Please use this identifier to cite or link to this item: https://ahro.austin.org.au/austinjspui/handle/1/12727
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dc.contributor.authorBerlowitz, David Jen
dc.contributor.authorHoward, Mark Een
dc.contributor.authorFiore, Julio Fen
dc.contributor.authorVander Hoorn, Stephenen
dc.contributor.authorO'Donoghue, Fergal Jen
dc.contributor.authorWestlake, Justineen
dc.contributor.authorSmith, Annaen
dc.contributor.authorBeer, Fionaen
dc.contributor.authorMathers, Susanen
dc.contributor.authorTalman, Paulen
dc.date.accessioned2015-05-16T02:27:33Z-
dc.date.available2015-05-16T02:27:33Z-
dc.date.issued2015-04-09en
dc.identifier.citationJournal of Neurology, Neurosurgery, and Psychiatry 2015; 87(3): 280-6en
dc.identifier.govdoc25857659en
dc.identifier.otherPUBMEDen
dc.identifier.urihttps://ahro.austin.org.au/austinjspui/handle/1/12727en
dc.description.abstractRespiratory failure is associated with significant morbidity and is the predominant cause of death in motor neurone disease/amyotrophic lateral sclerosis (MND/ALS). This study aimed to determine the effect of non-invasive ventilatory (NIV) support on survival and pulmonary function decline across MND/ALS phenotypes.Cohort recruited via a specialist, multidisciplinary clinic. Patients were categorised into four clinical phenotypes (ALS, flail arm, flail leg and primary lateral sclerosis) according to site of presenting symptom and the pattern of upper versus lower motor neurone involvement. NIV was initiated according to current consensus practice guidelines.Between 1991 and 2011, 1198 patients diagnosed with ALS/MND were registered. 929 patients (77.5%) fulfilled the selection criteria and their data were analysed. Median tracheostomy free survival from symptom onset was 28 months in NIV-treated patients compared to 15 months in untreated (Univariate Cox regression HR=0.61 (0.51 to 0.73), p<0.001). The positive survival effect of NIV persisted when the model was adjusted for age, gender, riluzole and percutaneous endoscopic gastrostomy use (HR=0.72 (0.60 to 0.88, p=0.001). In contrast with the only randomised controlled trial, NIV statistically significantly increased survival by 19 months in those with ALS-bulbar onset (Univariate HR=0.50 (0.36 to 0.70), multivariate HR=0.59 (0.41 to 0.83)). These data confirm that NIV improves survival in MND/ALS. The overall magnitude of benefit is 13 months and was largest in those with ALS-bulbar disease. Future research should explore the optimal timing of NIV initiation within phenotypes in order to optimise respiratory function, quality of life and survival.en
dc.language.isoenen
dc.subject.otherMOTOR NEURON DISEASEen
dc.subject.otherSLEEPen
dc.titleIdentifying who will benefit from non-invasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort.en
dc.typeJournal Articleen
dc.identifier.journaltitleJournal of neurology, neurosurgery, and psychiatryen
dc.identifier.affiliationStatistical Consulting Centre, The University of Melbourne, Melbourne, Victoria, Australiaen
dc.identifier.affiliationNeurology Unit, Calvary Health Care, Bethlehem Hospital, Caulfield, Victoria, Australiaen
dc.identifier.affiliationInstitute for Breathing and Sleep, Austin Health, Heidelberg, Victoria, Australiaen
dc.identifier.doi10.1136/jnnp-2014-310055en
dc.relation.urlhttps://pubmed.ncbi.nlm.nih.gov/25857659en
dc.identifier.orcid0000-0003-2543-8722-
dc.identifier.orcid0000-0001-5099-3184-
dc.type.austinJournal Articleen
local.name.researcherBerlowitz, David J
item.openairecristypehttp://purl.org/coar/resource_type/c_18cf-
item.cerifentitytypePublications-
item.fulltextNo Fulltext-
item.grantfulltextnone-
item.languageiso639-1en-
item.openairetypeJournal Article-
crisitem.author.deptPhysiotherapy-
crisitem.author.deptInstitute for Breathing and Sleep-
crisitem.author.deptData Analytics Research and Evaluation (DARE) Centre-
crisitem.author.deptInstitute for Breathing and Sleep-
crisitem.author.deptRespiratory and Sleep Medicine-
crisitem.author.deptInstitute for Breathing and Sleep-
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