8E4A.1 Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous system

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

Paraneoplastic and autoimmune disorders of the peripheral and autonomic nervous system result from a targeted immune attack on neurons or glial cells in the peripheral nervous systems (peripheral or autonomic neuropathies). 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. Onset may be subacute or insidious and these disorders may be limited, multifocal or generalised. Autoimmune somatic peripheral nerve disorders may affect the nerve at multiple levels including root, nerve and plexus and may be axonal, demyelinating or both. Autoimmune autonomic disorders result in autonomic failure that can be partial or generalised. The prototypic autonomic neuropathy is the autoimmune ganglionopathy associated with antibodies targeting the ganglionic nicotinic acetylcholine receptor (α3 gAChR). Screening for cancer is appropriate for those with specific neural antibody profiles and other risk factors. Associated neural antibodies in peripheral neuropathies include anti neuronal nuclear antibody type 1 or anti-Hu, CASPR2, gAChR, ganglioside (GM1 IgG and IGM) antibodies.

postcoordination

synonyms

• Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous system
• Paraneoplastic sensory ganglionopathy
• Paraneoplastic sensory neuronopathy
• Paraneoplastic sensory ganglionopathy, neural autoantibody negative
• Autoimmune sensory ganglionopathy
• Autoimmune sensory ganglionopathy, neural autoantibody negative
• Autoimmune sensory ganglionopathy, neural autoantibody positive
• Autoimmune neuritis
• Autoimmune brachial neuritis
• Autoimmune radiculoplexoneuropathy
• Radiculoplexoneuropathy
• Nondiabetic radiculoplexoneuropathy
• Chronic immune demyelinating polyradiculoneuropathy
• Brachial variant of chronic immune demyelinating polyradiculoneuropathy
• Post surgical plexitis
• Postsurgical plexitis
• Post surgical brachial plexitis
• Post surgical lumbosacral plexitis
• Paraneoplastic sensorimotor neuropathy
• Paraneoplastic sensorimotor neuropathy, neural autoantibody negative
• Paraneoplastic sensorimotor neuropathy, neural autoantibody positive
• Vasculitis or vasculitic neuropathy
• Systemic multiple mononeuropathy vasculitis
• Multiple mononeuropathy vasculitis with mixed cryoglobulinemia
• Multiple mononeuropathy vasculitis with perinuclear antineutrophil cytoplasmic antibody-associated vasculitis
• Multiple mononeuropathy vasculitis with Churg-Strauss syndrome
• Multiple mononeuropathy vasculitis with Wegener granulomatosis
• Non-systemic multiple mononeuropathy vasculitis
• Nerve restricted multiple mononeuropathy vasculitis
• Autoimmune sensorimotor neuropathy
• Autoimmune sensorimotor neuropathy, neural autoantibody negative
• Autoimmune sensorimotor neuropathy, neural autoantibody positive
• Autoimmune autonomic neuropathy
• autoimmune autonomic ganglionopathy
• autoimmune pure autonomic failure
• autoimmune dysautonomia
• Pandysautonomia
• Pandysautonomia neural autoantibody positive
• Pandysautonomia neural autoantibody negative
• Autoimmune autonomic neuropathy, ganglionic acetylcholine receptor antibody positive
• Autoimmune autonomic neuropathy, other neural autoantibody positive
• Autoimmune autonomic neuropathy, neural autoantibody negative
• Limited autoimmune autonomic neuropathy
• Limited autoimmune autonomic neuropathy, ganglionic acetylcholine receptor antibody positive
• Limited autoimmune autonomic neuropathy, neural autoantibody positive
• Limited autoimmune autonomic neuropathy, neural autoantibody negative
• Paraneoplastic autonomic intussusception
• Autoimmune acquired autonomic encephalomyelitis
• Paraneoplastic sensory neuropathy
• Denny Brown
• Paraneoplastic sensory neuropathy, neural autoantibody negative
• Paraneoplastic sensory neuropathy, neural autoantibody positive
• Autoimmune sensory neuropathy
• Autoimmune sensory neuropathy, neural autoantibody negative
• Autoimmune sensory neuropathy, neural autoantibody positive
• Paraneoplastic motor neuropathy
• Paraneoplastic motor neuropathy, neural autoantibody negative
• Autoimmune motor neuropathy
• Autoimmune motor neuropathy, neural autoantibody negative
• Autoimmune motor neuropathy, neural autoantibody positive
• Monoclonal gammopathy of undetermined significance-associated neuropathies
• MGUS - [monoclonal gammopathy of undetermined significance] associated neuropathies
• Castleman disease with IgM-associated neuropathy
• Myelin-associated glycoprotein
• MAG - [Myelin-associated glycoprotein]
• Distal acquired demyelinating neuropathy
• DADS - [Distal acquired demyelinating neuropathy]
• IgG, IgA or IgM-associated osteosclerotic myeloma with polyneuropathy organomegaly endocrinopathy M-spike and skin changes
• Paraneoplastic neuromyopathy and neuropathy
• Carcinomatous neuromyopathy
• carcinomatous neuropathy
• Sensorial paraneoplastic neuropathy [Denny Brown]
• Paraneoplastic disorders of the spinal cord
• Paraneoplastic autonomic neuropathy
• Paraneoplastic neuropathy of unspecified site

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