Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord (Q41477): Difference between revisions
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Transtornos paraneoplásicos e autoimunes do sistema nervoso central, cérebro e sistema nervoso da medula espinal resultam de um ataque imune direcionado a neurônios ou células gliais no sistema nervoso central (p.ex., encefalopatia, ataxia, mielopatia, mielite). 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. Esses transtornos são comumente multifocais causando lesões e sintomas decorrentes do envolvimento em vários níveis do sistema nervoso central. Uma história pessoal ou familiar de autoimunidade é frequentemente encontrada. Podem ser encontrados autoanticorpos neurais e anticorpos não órgão-específicos (anticorpos peroxidase da tireoide [TPO]). O perfil de autoanticorpos neurais pode ser preditivo de um tipo específico de câncer e pode estar associado a um fenótipo neurológico particular. A exclusão de etiologias alternativas (p.ex., infecções) é importante. A resposta a imunoterapia pode apoiar o diagnóstico. | |||
| description / en | description / en | ||
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. | |||
Revision as of 07:39, 13 August 2026
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.
| Language | Label | Description | Also known as |
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| default for all languages | 8E4A.0 |
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| English | Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord |
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. |
