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

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2025-01

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.

code elsewhere

• Paraneoplastic retinopathy  (9B71.4)
• Autoimmune retinopathy  (9B71.5)
• Opsoclonus-myoclonus  (9C85.02)

postcoordination

synonyms

• Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord
• Paraneoplastic encephalitis
• Paraneoplastic encephalitis, neural autoantibody positive
• 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
• immunotherapy responsive encephalopathy
• autoimmune dementia
• SREAT - [steroid-responsive-encephalopathy-associated with autoimmune thyroiditis]
• 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
• stiff man syndrome
• Moersch-Woltman 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
• Post infectious encephalitis
• Acute demyelinating encephalomyelopathy
• Paraneoplastic dystonia

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