{"entities":{"Q41038":{"pageid":31406,"ns":120,"title":"Item:Q41038","lastrevid":134816,"modified":"2026-08-13T07:05:24Z","type":"item","id":"Q41038","labels":{"mul":{"language":"mul","value":"8B44.0"},"pt-br":{"language":"pt-br","value":"Paraplegia esp\u00e1stica heredit\u00e1ria"},"en":{"language":"en","value":"Hereditary spastic paraplegia"}},"descriptions":{"pt-br":{"language":"pt-br","value":"Paraplegias esp\u00e1sticas heredit\u00e1rias (PEH) compreendem um grupo genetica e clinicamente heterog\u00eaneo de transtornos neurodegenerativos caracterizados por graus vari\u00e1veis de espasticidade de membros inferiores, fraqueza piramidal, hiperreflexia e envolvimento da bexiga hipert\u00f4nica. Clinicamente, as PEH podem ser divididas em dois grupos principais: formas n\u00e3o complicadas (puras) e complicadas (complexas), dependendo da presen\u00e7a de outras manifesta\u00e7\u00f5es neurol\u00f3gicas, incluindo ataxia, neuropatia perif\u00e9rica, comprometimento cognitivo, epilepsia, amiotrofia, retinopatia, surdez, ictiose e envolvimento extrapiramidal , al\u00e9m de paraparesia esp\u00e1stica. As PEH puras s\u00e3o caracterizadas por espasticidade e fraqueza lentamente progressivas em extremidades inferiores, frequentemente associadas a dist\u00farbios urin\u00e1rios hipert\u00f4nicos, leve redu\u00e7\u00e3o da sensibilidade vibrat\u00f3ria de extremidades inferiores e, ocasionalmente, da sensa\u00e7\u00e3o de posi\u00e7\u00e3o articular. As formas complexas de PEH s\u00e3o caracterizadas pela presen\u00e7a de manifesta\u00e7\u00f5es neurol\u00f3gicas ou n\u00e3o neurol\u00f3gicas adicionais. Uma hist\u00f3ria familiar positiva, particularmente em casos autoss\u00f4micos dominantes, est\u00e1 frequentemente presente, mas nem sempre. O diagn\u00f3stico pode ser auxiliado por neuroimagem e testes gen\u00e9ticos."},"en":{"language":"en","value":"Hereditary spastic paraplegias (HSP) comprise a genetically and clinically heterogeneous group of neurodegenerative disorders characterised by varying degrees of lower limb spasticity, pyramidal weakness, hyperreflexia and hypertonic bladder involvement. Clinically, HSPs can be divided into two main groups: uncomplicated (pure) and complicated (complex) forms depending on the presence of other neurological features including ataxia, peripheral neuropathy, cognitive impairment, epilepsy, amyotrophy, retinopathy, deafness, ichthyosis and extrapyramidal involvement, in addition to spastic paraparesis. Pure HSPs are characterised by slowly progressive lower extremity spasticity and weakness, often associated with hypertonic urinary disturbances, mild reduction of lower extremity vibration sense and, occasionally, of joint position sensation. Complex HSP forms are characterised by the presence of additional neurological or non-neurological features. A positive family history particularly in autosomal dominant cases is often but not always present. The diagnosis may be aided by neuroimaging and genetic testing."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"05d0ac8fbeb7afdc24a7d21158e2dcd58f3b5ac1","datavalue":{"value":"https://id.who.int/icd/entity/810807375","type":"string"},"datatype":"url"},"type":"statement","id":"Q41038$8D2D4D6A-9A18-4791-A447-041431D951F5","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"e6db672af6106e30292c9404f0a2d10f69cfd7c3","datavalue":{"value":"CID11:8B44.0","type":"string"},"datatype":"string"},"type":"statement","id":"Q41038$1478A25C-4036-468D-8712-EC99A4948CD1","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"cf5c1842046c7f76dae0aff9aee167e330a439dc","datavalue":{"value":"dki-india-8B44.0","type":"string"},"datatype":"string"},"type":"statement","id":"Q41038$0E98DD61-E74F-4B48-9759-F2D9AD615476","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q41038$8D25AAC1-8487-4E0B-8137-53DA3EA2B584","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":"Q41038$7E71575C-E4EC-4171-A729-25A80E80FE20","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":"Q41038$9DD28BC4-4638-4024-ADEC-8329B98AC724","rank":"normal"}],"P89":[{"mainsnak":{"snaktype":"value","property":"P89","hash":"35bbeb0ab6b74529a8011991e38ac8036e0ec7c0","datavalue":{"value":{"entity-type":"item","numeric-id":16821,"id":"Q16821"},"type":"wikibase-entityid"},"datatype":"wikibase-item"},"type":"statement","id":"Q41038$2F5F02B2-0286-4F88-8D46-2CBB8AFDF22D","rank":"normal"}]},"sitelinks":{}}}}