{"entities":{"Q41053":{"pageid":31421,"ns":120,"title":"Item:Q41053","lastrevid":134951,"modified":"2026-08-13T07:06:35Z","type":"item","id":"Q41053","labels":{"mul":{"language":"mul","value":"8B60.0"},"pt-br":{"language":"pt-br","value":"Esclerose lateral amiotr\u00f3fica"},"en":{"language":"en","value":"Amyotrophic lateral sclerosis"}},"descriptions":{"pt-br":{"language":"pt-br","value":"A esclerose lateral amiotr\u00f3fica (ELA) \u00e9 um transtorno progressivo e fatal em que sinais progressivos de NMI e degenera\u00e7\u00e3o NMS s\u00e3o vistos em uma ou mais das quatro regi\u00f5es: bulbar, cervical, tor\u00e1cica e lombossacra. Estudos eletrofisiol\u00f3gicos podem ser necess\u00e1rios para confirmar a degenera\u00e7\u00e3o do neur\u00f4nio motor inferior e para excluir causas alternativas. A neuroimagem pode ser realizada para excluir outras causas, o que pode explicar as caracter\u00edsticas cl\u00ednicas e eletrofisiol\u00f3gicas. ELA familiar de heran\u00e7a autoss\u00f4mica dominante, constitui 5 a 10% de ALS. O perfil cl\u00ednico de ELA familiar e ELA espor\u00e1dico \u00e9 semelhante. Muta\u00e7\u00f5es nos genes C9ORF72 e SOD1 (muta\u00e7\u00e3o da enzima Cu,Zn-super\u00f3xido dismutase) constituem 50-60% dos casos de ELA familiar."},"en":{"language":"en","value":"Amyotrophic lateral sclerosis (ALS) is a progressive, fatal disorder in which progressive signs of LMN and UMN degeneration are seen within one or more of the four regions: bulbar, cervical, thoracic and lumbosacral. Electrophysiological studies may be required to confirm lower motor neuron degeneration and to exclude alternative causes. Neuroimaging may be performed to exclude other causes, which might explain the clinical and electrophysiological features. Familial ALS (FALS) of autosomal dominant inheritance constitutes 5 to 10% of ALS. The clinical profile of FALS and sporadic ALS is similar. Mutations in the C9ORF72 and Cu/Zn superoxide dismutase (SOD1) genes constitute 50-60% of FALS."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"51cbcf7c676d34246f320e27f59dcacf9fc10aec","datavalue":{"value":"https://id.who.int/icd/entity/1982355687","type":"string"},"datatype":"url"},"type":"statement","id":"Q41053$974CA05E-6C2B-4385-B6E0-2F68AA25D0CE","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"7b313c3b66393c86ff7761ea85da06503955bc65","datavalue":{"value":"CID11:8B60.0","type":"string"},"datatype":"string"},"type":"statement","id":"Q41053$C7D6DA7A-0C96-4B2B-887F-A8EE605F0408","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"348d8415de1199ddd74a8b00bef1ee8b34c66c44","datavalue":{"value":"dki-india-8B60.0","type":"string"},"datatype":"string"},"type":"statement","id":"Q41053$01E06E9B-8A76-4908-9901-2F7D250C6DFE","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q41053$6461E835-82B6-4A2C-B171-0624E7099719","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":"Q41053$1D9110D3-5F0B-4FD3-99E5-2BBFC2B8B722","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"92b8222fcbb75718504f915073a3f43bd4003d18","datavalue":{"value":{"time":"+2026-08-13T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q41053$C30D5F40-D41D-4DDF-BC17-6B820A36EB53","rank":"normal"}],"P89":[{"mainsnak":{"snaktype":"value","property":"P89","hash":"ea31a01f219e449243632e6aea5d14e92c40b2c6","datavalue":{"value":{"entity-type":"item","numeric-id":16832,"id":"Q16832"},"type":"wikibase-entityid"},"datatype":"wikibase-item"},"type":"statement","id":"Q41053$20DF77A8-80C8-42D7-BB1C-4B0EA82B1346","rank":"normal"}]},"sitelinks":{}}}}