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.
Index Terms
Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous systemParaneoplastic sensory ganglionopathyParaneoplastic sensory neuronopathyParaneoplastic sensory ganglionopathy, neural autoantibody negativeAutoimmune sensory ganglionopathyAutoimmune sensory ganglionopathy, neural autoantibody negativeAutoimmune sensory ganglionopathy, neural autoantibody positiveAutoimmune neuritisAutoimmune brachial neuritisAutoimmune radiculoplexoneuropathyRadiculoplexoneuropathyNondiabetic radiculoplexoneuropathyChronic immune demyelinating polyradiculoneuropathyBrachial variant of chronic immune demyelinating polyradiculoneuropathyPost surgical plexitisPostsurgical plexitisPost surgical brachial plexitisPost surgical lumbosacral plexitisParaneoplastic sensorimotor neuropathyParaneoplastic sensorimotor neuropathy, neural autoantibody negativeParaneoplastic sensorimotor neuropathy, neural autoantibody positiveVasculitis or vasculitic neuropathySystemic multiple mononeuropathy vasculitisMultiple mononeuropathy vasculitis with mixed cryoglobulinemiaMultiple mononeuropathy vasculitis with perinuclear antineutrophil cytoplasmic antibody-associated vasculitisMultiple mononeuropathy vasculitis with Churg-Strauss syndromeMultiple mononeuropathy vasculitis with Wegener granulomatosisNon-systemic multiple mononeuropathy vasculitisNerve restricted multiple mononeuropathy vasculitisAutoimmune sensorimotor neuropathyAutoimmune sensorimotor neuropathy, neural autoantibody negativeAutoimmune sensorimotor neuropathy, neural autoantibody positiveAutoimmune autonomic neuropathyautoimmune autonomic ganglionopathyautoimmune pure autonomic failureautoimmune dysautonomiaPandysautonomiaPandysautonomia neural autoantibody positivePandysautonomia neural autoantibody negativeAutoimmune autonomic neuropathy, ganglionic acetylcholine receptor antibody positiveAutoimmune autonomic neuropathy, other neural autoantibody positiveAutoimmune autonomic neuropathy, neural autoantibody negativeLimited autoimmune autonomic neuropathyLimited autoimmune autonomic neuropathy, ganglionic acetylcholine receptor antibody positiveLimited autoimmune autonomic neuropathy, neural autoantibody positiveLimited autoimmune autonomic neuropathy, neural autoantibody negativeParaneoplastic autonomic intussusceptionAutoimmune acquired autonomic encephalomyelitisParaneoplastic sensory neuropathyDenny BrownParaneoplastic sensory neuropathy, neural autoantibody negativeParaneoplastic sensory neuropathy, neural autoantibody positiveAutoimmune sensory neuropathyAutoimmune sensory neuropathy, neural autoantibody negativeAutoimmune sensory neuropathy, neural autoantibody positiveParaneoplastic motor neuropathyParaneoplastic motor neuropathy, neural autoantibody negativeAutoimmune motor neuropathyAutoimmune motor neuropathy, neural autoantibody negativeAutoimmune motor neuropathy, neural autoantibody positiveMonoclonal gammopathy of undetermined significance-associated neuropathiesMGUS - [monoclonal gammopathy of undetermined significance] associated neuropathiesCastleman disease with IgM-associated neuropathyMyelin-associated glycoproteinMAG - [Myelin-associated glycoprotein]Distal acquired demyelinating neuropathyDADS - [Distal acquired demyelinating neuropathy]IgG, IgA or IgM-associated osteosclerotic myeloma with polyneuropathy organomegaly endocrinopathy M-spike and skin changesParaneoplastic neuromyopathy and neuropathyCarcinomatous neuromyopathycarcinomatous neuropathySensorial paraneoplastic neuropathy [Denny Brown]Paraneoplastic disorders of the spinal cordParaneoplastic autonomic neuropathyParaneoplastic neuropathy of unspecified site