Paraneoplastic or autoimmune neuromuscular transmission disorders (Q41473): Difference between revisions
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Os transtornos da transmissão neuromuscular são definidos por um distúrbio variável da função da transmissão neuromuscular, resultando em fraqueza muscular flutuante e fadiga. Geralmente são classificados em transtornos pré e pós-sinápticos._x000D_ _x000D_ Os transtornos pré-sinápticos, principalmente a síndrome miastênica de Lambert Eaton (LEMS), estão associados a anticorpos direcionados aos canais de cálcio dependentes de voltagem (tipo PQ e N). LEMS está associada a malignidade (carcinoma de pequenas células pulmonar ou extrapulmonar) em cerca de 50% dos casos. No contexto de LEMS, a detecção de anticorpos SOX 1 (anti-nucleares gliais) é altamente preditiva de câncer._x000D_ _x000D_ Pós-sináptica, a miastenia gravis está principalmente (> 90%) associada a anticorpos que visam o receptor de acetilcolina muscular (AChR) ou raramente outras proteínas (incluindo a quinase específica do músculo - MuSK). A miastenia geralmente não é considerada uma doença paraneoplásica, com exceção do timoma em cerca de 10% dos casos. | |||||||||||||||
| description / en | description / en | ||||||||||||||
NMT disorders are defined by a variable disturbance of the function of the neuromuscular transmission, resulting in fluctuating muscle weakness and fatigue. These are usually classified into pre- and postsynaptic disorders. Presynaptic disorders, mainly the Lambert Eaton Myasthenic Syndrome (LEMS) is associated with antibodies targeting the voltage gated calcium channels (PQ and N type). LEMS is associated with malignancy (pulmonary or extra-pulmonary small cell carcinoma) in about 50% of cases. In the context of LEMS the detection of SOX 1 (anti glial nuclear) antibodies is highly predictive of cancer. Postsynaptically, myasthenia gravis is mostly (> 90%) associated with antibodies targeting the muscle acetylcholine receptor (AChR) or rarely other proteins (including muscle -specific kinase -MuSK). Myasthenia is usually not considered a paraneoplastic disease, with the exception of thymoma in about 10% cases. | |||||||||||||||
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CID11:8E4A.2 | |||||||||||||||
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dki-india-8E4A.2 | |||||||||||||||
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| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
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13 August 2026
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| Property / Collection date: 13 August 2026 / rank | |||||||||||||||
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| Property / Linked ICD 10: G90-G99 / rank | |||||||||||||||
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Latest revision as of 07:39, 13 August 2026
NMT disorders are defined by a variable disturbance of the function of the neuromuscular transmission, resulting in fluctuating muscle weakness and fatigue. These are usually classified into pre- and postsynaptic disorders. Presynaptic disorders, mainly the Lambert Eaton Myasthenic Syndrome (LEMS) is associated with antibodies targeting the voltage gated calcium channels (PQ and N type). LEMS is associated with malignancy (pulmonary or extra-pulmonary small cell carcinoma) in about 50% of cases. In the context of LEMS the detection of SOX 1 (anti glial nuclear) antibodies is highly predictive of cancer. Postsynaptically, myasthenia gravis is mostly (> 90%) associated with antibodies targeting the muscle acetylcholine receptor (AChR) or rarely other proteins (including muscle -specific kinase -MuSK). Myasthenia is usually not considered a paraneoplastic disease, with the exception of thymoma in about 10% cases.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 8E4A.2 |
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| English | Paraneoplastic or autoimmune neuromuscular transmission disorders |
NMT disorders are defined by a variable disturbance of the function of the neuromuscular transmission, resulting in fluctuating muscle weakness and fatigue. These are usually classified into pre- and postsynaptic disorders. Presynaptic disorders, mainly the Lambert Eaton Myasthenic Syndrome (LEMS) is associated with antibodies targeting the voltage gated calcium channels (PQ and N type). LEMS is associated with malignancy (pulmonary or extra-pulmonary small cell carcinoma) in about 50% of cases. In the context of LEMS the detection of SOX 1 (anti glial nuclear) antibodies is highly predictive of cancer. Postsynaptically, myasthenia gravis is mostly (> 90%) associated with antibodies targeting the muscle acetylcholine receptor (AChR) or rarely other proteins (including muscle -specific kinase -MuSK). Myasthenia is usually not considered a paraneoplastic disease, with the exception of thymoma in about 10% cases. |
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CID11:8E4A.2
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dki-india-8E4A.2
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Concluído
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13 August 2026
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