Are cognitive changes in hereditary spastic paraplegias restricted to complicated forms?
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UFRGS |
Texto Completo: | http://hdl.handle.net/10183/204671 |
Resumo: | Background: Little is known about the cognitive profile of Hereditary Spastic Paraplegias (HSP), where most scientific attention has been given to motor features related to corticospinal tract degeneration. Objectives: We aimed to perform a broad characterization of the cognitive functions of patients with pure and complicated HSP as well as to determine the frequency of abnormal cognitive performances in the studied subtypes. Methods: A two-center cross-sectional case-control study was performed. All individuals underwent cognitive assessment through screening tests (Mini Mental State Examination—MEEM and Montreal Cognitive Assessment—MOCA) and tests to assess specific cognitive functions (Verbal fluency with phonological restriction—FAS; Verbal categorical fluency—FAS-cat and Rey's Verbal Auditory Learning Test -RAVLT). Results: Fifty four patients with genetically confirmed HSP diagnosis, 36 with spastic paraplegia type 4 (SPG4), 5 SPG11, 4 SPG5, 4 cerebrotendinous xanthomatosis (CTX), 3 SPG7 and 2 SPG3A, and 10 healthy, unrelated control subjects, with similar age, sex, and education participated in the study. SPG4 patients had worse performances in MOCA, FAS, FAS-cat, and RAVLT when compared to controls. Most SPG4 patients presented cognitive changes not compatible with dementia, performing poorly in memory, attention and executive functions. SPG5 patients scored lower in executive functions and memory, and SPG7 patients performed poorly on memory tasks. All evaluated cognitive functions were markedly altered in CTX and SPG11 patients. Conclusions: Cognitive abnormalities are frequent in HSP, being more severe in complicated forms. However, cognitive impairments of pure HSPs might impact patients' lives, decreasing families' socioeconomic status and contributing to the overall disease burden. |
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Jacinto, Lais AlvesMachado, Gustavo DarivaAyres, AnneliseBurguêz, DanielaBonatto, Márcia PoleseGonzález Salazar, Carelis Del ValleSiebert, MarinaFrança Júnior, Marcondes CavalcanteOlchik, Maira RozenfeldSaute, Jonas Alex Morales2020-01-21T04:15:26Z20191664-2295http://hdl.handle.net/10183/204671001109732Background: Little is known about the cognitive profile of Hereditary Spastic Paraplegias (HSP), where most scientific attention has been given to motor features related to corticospinal tract degeneration. Objectives: We aimed to perform a broad characterization of the cognitive functions of patients with pure and complicated HSP as well as to determine the frequency of abnormal cognitive performances in the studied subtypes. Methods: A two-center cross-sectional case-control study was performed. All individuals underwent cognitive assessment through screening tests (Mini Mental State Examination—MEEM and Montreal Cognitive Assessment—MOCA) and tests to assess specific cognitive functions (Verbal fluency with phonological restriction—FAS; Verbal categorical fluency—FAS-cat and Rey's Verbal Auditory Learning Test -RAVLT). Results: Fifty four patients with genetically confirmed HSP diagnosis, 36 with spastic paraplegia type 4 (SPG4), 5 SPG11, 4 SPG5, 4 cerebrotendinous xanthomatosis (CTX), 3 SPG7 and 2 SPG3A, and 10 healthy, unrelated control subjects, with similar age, sex, and education participated in the study. SPG4 patients had worse performances in MOCA, FAS, FAS-cat, and RAVLT when compared to controls. Most SPG4 patients presented cognitive changes not compatible with dementia, performing poorly in memory, attention and executive functions. SPG5 patients scored lower in executive functions and memory, and SPG7 patients performed poorly on memory tasks. All evaluated cognitive functions were markedly altered in CTX and SPG11 patients. Conclusions: Cognitive abnormalities are frequent in HSP, being more severe in complicated forms. However, cognitive impairments of pure HSPs might impact patients' lives, decreasing families' socioeconomic status and contributing to the overall disease burden.application/pdfengFrontiers in neurology. Lausanne. vol. 10 (May 2019), 508, 7 f.CogniçãoParaplegia espástica hereditáriaHereditary spastic paraplegiaHSPSPGCognitive profileMemoryExecutive functionAre cognitive changes in hereditary spastic paraplegias restricted to complicated forms?Estrangeiroinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFRGSinstname:Universidade Federal do Rio Grande do Sul (UFRGS)instacron:UFRGSTEXT001109732.pdf.txt001109732.pdf.txtExtracted Texttext/plain35261http://www.lume.ufrgs.br/bitstream/10183/204671/2/001109732.pdf.txt2ff5dcc68d26b25e131ff1f96ce820f1MD52ORIGINAL001109732.pdfTexto completo (inglês)application/pdf718659http://www.lume.ufrgs.br/bitstream/10183/204671/1/001109732.pdfa4ee60c88168b8d89680b0dcb5bfb1c5MD5110183/2046712020-11-29 05:10:46.678432oai:www.lume.ufrgs.br:10183/204671Repositório de PublicaçõesPUBhttps://lume.ufrgs.br/oai/requestopendoar:2020-11-29T07:10:46Repositório Institucional da UFRGS - Universidade Federal do Rio Grande do Sul (UFRGS)false |
dc.title.pt_BR.fl_str_mv |
Are cognitive changes in hereditary spastic paraplegias restricted to complicated forms? |
title |
Are cognitive changes in hereditary spastic paraplegias restricted to complicated forms? |
spellingShingle |
Are cognitive changes in hereditary spastic paraplegias restricted to complicated forms? Jacinto, Lais Alves Cognição Paraplegia espástica hereditária Hereditary spastic paraplegia HSP SPG Cognitive profile Memory Executive function |
title_short |
Are cognitive changes in hereditary spastic paraplegias restricted to complicated forms? |
title_full |
Are cognitive changes in hereditary spastic paraplegias restricted to complicated forms? |
title_fullStr |
Are cognitive changes in hereditary spastic paraplegias restricted to complicated forms? |
title_full_unstemmed |
Are cognitive changes in hereditary spastic paraplegias restricted to complicated forms? |
title_sort |
Are cognitive changes in hereditary spastic paraplegias restricted to complicated forms? |
author |
Jacinto, Lais Alves |
author_facet |
Jacinto, Lais Alves Machado, Gustavo Dariva Ayres, Annelise Burguêz, Daniela Bonatto, Márcia Polese González Salazar, Carelis Del Valle Siebert, Marina França Júnior, Marcondes Cavalcante Olchik, Maira Rozenfeld Saute, Jonas Alex Morales |
author_role |
author |
author2 |
Machado, Gustavo Dariva Ayres, Annelise Burguêz, Daniela Bonatto, Márcia Polese González Salazar, Carelis Del Valle Siebert, Marina França Júnior, Marcondes Cavalcante Olchik, Maira Rozenfeld Saute, Jonas Alex Morales |
author2_role |
author author author author author author author author author |
dc.contributor.author.fl_str_mv |
Jacinto, Lais Alves Machado, Gustavo Dariva Ayres, Annelise Burguêz, Daniela Bonatto, Márcia Polese González Salazar, Carelis Del Valle Siebert, Marina França Júnior, Marcondes Cavalcante Olchik, Maira Rozenfeld Saute, Jonas Alex Morales |
dc.subject.por.fl_str_mv |
Cognição Paraplegia espástica hereditária |
topic |
Cognição Paraplegia espástica hereditária Hereditary spastic paraplegia HSP SPG Cognitive profile Memory Executive function |
dc.subject.eng.fl_str_mv |
Hereditary spastic paraplegia HSP SPG Cognitive profile Memory Executive function |
description |
Background: Little is known about the cognitive profile of Hereditary Spastic Paraplegias (HSP), where most scientific attention has been given to motor features related to corticospinal tract degeneration. Objectives: We aimed to perform a broad characterization of the cognitive functions of patients with pure and complicated HSP as well as to determine the frequency of abnormal cognitive performances in the studied subtypes. Methods: A two-center cross-sectional case-control study was performed. All individuals underwent cognitive assessment through screening tests (Mini Mental State Examination—MEEM and Montreal Cognitive Assessment—MOCA) and tests to assess specific cognitive functions (Verbal fluency with phonological restriction—FAS; Verbal categorical fluency—FAS-cat and Rey's Verbal Auditory Learning Test -RAVLT). Results: Fifty four patients with genetically confirmed HSP diagnosis, 36 with spastic paraplegia type 4 (SPG4), 5 SPG11, 4 SPG5, 4 cerebrotendinous xanthomatosis (CTX), 3 SPG7 and 2 SPG3A, and 10 healthy, unrelated control subjects, with similar age, sex, and education participated in the study. SPG4 patients had worse performances in MOCA, FAS, FAS-cat, and RAVLT when compared to controls. Most SPG4 patients presented cognitive changes not compatible with dementia, performing poorly in memory, attention and executive functions. SPG5 patients scored lower in executive functions and memory, and SPG7 patients performed poorly on memory tasks. All evaluated cognitive functions were markedly altered in CTX and SPG11 patients. Conclusions: Cognitive abnormalities are frequent in HSP, being more severe in complicated forms. However, cognitive impairments of pure HSPs might impact patients' lives, decreasing families' socioeconomic status and contributing to the overall disease burden. |
publishDate |
2019 |
dc.date.issued.fl_str_mv |
2019 |
dc.date.accessioned.fl_str_mv |
2020-01-21T04:15:26Z |
dc.type.driver.fl_str_mv |
Estrangeiro info:eu-repo/semantics/article |
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info:eu-repo/semantics/publishedVersion |
format |
article |
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publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10183/204671 |
dc.identifier.issn.pt_BR.fl_str_mv |
1664-2295 |
dc.identifier.nrb.pt_BR.fl_str_mv |
001109732 |
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1664-2295 001109732 |
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http://hdl.handle.net/10183/204671 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.ispartof.pt_BR.fl_str_mv |
Frontiers in neurology. Lausanne. vol. 10 (May 2019), 508, 7 f. |
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info:eu-repo/semantics/openAccess |
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openAccess |
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