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8E4A.1 category

Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous system

Definition

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.

Also indexed as

Autoimmune acquired autonomic encephalomyelitisAutoimmune autonomic neuropathyAutoimmune autonomic neuropathy, ganglionic acetylcholine receptor antibody positiveAutoimmune autonomic neuropathy, neural autoantibody negativeAutoimmune autonomic neuropathy, other neural autoantibody positiveAutoimmune brachial neuritisAutoimmune motor neuropathyAutoimmune motor neuropathy, neural autoantibody negativeAutoimmune motor neuropathy, neural autoantibody positiveAutoimmune neuritisAutoimmune radiculoplexoneuropathyAutoimmune sensorimotor neuropathyAutoimmune sensorimotor neuropathy, neural autoantibody negativeAutoimmune sensorimotor neuropathy, neural autoantibody positiveAutoimmune sensory ganglionopathyAutoimmune sensory ganglionopathy, neural autoantibody negativeAutoimmune sensory ganglionopathy, neural autoantibody positiveAutoimmune sensory neuropathyAutoimmune sensory neuropathy, neural autoantibody negativeAutoimmune sensory neuropathy, neural autoantibody positiveBrachial variant of chronic immune demyelinating polyradiculoneuropathyCarcinomatous neuromyopathyCastleman disease with IgM-associated neuropathyChronic immune demyelinating polyradiculoneuropathyDADS - [Distal acquired demyelinating neuropathy]Denny BrownDistal acquired demyelinating neuropathyIgG, IgA or IgM-associated osteosclerotic myeloma with polyneuropathy organomegaly endocrinopathy M-spike and skin changesLimited autoimmune autonomic neuropathyLimited autoimmune autonomic neuropathy, ganglionic acetylcholine receptor antibody positiveLimited autoimmune autonomic neuropathy, neural autoantibody negativeLimited autoimmune autonomic neuropathy, neural autoantibody positiveMAG - [Myelin-associated glycoprotein]MGUS - [monoclonal gammopathy of undetermined significance] associated neuropathiesMonoclonal gammopathy of undetermined significance-associated neuropathiesMultiple mononeuropathy vasculitis with Churg-Strauss syndromeMultiple mononeuropathy vasculitis with Wegener granulomatosisMultiple mononeuropathy vasculitis with mixed cryoglobulinemiaMultiple mononeuropathy vasculitis with perinuclear antineutrophil cytoplasmic antibody-associated vasculitisMyelin-associated glycoproteinNerve restricted multiple mononeuropathy vasculitisNon-systemic multiple mononeuropathy vasculitisNondiabetic radiculoplexoneuropathyPandysautonomiaPandysautonomia neural autoantibody negativePandysautonomia neural autoantibody positiveParaneoplastic autonomic intussusceptionParaneoplastic autonomic neuropathyParaneoplastic disorders of the spinal cordParaneoplastic motor neuropathyParaneoplastic motor neuropathy, neural autoantibody negativeParaneoplastic neuromyopathy and neuropathyParaneoplastic neuropathy of unspecified siteParaneoplastic or autoimmune disorders of the peripheral or autonomic nervous systemParaneoplastic sensorimotor neuropathyParaneoplastic sensorimotor neuropathy, neural autoantibody negativeParaneoplastic sensorimotor neuropathy, neural autoantibody positiveParaneoplastic sensory ganglionopathyParaneoplastic sensory ganglionopathy, neural autoantibody negativeParaneoplastic sensory neuronopathyParaneoplastic sensory neuropathyParaneoplastic sensory neuropathy, neural autoantibody negativeParaneoplastic sensory neuropathy, neural autoantibody positivePost surgical brachial plexitisPost surgical lumbosacral plexitisPost surgical plexitisPostsurgical plexitisRadiculoplexoneuropathySensorial paraneoplastic neuropathy [Denny Brown]Systemic multiple mononeuropathy vasculitisVasculitis or vasculitic neuropathyautoimmune autonomic ganglionopathyautoimmune dysautonomiaautoimmune pure autonomic failurecarcinomatous neuropathy

Nearby in Paraneoplastic or autoimmune disorders of the nervous system