Evidence map›Paper›PMID 29868980›Full record

ArticleJournal of neurology2018

Primary lateral sclerosis and the amyotrophic lateral sclerosis-frontotemporal dementia spectrum.

Smriti Agarwal, Elizabeth Highton-Williamson, Jashelle Caga, José M Matamala, Thanuja Dharmadasa, James Howells, Margaret C Zoing, Kazumoto Shibuya, Nimeshan Geevasinga, Steve Vucic and 3 more

Abstract read
PubMed Publisher
In one paragraph

Article in Journal of neurology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.

0numbers the graph read from it
0cells of the map it votes in
17citing papers in PubMed
4.9field-weighted citation impact, top 4% 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

17 citing papers in PubMed, 48 citations in OpenAlex.

  1. Article
  2. Review
  3. Review
  4. Primary Lateral Sclerosis: An Overview.Journal of clinical medicine · 2024
    Review
  5. Article
  6. Article
  7. Article
  8. Review
  9. Article
  10. Review
  11. Article
  12. Better understanding the neurobiology of primary lateral sclerosis.Amyotrophic lateral sclerosis & frontotemporal degeneration · 2020
    Review
  13. Primary lateral sclerosis: consensus diagnostic criteria.Journal of neurology, neurosurgery, and psychiatry · 2020
    Article
  14. Review
  15. Review
  16. Article
  17. 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

13 authors at 2 institutions in 1 country.

Smriti AgarwalBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia. smriti.agarwal@cantab.net.
Elizabeth Highton-WilliamsonBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
Jashelle CagaBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
José M MatamalaBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
Thanuja DharmadasaBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
James HowellsBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
Margaret C ZoingBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
Kazumoto ShibuyaBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
Nimeshan GeevasingaWestmead Clinical School, University of Sydney, Sydney, NSW, Australia.
Steve VucicWestmead Clinical School, University of Sydney, Sydney, NSW, Australia.
John R HodgesBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
Rebekah M AhmedBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
Matthew C KiernanBrain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, 2050, Australia.
University of Sydney · AURoyal Prince Alfred Hospital · AU

Funding

National Health and Medical Research Council APP1037746
6 · The paper itself

Abstract

aimTo investigate whether primary lateral sclerosis (PLS) represents part of the amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) spectrum of diseases.

methodsComprehensive assessment was taken on 21 patients with PLS and results were compared to patients diagnosed with pure motor ALS (n = 27) and ALS-FTD (n = 12). Clinical features, Addenbrooke's Cognitive Examination (ACE) scores, Motor Neuron Disease Behaviour (Mind-B) scores, motor disability on the ALS functional rating scale (ALSFRS) and survival times were documented. Motor cortex excitability was evaluated using transcranial magnetic stimulation (TMS).

resultsGlobal cognition was impaired in PLS (mean total ACE score 82.5 ± 13.6), similar to ALS-FTD (mean total ACE score 76.3 ± 7.7, p > 0.05) while behavioural impairments were not prominent. TMS revealed that resting motor threshold (RMT) was significantly higher in PLS (75.5 ± 6.2) compared ALS-FTD (50.1 ± 7.2, p < 0.001) and ALS (62.3 ± 12.6, p = 0.046). Average short-interval intracortical inhibition (SICI) was similar in all three patient groups. The mean survival time was longest in PLS (217.4 ± 22.4 months) and shortest in ALS-FTD (38.5 ± 4.5 months, p = 0.002). Bulbar onset disease (β = - 0.45, p = 0.007) and RMT (β = 0.54, p = 0.001) were independent predictors of global cognition while motor scores (β = 0.47, p = 0.036) and SICI (β = 0.58, p = 0.006) were significantly associated with ALSFRS.

conclusionThe cognitive profile in PLS resembles ALS-FTD, without prominent behavioural disturbances. A higher RMT in PLS than ALS and ALS-FTD is consistent with differential cortical motor neuronal abnormalities and more severe involvement of corticospinal axons while SICI, indicative of inhibitory interneuronal dysfunction was comparable with ALS and ALS-FTD. Overall, while these findings support the notion that PLS lies on the ALS-FTD spectrum, the mechanisms underlying slow disease progression are likely to be distinct in PLS.

Indexed as

AgedAmyotrophic Lateral SclerosisCognitionDisability EvaluationDisease ProgressionFemaleFrontotemporal DementiaHumansKaplan-Meier EstimateMaleMiddle AgedMotor CortexMotor Neuron DiseaseNeuropsychological TestsSurvival AnalysisTranscranial Magnetic StimulationALS–FTD spectrumClinicalCognitiveMotor cortical functionPLSTMS

Identifiers

PMID29868980
OpenAlexW2806364545

What OpenQuestion holds

Textmetadata
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.