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Title: | Identification and outcomes of clinical phenotypes in amyotrophic lateral sclerosis/motor neuron disease: Australian National Motor Neuron Disease observational cohort | Austin Authors: | Talman, Paul;Duong, Thi;Vucic, Steve;Mathers, Susan;Venkatesh, Svetha;Henderson, Robert;Rowe, Dominic;Schultz, David;Edis, Robert;Needham, Merrilee;Macdonnell, Richard AL;McCombe, Pamela A;Birks, Carol;Kiernan, Matthew | Affiliation: | Neurosciences Department, University Hospital Geelong, Geelong, Victoria, Australia Center for Pattern Recognition and Data Analytics (PRaDa), Deakin University, Waurn Ponds, Victoria, Australia St Joseph's Hospital, Auburn, NSW, Australia Calvary Healthcare Bethlehem Hospital Neuro-Consultancy, South Caulfield, Victoria, Australia Department of Neurology, Royal Brisbane and Women's Hospital, Herston, Queensland, Australia Faculty of Medicine and Health Sciences, Macquarie University, Sydney, NSW, Australia Department of Neurology, Flinders Medical Centre, Bedford Park, South Australia, Australia Royal Perth Hospital, Shenton Park, Western Australia, Australia Neurosciences Department, Fiona Stanley Hospital, Murdoch, Western Australia, Australia Neurosciences Department, Austin Health, Heidelberg, Victoria, Australia Department of Neurology, University of Queensland Centre for Clinical Research and Royal Brisbane and Women's Hospital, Herston, Queensland, Australia Motor Neurone Disease Association of Australia, Australia Sydney Medical School, The University of Sydney, Sydney, NSW, Australia |
Issue Date: | 30-Sep-2016 | Date: | 2016-09-30 | Publication information: | BMJ Open 2016; 6(9): e012054 | Abstract: | OBJECTIVE: To capture the clinical patterns, timing of key milestones and survival of patients presenting with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) within Australia. METHODS: Data were prospectively collected and were timed to normal clinical assessments. An initial registration clinical report form (CRF) and subsequent ongoing assessment CRFs were submitted with a completion CRF at the time of death. DESIGN: Prospective observational cohort study. PARTICIPANTS: 1834 patients with a diagnosis of ALS/MND were registered and followed in ALS/MND clinics between 2005 and 2015. RESULTS: 5 major clinical phenotypes were determined and included ALS bulbar onset, ALS cervical onset and ALS lumbar onset, flail arm and leg and primary lateral sclerosis (PLS). Of the 1834 registered patients, 1677 (90%) could be allocated a clinical phenotype. ALS bulbar onset had a significantly lower length of survival when compared with all other clinical phenotypes (p<0.004). There were delays in the median time to diagnosis of up to 12 months for the ALS phenotypes, 18 months for the flail limb phenotypes and 19 months for PLS. Riluzole treatment was started in 78-85% of cases. The median delays in initiating riluzole therapy, from symptom onset, varied from 10 to 12 months in the ALS phenotypes and 15-18 months in the flail limb phenotypes. Percutaneous endoscopic gastrostomy was implemented in 8-36% of ALS phenotypes and 2-9% of the flail phenotypes. Non-invasive ventilation was started in 16-22% of ALS phenotypes and 21-29% of flail phenotypes. CONCLUSIONS: The establishment of a cohort registry for ALS/MND is able to determine clinical phenotypes, survival and monitor time to key milestones in disease progression. It is intended to expand the cohort to a more population-based registry using opt-out methodology and facilitate data linkage to other national registries. | URI: | https://ahro.austin.org.au/austinjspui/handle/1/16399 | DOI: | 10.1136/bmjopen-2016-012054 | Journal: | BMJ Open | PubMed URL: | https://pubmed.ncbi.nlm.nih.gov/27694488 | Type: | Journal Article | Subjects: | Amyotrophic Lateral Sclerosis Motor Neuron Disease |
Appears in Collections: | Journal articles |
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