Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous system (Q41479): Difference between revisions
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Transtornos paraneoplásicos e autoimunes do sistema nervoso periférico e autônomo resultam de um ataque imune direcionado a neurônios ou células gliais do sistema nervoso periférico (neuropatias periféricas ou autonômicas). No contexto paraneoplásico, esse ataque é uma consequência de uma resposta imune potencialmente eficaz ao tumor iniciada por antígenos onconeurais derivados de um câncer sistêmico. No contexto não paraneoplásico denominado autoimune, a etiologia permanece indefinida, embora evidências crescentes indiquem um gatilho infeccioso anterior em pelo menos alguns casos. O início pode ser subagudo ou insidioso e esses transtornos podem ser limitados, multifocais ou generalizados. Os transtornos nervosos periféricos somáticos autoimunes podem afetar o nervo em vários níveis, incluindo raiz, nervo e plexo e podem ser axonais, desmielinizantes ou ambos. Os transtornos autonômicos autoimunes resultam em insuficiência autonômica que pode ser parcial ou generalizada. A neuropatia autonômica prototípica é a ganglionopatia autoimune associada a anticorpos direcionados ao receptor nicotínico ganglionar de acetilcolina (α3 gAChR). Rastreamento de câncer é apropriado para aqueles com perfis específicos de anticorpos neurais e outros fatores de risco._x000D_ _x000D_ Anticorpos neurais associados em neuropatias periféricas incluem anticorpos anti-núcleo nervoso tipo 1 ou anti-Hu, CASPR2, gAChR, gangliosídeo (GM1 IgG e IGM). | |||
| description / en | description / en | ||
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. | |||
Revision as of 07:39, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 8E4A.1 |
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| English | Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous system |
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. |
