Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias
ABSTRACT Hereditary spastic paraplegias (HSP) are a group of genetic diseases characterized by lower limb spasticity with or without additional neurological features. Swallowing dysfunction is poorly studied in HSP and its presence can lead to significant respiratory and nutritional complications....
| Main Authors: | , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Academia Brasileira de Neurologia (ABNEURO)
2020-01-01
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| Series: | Arquivos de Neuro-Psiquiatria |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2019001200843&tlng=en |
| _version_ | 1819005653544337408 |
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| author | Laís Alves Jacinto-Scudeiro Gustavo Dariva Machado Annelise Ayres Daniela Burguêz Marcia Polese-Bonatto Carelis González-Salazar Marina Siebert Marcondes Cavalcante França Junior Maira Rozenfeld Olchik Jonas Alex Morales Saute |
| author_facet | Laís Alves Jacinto-Scudeiro Gustavo Dariva Machado Annelise Ayres Daniela Burguêz Marcia Polese-Bonatto Carelis González-Salazar Marina Siebert Marcondes Cavalcante França Junior Maira Rozenfeld Olchik Jonas Alex Morales Saute |
| author_sort | Laís Alves Jacinto-Scudeiro |
| collection | DOAJ |
| description | ABSTRACT Hereditary spastic paraplegias (HSP) are a group of genetic diseases characterized by lower limb spasticity with or without additional neurological features. Swallowing dysfunction is poorly studied in HSP and its presence can lead to significant respiratory and nutritional complications. Objectives: The aim of this study was to evaluate the frequency and clinical characteristics of dysphagia in different types of HSP. Methods: A two-center cross-sectional prevalence study was performed. Genetically confirmed HSP patients were evaluated using the Northwestern Dysphagia Patient Check Sheet and the Functional Oral Intake Scale. In addition, self-perception of dysphagia was assessed by the Eat Assessment Tool-10 and the Swallowing Disturbance Questionnaire. Results: Thirty-six patients with spastic paraplegia type 4 (SPG4), five with SPG11, four with SPG5, four with cerebrotendinous xanthomatosis (CTX), three with SPG7, and two with SPG3A were evaluated. Mild to moderate oropharyngeal dysphagia was present in 3/5 (60%) of SPG11 and 2/4 (50%) of CTX patients. A single SPG4 (2%) and a single SPG7 (33%) patient had mild oropharyngeal dysphagia. All other evaluated patients presented with normal or functional swallowing. Conclusions: Clinically significant oropharyngeal dysphagia was only present in complicated forms of HSP Patients with SPG11 and CTX had the highest risks for dysphagia, suggesting that surveillance of swallowing function should be part of the management of patients with these disorders. |
| first_indexed | 2024-12-20T23:56:13Z |
| format | Article |
| id | doaj.art-e241ffc7ddaa49a2a346b33ea69c4e40 |
| institution | Directory Open Access Journal |
| issn | 1678-4227 |
| language | English |
| last_indexed | 2024-12-20T23:56:13Z |
| publishDate | 2020-01-01 |
| publisher | Academia Brasileira de Neurologia (ABNEURO) |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj.art-e241ffc7ddaa49a2a346b33ea69c4e402022-12-21T19:22:44ZengAcademia Brasileira de Neurologia (ABNEURO)Arquivos de Neuro-Psiquiatria1678-42272020-01-01771284384710.1590/0004-282x20190180Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegiasLaís Alves Jacinto-Scudeirohttps://orcid.org/0000-0003-2567-5322Gustavo Dariva Machadohttps://orcid.org/0000-0003-3388-3666Annelise Ayreshttps://orcid.org/0000-0003-3205-3660Daniela BurguêzMarcia Polese-Bonattohttps://orcid.org/0000-0001-6049-926XCarelis González-Salazarhttps://orcid.org/0000-0003-2300-682XMarina Sieberthttps://orcid.org/0000-0003-3366-2177Marcondes Cavalcante França Juniorhttps://orcid.org/0000-0003-0898-2419Maira Rozenfeld Olchikhttps://orcid.org/0000-0002-8732-9225Jonas Alex Morales Sautehttps://orcid.org/0000-0003-1141-6573ABSTRACT Hereditary spastic paraplegias (HSP) are a group of genetic diseases characterized by lower limb spasticity with or without additional neurological features. Swallowing dysfunction is poorly studied in HSP and its presence can lead to significant respiratory and nutritional complications. Objectives: The aim of this study was to evaluate the frequency and clinical characteristics of dysphagia in different types of HSP. Methods: A two-center cross-sectional prevalence study was performed. Genetically confirmed HSP patients were evaluated using the Northwestern Dysphagia Patient Check Sheet and the Functional Oral Intake Scale. In addition, self-perception of dysphagia was assessed by the Eat Assessment Tool-10 and the Swallowing Disturbance Questionnaire. Results: Thirty-six patients with spastic paraplegia type 4 (SPG4), five with SPG11, four with SPG5, four with cerebrotendinous xanthomatosis (CTX), three with SPG7, and two with SPG3A were evaluated. Mild to moderate oropharyngeal dysphagia was present in 3/5 (60%) of SPG11 and 2/4 (50%) of CTX patients. A single SPG4 (2%) and a single SPG7 (33%) patient had mild oropharyngeal dysphagia. All other evaluated patients presented with normal or functional swallowing. Conclusions: Clinically significant oropharyngeal dysphagia was only present in complicated forms of HSP Patients with SPG11 and CTX had the highest risks for dysphagia, suggesting that surveillance of swallowing function should be part of the management of patients with these disorders.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2019001200843&tlng=enSpastic paraplegia, hereditarydeglutitionxanthomatosis, cerebrotendinousdeglutition disorders |
| spellingShingle | Laís Alves Jacinto-Scudeiro Gustavo Dariva Machado Annelise Ayres Daniela Burguêz Marcia Polese-Bonatto Carelis González-Salazar Marina Siebert Marcondes Cavalcante França Junior Maira Rozenfeld Olchik Jonas Alex Morales Saute Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias Arquivos de Neuro-Psiquiatria Spastic paraplegia, hereditary deglutition xanthomatosis, cerebrotendinous deglutition disorders |
| title | Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias |
| title_full | Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias |
| title_fullStr | Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias |
| title_full_unstemmed | Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias |
| title_short | Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias |
| title_sort | prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias |
| topic | Spastic paraplegia, hereditary deglutition xanthomatosis, cerebrotendinous deglutition disorders |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2019001200843&tlng=en |
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