{"entities":{"Q95981":{"pageid":86374,"ns":120,"title":"Item:Q95981","lastrevid":540807,"modified":"2026-08-16T11:49:08Z","type":"item","id":"Q95981","labels":{"mul":{"language":"mul","value":"ID_730637493"},"pt-br":{"language":"pt-br","value":"Distrofia neuroaxonal infantil"},"en":{"language":"en","value":"Infantile neuroaxonal dystrophy"}},"descriptions":{"pt-br":{"language":"pt-br","value":"Distrofia neuroaxonal infantil (INAD) \u00e9 um dos tr\u00eas fen\u00f3tipos com caracter\u00edsticas cl\u00ednicas e radiol\u00f3gicas sobrepostas da neurodegenera\u00e7\u00e3o associada \u00e0 PLA2G6 (PLAN), uma variante da Neurodegenera\u00e7\u00e3o com ac\u00famulo de ferro no c\u00e9rebro (NBIA)._x000D_ A INAD geralmente come\u00e7a entre os seis meses e os tr\u00eas anos de idade, com regress\u00e3o ou atraso psicomotor, hipotonia e tetraparesia esp\u00e1stica progressiva. Muitas crian\u00e7as afetadas nunca aprendem a andar ou perdem a habilidade logo ap\u00f3s ao in\u00edcio da condi\u00e7\u00e3o. Estrabismo, nistagmo e atrofia \u00f3ptica s\u00e3o comuns. A progress\u00e3o da doen\u00e7a \u00e9 r\u00e1pida, resultando em espasticidade grave, decl\u00ednio cognitivo progressivo e comprometimento visual. Muitas crian\u00e7as afetadas n\u00e3o sobrevivem al\u00e9m da primeira d\u00e9cada de vida."},"en":{"language":"en","value":"Infantile neuroaxonal dystrophy (INAD) is one of the three phenotypes with overlapping clinical and radiologic features of PLA2G6-associated neurodegeneration (PLAN) a variant of Neurodegeneration with brain iron accumulation (NBIA). INAD usually begins between ages six months and three years with psychomotor regression or delay, hypotonia, and progressive spastic tetraparesis. Many affected children never learn to walk or lose the ability shortly after attaining it. Strabismus, nystagmus, and optic atrophy are common. Disease progression is rapid, resulting in severe spasticity, progressive cognitive decline, and visual impairment. Many affected children do not survive beyond their first decade."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"2dabac25d181b9450cbc9d5e01fafe83ab62b49e","datavalue":{"value":"https://id.who.int/icd/entity/730637493","type":"string"},"datatype":"url"},"type":"statement","id":"Q95981$E015320D-8D80-45AC-BAF5-D2773A7973AB","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"01677ca2e9b80acf52558e3da14eafbaf025b1d7","datavalue":{"value":"CID11:ID_730637493","type":"string"},"datatype":"string"},"type":"statement","id":"Q95981$5F051A21-7EB9-4967-8357-7E1B9FE97B67","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"211f7c25e17387c01152c6c09342fa09e55667be","datavalue":{"value":"dki-india-ID_730637493","type":"string"},"datatype":"string"},"type":"statement","id":"Q95981$CC522704-FFF3-4F03-8D10-4C4139DCA790","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q95981$87AB1914-D89D-4448-A761-BFC4E1EAED88","rank":"normal"}],"P78":[{"mainsnak":{"snaktype":"value","property":"P78","hash":"5f9b5be68b7400da549761cf15abfdbc4d1e075b","datavalue":{"value":"https://pauloleads.com.br/cases-publicos/","type":"string"},"datatype":"url"},"type":"statement","id":"Q95981$A4CBE726-E797-4FCF-9975-4AAAFF59A2A7","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"378ac503ed43f21e1f461d2823a6ad59da580514","datavalue":{"value":{"time":"+2026-08-15T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q95981$C6859287-C0B0-4221-9CA2-DA429154FC3F","rank":"normal"}]},"sitelinks":{}}}}