{"id":"https://openalex.org/W4225689915","doi":"https://doi.org/10.1038/s41746-022-00588-8","title":"A machine-learning based objective measure for ALS disease severity","display_name":"A machine-learning based objective measure for ALS disease severity","publication_year":2022,"publication_date":"2022-04-08","ids":{"openalex":"https://openalex.org/W4225689915","doi":"https://doi.org/10.1038/s41746-022-00588-8","pmid":"https://pubmed.ncbi.nlm.nih.gov/35396385"},"language":"en","primary_location":{"id":"doi:10.1038/s41746-022-00588-8","is_oa":true,"landing_page_url":"https://doi.org/10.1038/s41746-022-00588-8","pdf_url":"https://www.nature.com/articles/s41746-022-00588-8.pdf","source":{"id":"https://openalex.org/S4210195431","display_name":"npj Digital Medicine","issn_l":"2398-6352","issn":["2398-6352"],"is_oa":true,"is_in_doaj":true,"is_core":true,"host_organization":"https://openalex.org/P4310319908","host_organization_name":"Nature Portfolio","host_organization_lineage":["https://openalex.org/P4310319908","https://openalex.org/P4310319965"],"host_organization_lineage_names":["Nature Portfolio","Springer Nature"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"npj Digital Medicine","raw_type":"journal-article"},"type":"article","indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://www.nature.com/articles/s41746-022-00588-8.pdf","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5005231504","display_name":"Fernando G. Vieira","orcid":"https://orcid.org/0000-0003-2996-6418"},"institutions":[{"id":"https://openalex.org/I2800438382","display_name":"Amyotrophic Lateral Sclerosis Therapy Development Institute","ror":"https://ror.org/04khs0d04","country_code":"US","type":"nonprofit","lineage":["https://openalex.org/I2800438382"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Fernando G. Vieira","raw_affiliation_strings":["ALS Therapy Development Institute, Watertown, MA, USA. fvieira@als.net","ALS Therapy Development Institute, Watertown, MA, USA"],"affiliations":[{"raw_affiliation_string":"ALS Therapy Development Institute, Watertown, MA, USA. fvieira@als.net","institution_ids":[]},{"raw_affiliation_string":"ALS Therapy Development Institute, Watertown, MA, USA","institution_ids":["https://openalex.org/I2800438382"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5025689655","display_name":"Subhashini Venugopalan","orcid":"https://orcid.org/0000-0003-3729-8456"},"institutions":[{"id":"https://openalex.org/I1291425158","display_name":"Google (United States)","ror":"https://ror.org/00njsd438","country_code":"US","type":"company","lineage":["https://openalex.org/I1291425158","https://openalex.org/I4210128969"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Subhashini Venugopalan","raw_affiliation_strings":["Google Research, Google, Mountain View, CA, USA. vsubhashini@google.com","Google Research, Google, Mountain View, CA, USA"],"affiliations":[{"raw_affiliation_string":"Google Research, Google, Mountain View, CA, USA. vsubhashini@google.com","institution_ids":[]},{"raw_affiliation_string":"Google Research, Google, Mountain View, CA, USA","institution_ids":["https://openalex.org/I1291425158"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5005113764","display_name":"Alan Premasiri","orcid":"https://orcid.org/0000-0002-0261-9953"},"institutions":[{"id":"https://openalex.org/I2800438382","display_name":"Amyotrophic Lateral Sclerosis Therapy Development Institute","ror":"https://ror.org/04khs0d04","country_code":"US","type":"nonprofit","lineage":["https://openalex.org/I2800438382"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Alan S. Premasiri","raw_affiliation_strings":["ALS Therapy Development Institute, Watertown, MA, USA"],"affiliations":[{"raw_affiliation_string":"ALS Therapy Development Institute, Watertown, MA, USA","institution_ids":["https://openalex.org/I2800438382"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5042482839","display_name":"Maeve McNally","orcid":null},"institutions":[{"id":"https://openalex.org/I2800438382","display_name":"Amyotrophic Lateral Sclerosis Therapy Development Institute","ror":"https://ror.org/04khs0d04","country_code":"US","type":"nonprofit","lineage":["https://openalex.org/I2800438382"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Maeve McNally","raw_affiliation_strings":["ALS Therapy Development Institute, Watertown, MA, USA"],"affiliations":[{"raw_affiliation_string":"ALS Therapy Development Institute, Watertown, MA, USA","institution_ids":["https://openalex.org/I2800438382"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5103622427","display_name":"Aren Jansen","orcid":null},"institutions":[{"id":"https://openalex.org/I1291425158","display_name":"Google (United States)","ror":"https://ror.org/00njsd438","country_code":"US","type":"company","lineage":["https://openalex.org/I1291425158","https://openalex.org/I4210128969"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Aren Jansen","raw_affiliation_strings":["Google Research, Google, Mountain View, CA, USA"],"affiliations":[{"raw_affiliation_string":"Google Research, Google, Mountain View, CA, USA","institution_ids":["https://openalex.org/I1291425158"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5062785992","display_name":"Kevin McCloskey","orcid":"https://orcid.org/0000-0001-9967-4117"},"institutions":[{"id":"https://openalex.org/I1291425158","display_name":"Google (United States)","ror":"https://ror.org/00njsd438","country_code":"US","type":"company","lineage":["https://openalex.org/I1291425158","https://openalex.org/I4210128969"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Kevin McCloskey","raw_affiliation_strings":["Google Research, Google, Mountain View, CA, USA"],"affiliations":[{"raw_affiliation_string":"Google Research, Google, Mountain View, CA, USA","institution_ids":["https://openalex.org/I1291425158"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5001027226","display_name":"Michael P. Brenner","orcid":"https://orcid.org/0000-0002-5673-7947"},"institutions":[{"id":"https://openalex.org/I136199984","display_name":"Harvard University","ror":"https://ror.org/03vek6s52","country_code":"US","type":"education","lineage":["https://openalex.org/I136199984"]},{"id":"https://openalex.org/I1291425158","display_name":"Google (United States)","ror":"https://ror.org/00njsd438","country_code":"US","type":"company","lineage":["https://openalex.org/I1291425158","https://openalex.org/I4210128969"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Michael P. Brenner","raw_affiliation_strings":["Google Research, Google, Mountain View, CA, USA","School of Engineering and Applied Sciences, Harvard University, Cambridge, MA, USA"],"affiliations":[{"raw_affiliation_string":"Google Research, Google, Mountain View, CA, USA","institution_ids":["https://openalex.org/I1291425158"]},{"raw_affiliation_string":"School of Engineering and Applied Sciences, Harvard University, Cambridge, MA, USA","institution_ids":["https://openalex.org/I136199984"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5034727139","display_name":"Steven Perrin","orcid":"https://orcid.org/0009-0004-9863-4948"},"institutions":[{"id":"https://openalex.org/I2800438382","display_name":"Amyotrophic Lateral Sclerosis Therapy Development Institute","ror":"https://ror.org/04khs0d04","country_code":"US","type":"nonprofit","lineage":["https://openalex.org/I2800438382"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Steven Perrin","raw_affiliation_strings":["ALS Therapy Development Institute, Watertown, MA, USA","Eledon Pharmaceuticals, Irvine, CA, USA"],"affiliations":[{"raw_affiliation_string":"ALS Therapy Development Institute, Watertown, MA, USA","institution_ids":["https://openalex.org/I2800438382"]},{"raw_affiliation_string":"Eledon Pharmaceuticals, Irvine, CA, USA","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":8,"corresponding_author_ids":["https://openalex.org/A5005231504","https://openalex.org/A5025689655"],"corresponding_institution_ids":["https://openalex.org/I1291425158","https://openalex.org/I2800438382"],"apc_list":{"value":3060,"currency":"USD","value_usd":3060},"apc_paid":{"value":3060,"currency":"USD","value_usd":3060},"fwci":4.9798,"has_fulltext":true,"cited_by_count":43,"citation_normalized_percentile":{"value":0.96378437,"is_in_top_1_percent":false,"is_in_top_10_percent":true},"cited_by_percentile_year":{"min":97,"max":100},"biblio":{"volume":"5","issue":"1","first_page":"45","last_page":"45"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10855","display_name":"Amyotrophic Lateral Sclerosis Research","score":0.9997000098228455,"subfield":{"id":"https://openalex.org/subfields/2728","display_name":"Neurology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10855","display_name":"Amyotrophic Lateral Sclerosis Research","score":0.9997000098228455,"subfield":{"id":"https://openalex.org/subfields/2728","display_name":"Neurology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10085","display_name":"Parkinson's Disease Mechanisms and Treatments","score":0.9879999756813049,"subfield":{"id":"https://openalex.org/subfields/2728","display_name":"Neurology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11358","display_name":"Dysphagia Assessment and Management","score":0.9821000099182129,"subfield":{"id":"https://openalex.org/subfields/3616","display_name":"Speech and Hearing"},"field":{"id":"https://openalex.org/fields/36","display_name":"Health Professions"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/amyotrophic-lateral-sclerosis","display_name":"Amyotrophic lateral sclerosis","score":0.7039083242416382},{"id":"https://openalex.org/keywords/medicine","display_name":"Medicine","score":0.5981521606445312},{"id":"https://openalex.org/keywords/rating-scale","display_name":"Rating scale","score":0.5956935286521912},{"id":"https://openalex.org/keywords/physical-therapy","display_name":"Physical therapy","score":0.5725120306015015},{"id":"https://openalex.org/keywords/cohort","display_name":"Cohort","score":0.5275121331214905},{"id":"https://openalex.org/keywords/physical-medicine-and-rehabilitation","display_name":"Physical medicine and rehabilitation","score":0.5088565349578857},{"id":"https://openalex.org/keywords/disease","display_name":"Disease","score":0.3499285578727722},{"id":"https://openalex.org/keywords/psychology","display_name":"Psychology","score":0.2900102734565735},{"id":"https://openalex.org/keywords/internal-medicine","display_name":"Internal medicine","score":0.21880972385406494}],"concepts":[{"id":"https://openalex.org/C2780596555","wikidata":"https://www.wikidata.org/wiki/Q206901","display_name":"Amyotrophic lateral sclerosis","level":3,"score":0.7039083242416382},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.5981521606445312},{"id":"https://openalex.org/C83849319","wikidata":"https://www.wikidata.org/wiki/Q7295720","display_name":"Rating scale","level":2,"score":0.5956935286521912},{"id":"https://openalex.org/C1862650","wikidata":"https://www.wikidata.org/wiki/Q186005","display_name":"Physical therapy","level":1,"score":0.5725120306015015},{"id":"https://openalex.org/C72563966","wikidata":"https://www.wikidata.org/wiki/Q1303415","display_name":"Cohort","level":2,"score":0.5275121331214905},{"id":"https://openalex.org/C99508421","wikidata":"https://www.wikidata.org/wiki/Q2678675","display_name":"Physical medicine and rehabilitation","level":1,"score":0.5088565349578857},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.3499285578727722},{"id":"https://openalex.org/C15744967","wikidata":"https://www.wikidata.org/wiki/Q9418","display_name":"Psychology","level":0,"score":0.2900102734565735},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.21880972385406494},{"id":"https://openalex.org/C138496976","wikidata":"https://www.wikidata.org/wiki/Q175002","display_name":"Developmental psychology","level":1,"score":0.0}],"mesh":[],"locations_count":4,"locations":[{"id":"doi:10.1038/s41746-022-00588-8","is_oa":true,"landing_page_url":"https://doi.org/10.1038/s41746-022-00588-8","pdf_url":"https://www.nature.com/articles/s41746-022-00588-8.pdf","source":{"id":"https://openalex.org/S4210195431","display_name":"npj Digital Medicine","issn_l":"2398-6352","issn":["2398-6352"],"is_oa":true,"is_in_doaj":true,"is_core":true,"host_organization":"https://openalex.org/P4310319908","host_organization_name":"Nature Portfolio","host_organization_lineage":["https://openalex.org/P4310319908","https://openalex.org/P4310319965"],"host_organization_lineage_names":["Nature Portfolio","Springer Nature"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"npj Digital Medicine","raw_type":"journal-article"},{"id":"pmid:35396385","is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/35396385","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"NPJ digital medicine","raw_type":null},{"id":"pmh:oai:doaj.org/article:f020b25d7ebb4e21a10ad430ce2cc01b","is_oa":true,"landing_page_url":"https://doaj.org/article/f020b25d7ebb4e21a10ad430ce2cc01b","pdf_url":null,"source":{"id":"https://openalex.org/S112646816","display_name":"SHILAP Revista de lepidopterolog\u00eda","issn_l":"0300-5267","issn":["0300-5267","2340-4078"],"is_oa":true,"is_in_doaj":true,"is_core":false,"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"npj Digital Medicine, Vol 5, Iss 1, Pp 1-9 (2022)","raw_type":"article"},{"id":"pmh:oai:pubmedcentral.nih.gov:8993812","is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/8993812","pdf_url":null,"source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"NPJ Digit Med","raw_type":"Text"}],"best_oa_location":{"id":"doi:10.1038/s41746-022-00588-8","is_oa":true,"landing_page_url":"https://doi.org/10.1038/s41746-022-00588-8","pdf_url":"https://www.nature.com/articles/s41746-022-00588-8.pdf","source":{"id":"https://openalex.org/S4210195431","display_name":"npj Digital Medicine","issn_l":"2398-6352","issn":["2398-6352"],"is_oa":true,"is_in_doaj":true,"is_core":true,"host_organization":"https://openalex.org/P4310319908","host_organization_name":"Nature Portfolio","host_organization_lineage":["https://openalex.org/P4310319908","https://openalex.org/P4310319965"],"host_organization_lineage_names":["Nature Portfolio","Springer Nature"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"npj Digital Medicine","raw_type":"journal-article"},"sustainable_development_goals":[{"score":0.7599999904632568,"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3"}],"awards":[],"funders":[],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W4225689915.pdf","grobid_xml":"https://content.openalex.org/works/W4225689915.grobid-xml"},"referenced_works_count":34,"referenced_works":["https://openalex.org/W1972573064","https://openalex.org/W1978808176","https://openalex.org/W1993184479","https://openalex.org/W1996748981","https://openalex.org/W1998437790","https://openalex.org/W2001918801","https://openalex.org/W2026980091","https://openalex.org/W2030932792","https://openalex.org/W2032162800","https://openalex.org/W2048891340","https://openalex.org/W2072562340","https://openalex.org/W2081468682","https://openalex.org/W2085165928","https://openalex.org/W2108381339","https://openalex.org/W2113575093","https://openalex.org/W2120449849","https://openalex.org/W2129432034","https://openalex.org/W2143911129","https://openalex.org/W2152930788","https://openalex.org/W2475597696","https://openalex.org/W2513203369","https://openalex.org/W2526050071","https://openalex.org/W2625127693","https://openalex.org/W2750922690","https://openalex.org/W2766949700","https://openalex.org/W2792503082","https://openalex.org/W2889263571","https://openalex.org/W2902309040","https://openalex.org/W2909404091","https://openalex.org/W2928494154","https://openalex.org/W2998055001","https://openalex.org/W3174363935","https://openalex.org/W3186808236","https://openalex.org/W4248058730"],"related_works":["https://openalex.org/W2070831599","https://openalex.org/W4206749202","https://openalex.org/W2154294661","https://openalex.org/W2393752460","https://openalex.org/W2048256717","https://openalex.org/W2059706093","https://openalex.org/W4292226083","https://openalex.org/W2938751847","https://openalex.org/W2970628625","https://openalex.org/W4387118726"],"abstract_inverted_index":{"Amyotrophic":[0],"Lateral":[1],"Sclerosis":[2],"(ALS)":[3],"disease":[4,22,55,244],"severity":[5,23,56,245],"is":[6],"usually":[7],"measured":[8],"using":[9],"the":[10,115,122,139,177,190,205,211,216,219,231,237],"subjective,":[11],"questionnaire-based":[12],"revised":[13],"ALS":[14,54,73],"Functional":[15],"Rating":[16],"Scale":[17],"(ALSFRS-R).":[18],"Objective":[19],"measures":[20],"of":[21,129,147,180,192,201,239],"would":[24],"be":[25],"powerful":[26],"tools":[27,241],"for":[28,38,41,53,185,242],"evaluating":[29],"real-world":[30,178],"drug":[31,183,248],"effectiveness,":[32],"efficacy":[33],"in":[34,158,164,187,230],"clinical":[35],"trials,":[36],"and":[37,61,75,80,89,110,171],"identifying":[39],"participants":[40,84,195],"cohort":[42,191],"studies.":[43],"We":[44,167],"developed":[45],"a":[46,65,126,143,182],"machine":[47],"learning":[48],"(ML)":[49],"based":[50,57],"objective":[51],"measure":[52],"on":[58,134,149],"voice":[59,87,123],"samples":[60],"accelerometer":[62,93,140],"measurements":[63],"from":[64],"four-year":[66],"longitudinal":[67],"dataset.":[68],"584":[69],"people":[70],"living":[71],"with":[72,210],"consented":[74],"carried":[76],"out":[77],"prescribed":[78],"speaking":[79],"limb-based":[81],"tasks.":[82],"542":[83],"contributed":[85,91],"5814":[86],"recordings,":[88],"350":[90],"13,009":[92],"samples,":[94],"while":[95],"simultaneously":[96],"measuring":[97],"ALSFRS-R":[98,112,136,160,173,213,233],"scores.":[99,113,166,214,234],"Using":[100],"these":[101,169,240],"data,":[102],"we":[103],"trained":[104],"ML":[105],"models":[106,124,141,170],"to":[107,175],"predict":[108],"bulbar-related":[109],"limb-related":[111,151],"On":[114],"test":[116,194],"set":[117],"(n":[118],"=":[119],"109":[120],"participants)":[121],"achieved":[125,142],"multiclass":[127,145],"AUC":[128,146],"0.86":[130],"(95%":[131],"CI,":[132],"0.85-0.88)":[133],"speech":[135],"prediction,":[137],"whereas":[138],"median":[144],"0.73":[148],"6":[150],"functions.":[152],"The":[153],"correlations":[154],"across":[155],"functions":[156],"observed":[157],"self-reported":[159,172,212],"scores":[161,174,207],"were":[162,208],"preserved":[163],"ML-derived":[165,206,221],"used":[168],"evaluate":[176],"effects":[179],"edaravone,":[181],"approved":[184],"use":[186],"ALS.":[188],"In":[189],"54":[193],"who":[196],"received":[197],"edaravone":[198],"as":[199],"part":[200],"their":[202],"usual":[203],"care,":[204],"consistent":[209],"At":[215],"individual":[217],"level,":[218],"continuous":[220],"score":[222],"can":[223],"capture":[224],"gradual":[225],"changes":[226],"that":[227],"are":[228],"absent":[229],"integer":[232],"This":[235],"demonstrates":[236],"value":[238],"assessing":[243],"and,":[246],"potentially,":[247],"effects.":[249]},"counts_by_year":[{"year":2026,"cited_by_count":3},{"year":2025,"cited_by_count":17},{"year":2024,"cited_by_count":9},{"year":2023,"cited_by_count":9},{"year":2022,"cited_by_count":5}],"updated_date":"2026-03-20T23:20:44.827607","created_date":"2025-10-10T00:00:00"}