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The role of gastrostomy and noninvasive ventilation in primary lateral sclerosis

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dc.contributor.authorOliveira Santos, Miguel
dc.contributor.authorDomingues, Sara
dc.contributor.authorSimão, Sara
dc.contributor.authorGromicho, Marta
dc.contributor.authorAlves, Inês
dc.contributor.authorCarvalho, Mamede
dc.date.accessioned2025-02-11T14:36:28Z
dc.date.available2025-02-11T14:36:28Z
dc.date.issued2025
dc.description© 2025 Wiley Periodicals LLC.pt_PT
dc.description.abstractIntroduction/aims: Literature on the role of gastrostomy and noninvasive ventilation (NIV) in primary lateral sclerosis (PLS) is limited. We aim to investigate whether PLS patients develop dysphagia requiring feeding tubes or respiratory failure necessitating NIV. Methods: We conducted a retrospective study of PLS patients with a definite diagnosis followed at our center (1994-2024). Patients with marked dysphagia (score < 3 on Question 3 of the ALSFRS-R) received a recommendation for gastrostomy and were divided into two groups: G1/G2 (accepted/declined gastrostomy). We investigated NIV indications due to respiratory failure and compared these patients (G3) to those without respiratory impairment (G4). Demographic, clinical, and neurophysiological data were collected and compared. Results: Forty-eight patients had a definite diagnosis of PLS. Gastrostomy was recommended to 18 (37.5%), yet only 7 patients (38.9%-G1) consented. The median time to gastrostomy was 77 months. Total survival and survival post-gastrostomy recommendation were not different between G1 and G2. Six PLS patients (12.5%-G3) developed respiratory failure and initiated NIV (median of 63 months). At 63 months, G3 had significantly lower median forced vital capacity (65% vs. 99%; p < 0.001) and phrenic nerve amplitude (0.43 vs. 0.75 mV; p = 0.039), but a greater ALSFRS-R slope (0.34 vs. 0.14; p = 0.046) and shorter survival (35 vs. 94.9 months; p = 0.009) compared to G4. Discussion: Dysphagia requiring gastrostomy was common in our PLS cohort, but survival after gastrostomy recommendation did not differ between groups. Patients who developed respiratory impairment may represent a distinct group with faster disease progression and shorter survival. Our findings may contribute to a deeper understanding and improved management of PLS.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationMuscle Nerve. 2025 Mar;71(3):450-456pt_PT
dc.identifier.doi10.1002/mus.28346pt_PT
dc.identifier.eissn1097-4598
dc.identifier.issn0148-639X
dc.identifier.urihttp://hdl.handle.net/10400.5/98327
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherWileypt_PT
dc.relation.publisherversionhttps://onlinelibrary.wiley.com/journal/10974598pt_PT
dc.subjectAmyotrophic lateral sclerosispt_PT
dc.subjectGastrostomypt_PT
dc.subjectNoninvasive ventilationpt_PT
dc.subjectPrimary lateral sclerosispt_PT
dc.subjectPrognosispt_PT
dc.titleThe role of gastrostomy and noninvasive ventilation in primary lateral sclerosispt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage456pt_PT
oaire.citation.issue3pt_PT
oaire.citation.startPage450pt_PT
oaire.citation.titleMuscle & Nervept_PT
oaire.citation.volume71pt_PT
person.familyNameOliveira Santos
person.familyNameDomingues
person.familyNameAguiar Simão
person.familyNameSilva
person.familyNameRibeiro Lopes Alves
person.familyNamede Carvalho
person.givenNameMiguel
person.givenNameSara
person.givenNameSara
person.givenNameMarta Luísa Gromicho Morgado
person.givenNameInês Alexandra
person.givenNameMamede
person.identifierB-2171-2018
person.identifier.ciencia-idB511-DD32-12D7
person.identifier.ciencia-idEA1D-1979-5C3A
person.identifier.ciencia-id461E-71A2-7526
person.identifier.orcid0000-0002-8290-0410
person.identifier.orcid0000-0002-8649-9038
person.identifier.orcid0000-0002-1555-9339
person.identifier.orcid0000-0003-2111-4579
person.identifier.orcid0000-0002-7146-414X
person.identifier.orcid0000-0001-7556-0158
person.identifier.scopus-author-id57217474349
person.identifier.scopus-author-id6506583274
person.identifier.scopus-author-id7101893769
rcaap.rightsrestrictedAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublicationeab02009-54a9-4518-8b22-9426433cea69
relation.isAuthorOfPublicationed45c1ea-7a72-476f-9270-0d9e849b93bd
relation.isAuthorOfPublication9938877e-9069-43f3-8699-25eb85414ff4
relation.isAuthorOfPublicationc6f0ac72-fdd9-499d-87e2-f13aa1545565
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relation.isAuthorOfPublication.latestForDiscoveryc6f0ac72-fdd9-499d-87e2-f13aa1545565

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