Stiff person syndrome classic form, other neural autoantibody negative
- Stiff person syndrome classic form, other neural autoantibody positive
- Paraneoplastic stiff person syndrome classic form
- Stiff person syndrome variant form
- Stiff person syndrome variant form, glutamic acid decarboxylase autoantibody positive
- Stiff person syndrome variant form, other neural antibody positive
- Stiff person syndrome variant form, neural antibody negative
- Paraneoplastic stiff person syndrome variant form
- Neuromyelitis optica spectrum disorder
- Certain specified neurological disorder aquaporin-4 antibody positive
- Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids
- CLIPPERS - [Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids]
- Anti-NMDA receptor encephalitis
- Paraneoplastic dystonia
Exclusions from above levels
05– Endocrine, nutritional or metabolic diseases18– Complications of pregnancy, childbirth and the puerperium19– Certain conditions originating in the perinatal period
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22– Injury, poisoning or certain other consequences of external causes
Foundation children elsewhere
Sibling codes
8E4A.1– Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous system8E4A.2– Paraneoplastic or autoimmune neuromuscular transmission disorders8E4A.3– Paraneoplastic or autoimmune disorders of the muscle8E4A.Y– Other specified paraneoplastic or autoimmune disorders of the nervous system8E4A.Z– Paraneoplastic or autoimmune disorders of the nervous system, unspecified
Foundation entity
Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord – http://id.who.int/icd/entity/496011112
Allowed postcoordination axes
Part of: 8E4A: Paraneoplastic or autoimmune disorders of the nervous system › 08: Diseases of the nervous system