Literature DB >> 24463480

Use of clinical staging in amyotrophic lateral sclerosis for phase 3 clinical trials.

Rubika Balendra1, Ashley Jones1, Naheed Jivraj2, I Nick Steen3, Carolyn A Young4, Pamela J Shaw5, Martin R Turner6, P Nigel Leigh7, Ammar Al-Chalabi1.   

Abstract

OBJECTIVES: The use of clinical staging in the fatal neurodegenerative disease amyotrophic lateral sclerosis would have value in optimising future therapeutic trials. We aimed to use previous clinical trial data to determine the length of time patients spend in each of four proposed stages, its range and transition patterns to subsequent stages.
METHODS: Using databases from two multicentre clinical trials, patients were retrospectively staged through the trial course. At each stage we assessed whether patients then progressed to an earlier, consecutive or later stage or death. Duration spent in each stage before progression to a later stage was calculated.
RESULTS: There were 725 patients. No patients moved to an earlier stage. More patients at stages 1, 2 and 3 progressed to the consecutive stage rather than skipping a stage. 59.3% of patients at Stage 1 progressed to Stage 2, 54.0% of patients at Stage 2 progressed to Stage 3, 42.3% of patients at Stage 3 progressed to Stage 4 and 47.0% of Stage 4 patients progressed to death. Transition times between stages had a median duration of 3 to 7 months for stages 2 to 4. DISCUSSION: We have shown using trial data that transition times between stages are short. Use of stage duration as an endpoint might allow a shorter trial duration. We have shown face validity in this system as most patients progress through consecutive stages, and none revert to earlier stages. Furthermore, we have shown the system is reliable across populations and therefore has content validity. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Entities:  

Keywords:  ALS; MOTOR NEURON DISEASE; NEUROMUSCULAR; QUALITY OF LIFE; RANDOMISED TRIALS

Mesh:

Year:  2014        PMID: 24463480     DOI: 10.1136/jnnp-2013-306865

Source DB:  PubMed          Journal:  J Neurol Neurosurg Psychiatry        ISSN: 0022-3050            Impact factor:   10.154


  25 in total

Review 1.  Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease.

Authors:  Jeffrey Rosenfeld; Michael J Strong
Journal:  Neurotherapeutics       Date:  2015-04       Impact factor: 7.620

Review 2.  Quo vadis motor neuron disease?

Authors:  Rubika Balendra; Rickie Patani
Journal:  World J Methodol       Date:  2016-03-26

Review 3.  Supportive and symptomatic management of amyotrophic lateral sclerosis.

Authors:  Esther V Hobson; Christopher J McDermott
Journal:  Nat Rev Neurol       Date:  2016-08-12       Impact factor: 42.937

4.  Tollgate-based progression pathways of ALS patients.

Authors:  Özden O Dalgıç; F Safa Erenay; Kalyan S Pasupathy; Osman Y Özaltın; Brian A Crum; Mustafa Y Sir
Journal:  J Neurol       Date:  2019-01-25       Impact factor: 4.849

5.  Zebra sign of precentral gyri in amyotrophic lateral sclerosis: A novel finding using phase difference enhanced (PADRE) imaging-initial results.

Authors:  Shingo Kakeda; Tetsuya Yoneda; Satoru Ide; Mari Miyata; Tomoyo Hashimoto; Koichiro Futatsuya; Keita Watanabe; Atsushi Ogasawara; Junji Moriya; Toru Sato; Kazumasa Okada; Takenori Uozumi; Hiroaki Adachi; Yukunori Korogi
Journal:  Eur Radiol       Date:  2016-01-28       Impact factor: 5.315

6.  Intuitive Staging Correlates With King's Clinical Stage.

Authors:  Ahmad Al Khleifat; Rubika Balendra; Ton Fang; Ammar Al-Chalabi
Journal:  Amyotroph Lateral Scler Frontotemporal Degener       Date:  2021-04-06       Impact factor: 4.092

7.  Milano-Torino Staging and Long-Term Survival in Chinese Patients with Amyotrophic Lateral Sclerosis.

Authors:  Ruojie He; Minying Zheng; Ling Lian; Xiaoli Yao
Journal:  Cells       Date:  2021-05-17       Impact factor: 6.600

8.  Clusters of anatomical disease-burden patterns in ALS: a data-driven approach confirms radiological subtypes.

Authors:  Peter Bede; Aizuri Murad; Jasmin Lope; Orla Hardiman; Kai Ming Chang
Journal:  J Neurol       Date:  2022-03-25       Impact factor: 6.682

Review 9.  Quantifying disease progression in amyotrophic lateral sclerosis.

Authors:  Neil G Simon; Martin R Turner; Steve Vucic; Ammar Al-Chalabi; Jeremy Shefner; Catherine Lomen-Hoerth; Matthew C Kiernan
Journal:  Ann Neurol       Date:  2014-09-30       Impact factor: 10.422

Review 10.  Ensuring continued progress in biomarkers for amyotrophic lateral sclerosis.

Authors:  Martin R Turner; Michael Benatar
Journal:  Muscle Nerve       Date:  2014-11-24       Impact factor: 3.217

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