Comprehensive Analysis Platform To Understand, Remedy and Eliminate ALS

Study Overview
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Brief Summary
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CAPTURE ALS is a long-term data and biorepository platform that will facilitate future ALS research. CAPTURE ALS will provide the standardized systems and tools necessary to collect, store, and analyze vast amounts of multimodal information about ALS. These multimodal datasets and biosamples will be made available for use by researchers or industry across Canada and around the world in accordance with the CAPTURE ALS Data Sharing Policy to advance research on ALS.

Detailed Description
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In this observational study, participants with ALS and related disorders will be followed longitudinally over the course of a year to examine disease progression. Participants with ALS or a related disorder will have up to 5 in-person visits at screening, 0, 4, 8 and 12 months. Healthy controls are included as comparisons and will have 3 in-person visits at screening, 0 and 8 months. At each visit after screening, patients and healthy controls will undergo an MRI, neurocognitive and speech testing, clinical evaluations specific to ALS (ALSFRS-R, FVC, neurological exam, which are not completed by controls), answer questionnaires about their health and have biospecimens, including blood, saliva and optionally cerebrospinal fluid, collected.

Official Title
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Comprehensive Analysis Platform To Understand, Remedy and Eliminate ALS

Conditions
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Amyotrophic Lateral Sclerosis, Primary Lateral Sclerosis, Progressive Muscular Atrophy, Frontotemporal Degeneration

Participation Criteria
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Eligibility Criteria
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[PATIENTS] Inclusion Criteria: Has ALS, classified as definite, probable, laboratory-supported probable, or possible ALS according to the revised El Escorial criteria or a related neurodegenerative disorder including ALS-FTD, PLS, PMA or asymptomatic individuals with a known ALS mutation (as previously confirmed during regular clinical care) Be of the age of majority in their province of residence/treatment Have the cognitive capacity to provide informed consent Have proficiency in English or French in order to understand study instructions and respond to questionnaires Exclusion Criteria: N/A [HEALTHY CONTROLS] Inclusion Criteria: Be between the ages of 40-80 unless age matched to a patient (+/-3 years) who is already enrolled Be the age of majority in their province of residence/treatment Have the cognitive capacity to provide informed consent Have proficiency in English of French to understand study instructions and respond to questionnaires Exclusion Criteria: A history of a neurological disease, including Central Nervous System disease (e.g., stroke, head injury, epilepsy) or Peripheral Nervous System disease (neuropathy, myopathy) A history of psychiatric disease (e.g. depression, bipolar disease) that is clinically diagnosed and/or with the current use of psychiatric medications (e.g. antidepressants) for an indication of a psychiatric disease. Subjects ineligible for an MRI due to a pacemaker or other contraindication according to local MRI policies of the study centre. Significant claustrophobia that would prohibit an MRI (subjects will be required to lie in an MRI scanner for approximately one hour).

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| ALS Functional Rating Scale-Revised (ALSFRS-R) | The ALSFRS-R will be used as a global measure of disease status. It measures activities of daily living and consists of 12 questions, each scored from 0-4, with higher scores representing better function. Changes in ALSFRS-R score over time will be measured. | Once every 4 months for a year | 
| Forced Vital Capacity (FVC) | FVC will be used as an index of respiratory function over time. FVC is the maximal volume of air exhaled with maximally forced effort from a maximal inspiration. | Once every 4 months for a year | 
| Speech Testing | Changes in speech function over time will be measured using a web-browser based audio and video data collection tool. | Once every 4 months for a year | 
| Neurological dysfunction as detected during standard neurological examination | Changes in neurological function over time will be measured through a standard neurological examination. | Once every 4 months for a year | 
| Magnetic Resonance Imaging (MRI) | Various advanced MRI techniques will be used to assess nervous system structure and function. | Once every 4 months for a year | 
| Edinburgh Cognitive and Behavioural Screen (ECAS) | Changes in cognitive function over time will be measured by the ECAS. | Once every 4 months for a year | 
| ALS Assessment Questionnaire (ALSAQ-5) | Changes in quality of life over time will be measured using the ALSAQ-5, a participant reported outcome measure. | Once every 4 months for a year | 
| World Health Organization Quality of Life Questionnaire (WHO-QOL-BREF) | Changes in quality of life over time will be measured using the WHO-QOL-BREF, a participant reported outcome measure. | Once every 4 months for a year | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Biofluids, Biomarker, Biorepository, ALS, Disease Progression, MRI