Evidence map›Paper›PMID 27694488›Full record

Observational studyBMJ open2016

Identification and outcomes of clinical phenotypes in amyotrophic lateral sclerosis/motor neuron disease: Australian National Motor Neuron Disease observational cohort.

Paul Talman, Thi Duong, Steve Vucic, Susan Mathers, Svetha Venkatesh, Robert Henderson, Dominic Rowe, David Schultz, Robert Edis, Merrilee Needham and 4 more

Open access · goldAbstract readObservational Study
PubMed Full text at PMC Publisher
In one paragraph

Observational study in BMJ open, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.

0numbers the graph read from it
0cells of the map it votes in
31citing papers in PubMed
2.9field-weighted citation impact, top 9% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

31 citing papers in PubMed, 76 citations in OpenAlex.

  1. Trial
  2. Natural history of 441 cases of bulbar onset motor neuron disease (MND) in China: a single-center retrospective study.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026
    Article
  3. Article
  4. Article
  5. Article
  6. Article
  7. Review
  8. Banking on My Voice: Life with Motor Neurone Disease.Healthcare (Basel, Switzerland) · 2025
    Article
  9. Article
  10. Article
  11. Article
  12. Article
  13. Review
  14. Article
  15. Article
  16. Review
  17. Review
  18. Enteral tube feeding for amyotrophic lateral sclerosis/motor neuron disease.The Cochrane database of systematic reviews · 2023
    Review
  19. Amyotrophic Lateral Sclerosis: Advances and Prospects.Journal of clinical medicine · 2023
    Article
  20. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

14 authors at 12 institutions in 1 country.

Paul TalmanNeurosciences Department, University Hospital Geelong, Geelong, Victoria, Australia.
Thi DuongCenter for Pattern Recognition and Data Analytics (PRaDa), Deakin University, Waurn Ponds, Geelong, Victoria, Australia.
Steve VucicSt Joseph's Hospital Nomanby Road, Auburn, New South Wales, Australia.
Susan MathersCalvary Healthcare Bethlehem Hospital Neuro-Consultancy, South Caulfield, Victoria, Australia.
Svetha VenkateshCenter for Pattern Recognition and Data Analytics (PRaDa), Deakin University, Waurn Ponds, Geelong, Victoria, Australia.
Robert HendersonDepartment of Neurology, Royal Brisbane and Women's Hospital, Herston, Queensland, Australia Caulfield, Victoria, Australia.
Dominic RoweFaculty of Medicine and Health Sciences, Macquarie University, Sydney, New South Wales, Australia.
David SchultzDepartment of Neurology, Flinders Medical Centre, Flinders Drive, Bedford Park, South Australia, Australia.
Robert EdisRoyal Perth Hospital, Shenton Park Campus Selby Street, Shenton Park, Western Australia, Australia.
Merrilee NeedhamNeurosciences Department, Fiona Stanley Hospital Murdoch, Western Australia, Australia.
Richard MacdonnellNeurosciences Department, Austin Health Studly Road, Heidelberg, Victoria, Australia.
Pamela McCombeDepartment of Neurology, University of Queensland Centre for Clinical Research and Royal Brisbane and Women's Hospital, Herston Road, Queensland, Australia.
Carol BirksMotor Neurone Disease Association of Australia, Australia.
Matthew KiernanSydney Medical School, The University of Sydney, Sydney, New South Wales, Australia.
Deakin University · AURoyal Brisbane and Women's Hospital · AUAuburn Hospital · AUAustin Health · AUCalvary Health Care Bethlehem · AUFiona Stanley Hospital · AUFlinders Medical Centre · AUGeelong Hospital · AUMacquarie University · AUMotor Neurone Disease Research Australia · AURoyal Perth Hospital · AUThe University of Sydney · AU

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveTo capture the clinical patterns, timing of key milestones and survival of patients presenting with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) within Australia.

methodsData 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.

designProspective observational cohort study.

participants1834 patients with a diagnosis of ALS/MND were registered and followed in ALS/MND clinics between 2005 and 2015.

results5 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.

conclusionsThe 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.

Indexed as

Disease ProgressionAmyotrophic Lateral SclerosisAustraliaFemaleGastrostomyHumansKaplan-Meier EstimateMaleMiddle AgedNeuroprotective AgentsNoninvasive VentilationPhenotypeProspective StudiesRegistriesRiluzoleTime FactorsNeuroprotective AgentsRiluzoleEPIDEMIOLOGY

Identifiers

PMID27694488
PMCPMC5051496
OpenAlexW2524391050

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.