{"entities":{"Q41473":{"pageid":31841,"ns":120,"title":"Item:Q41473","lastrevid":138476,"modified":"2026-08-13T07:39:29Z","type":"item","id":"Q41473","labels":{"mul":{"language":"mul","value":"8E4A.2"},"pt-br":{"language":"pt-br","value":"Transtornos de transmiss\u00e3o neuromuscular paraneopl\u00e1sicos ou autoimunes"},"en":{"language":"en","value":"Paraneoplastic or autoimmune neuromuscular transmission disorders"}},"descriptions":{"pt-br":{"language":"pt-br","value":"Os transtornos da transmiss\u00e3o neuromuscular s\u00e3o definidos por um dist\u00farbio vari\u00e1vel da fun\u00e7\u00e3o da transmiss\u00e3o neuromuscular, resultando em fraqueza muscular flutuante e fadiga. Geralmente s\u00e3o classificados em transtornos pr\u00e9 e p\u00f3s-sin\u00e1pticos._x000D_ _x000D_ Os transtornos pr\u00e9-sin\u00e1pticos, principalmente a s\u00edndrome miast\u00eanica de Lambert Eaton (LEMS), est\u00e3o associados a anticorpos direcionados aos canais de c\u00e1lcio dependentes de voltagem (tipo PQ e N). LEMS est\u00e1 associada a malignidade (carcinoma de pequenas c\u00e9lulas pulmonar ou extrapulmonar) em cerca de 50% dos casos. No contexto de LEMS, a detec\u00e7\u00e3o de anticorpos SOX 1 (anti-nucleares gliais) \u00e9 altamente preditiva de c\u00e2ncer._x000D_ _x000D_ P\u00f3s-sin\u00e1ptica, a miastenia gravis est\u00e1 principalmente (> 90%) associada a anticorpos que visam o receptor de acetilcolina muscular (AChR) ou raramente outras prote\u00ednas (incluindo a quinase espec\u00edfica do m\u00fasculo - MuSK). A miastenia geralmente n\u00e3o \u00e9 considerada uma doen\u00e7a paraneopl\u00e1sica, com exce\u00e7\u00e3o do timoma em cerca de 10% dos casos."},"en":{"language":"en","value":"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."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"87d29c2e1dc135f3aae18f4b8633ea651a933561","datavalue":{"value":"https://id.who.int/icd/entity/1381491944","type":"string"},"datatype":"url"},"type":"statement","id":"Q41473$A825CDC3-A878-4385-B952-1F3484F35F39","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"88e27f2e66739dd9c4ace9a1c42fc0a7580ce616","datavalue":{"value":"CID11:8E4A.2","type":"string"},"datatype":"string"},"type":"statement","id":"Q41473$36F3DB27-11B3-45B8-B8F3-5410571B0567","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"c71b7ce54cbda69bd035487a0cd842ef7b5d2367","datavalue":{"value":"dki-india-8E4A.2","type":"string"},"datatype":"string"},"type":"statement","id":"Q41473$682205F8-A369-4882-80FB-52A640953A15","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q41473$69996EA7-1559-41BE-AFC3-5AE652018331","rank":"normal"}],"P78":[{"mainsnak":{"snaktype":"value","property":"P78","hash":"5f9b5be68b7400da549761cf15abfdbc4d1e075b","datavalue":{"value":"https://pauloleads.com.br/cases-publicos/","type":"string"},"datatype":"url"},"type":"statement","id":"Q41473$A1C7FBF4-9058-4FAB-B9F3-B0132ACF0B5A","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"92b8222fcbb75718504f915073a3f43bd4003d18","datavalue":{"value":{"time":"+2026-08-13T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q41473$6FAD4FED-1891-4ABD-A09D-4E8D595E8CF5","rank":"normal"}],"P89":[{"mainsnak":{"snaktype":"value","property":"P89","hash":"cecdf419f8b7fdd59e37e32c354a44d164d40346","datavalue":{"value":{"entity-type":"item","numeric-id":41472,"id":"Q41472"},"type":"wikibase-entityid"},"datatype":"wikibase-item"},"type":"statement","id":"Q41473$1E042FA6-8DA7-41D2-AA65-82195435A4D3","rank":"normal"}]},"sitelinks":{}}}}