{"id":"https://openalex.org/W4400740258","doi":"https://doi.org/10.3389/fneur.2024.1452409","title":"Hypermobile Ehlers-Danlos syndrome and spontaneous CSF leaks: the connective tissue conundrum","display_name":"Hypermobile Ehlers-Danlos syndrome and spontaneous CSF leaks: the connective tissue conundrum","publication_year":2024,"publication_date":"2024-07-17","ids":{"openalex":"https://openalex.org/W4400740258","doi":"https://doi.org/10.3389/fneur.2024.1452409","pmid":"https://pubmed.ncbi.nlm.nih.gov/39087003","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/11289524"},"language":"en","primary_location":{"id":"doi:10.3389/fneur.2024.1452409","doi":"https://doi.org/10.3389/fneur.2024.1452409","is_oa":true,"landing_page_url":"https://doi.org/10.3389/fneur.2024.1452409","pdf_url":"https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2024.1452409/pdf","source":{"id":"https://openalex.org/S96975567","display_name":"Frontiers in Neurology","issn_l":"1664-2295","issn":["1664-2295"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","jufo-1","medline","norway-1","poland-100","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310320527","host_organization_name":"Frontiers Media","host_organization_lineage":["https://openalex.org/P4310320527"],"host_organization_lineage_names":["Frontiers Media"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Frontiers in Neurology","raw_type":"journal-article"},"volume":null,"type":"review","indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2024.1452409/pdf","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5099072207","display_name":"Sydney Severance","orcid":"https://orcid.org/0009-0002-5488-7025","observed_orcids":["https://orcid.org/0009-0002-5488-7025"]},"institutions":[{"id":"https://openalex.org/I153297377","display_name":"Medical University of South Carolina","ror":"https://ror.org/012jban78","country_code":"US","type":"education","lineage":["https://openalex.org/I153297377"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Sydney Severance","raw_affiliation_strings":["Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States","institution_ids":["https://openalex.org/I153297377"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5069617958","display_name":"Victoria Daylor","orcid":"https://orcid.org/0009-0002-4847-0709","observed_orcids":["https://orcid.org/0009-0002-4847-0709"]},"institutions":[{"id":"https://openalex.org/I153297377","display_name":"Medical University of South Carolina","ror":"https://ror.org/012jban78","country_code":"US","type":"education","lineage":["https://openalex.org/I153297377"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Victoria Daylor","raw_affiliation_strings":["Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States","institution_ids":["https://openalex.org/I153297377"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5091472298","display_name":"Taylor Petrucci","orcid":"https://orcid.org/0000-0003-1524-5804","observed_orcids":["https://orcid.org/0000-0003-1524-5804"]},"institutions":[{"id":"https://openalex.org/I153297377","display_name":"Medical University of South Carolina","ror":"https://ror.org/012jban78","country_code":"US","type":"education","lineage":["https://openalex.org/I153297377"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Taylor Petrucci","raw_affiliation_strings":["Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, United States","Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, United States","institution_ids":["https://openalex.org/I153297377"]},{"raw_affiliation_string":"Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States","institution_ids":["https://openalex.org/I153297377"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5088415665","display_name":"Cortney Gensemer","orcid":"https://orcid.org/0000-0002-8492-3536","observed_orcids":["https://orcid.org/0000-0002-8492-3536"]},"institutions":[{"id":"https://openalex.org/I153297377","display_name":"Medical University of South Carolina","ror":"https://ror.org/012jban78","country_code":"US","type":"education","lineage":["https://openalex.org/I153297377"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Cortney Gensemer","raw_affiliation_strings":["Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, United States","Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, United States","institution_ids":["https://openalex.org/I153297377"]},{"raw_affiliation_string":"Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States","institution_ids":["https://openalex.org/I153297377"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5112357417","display_name":"Sunil M. Patel","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I153297377","display_name":"Medical University of South Carolina","ror":"https://ror.org/012jban78","country_code":"US","type":"education","lineage":["https://openalex.org/I153297377"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Sunil Patel","raw_affiliation_strings":["Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, United States","institution_ids":["https://openalex.org/I153297377"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5013120513","display_name":"Russell A. Norris","orcid":"https://orcid.org/0000-0001-8462-5726","observed_orcids":["https://orcid.org/0000-0001-8462-5726"]},"institutions":[{"id":"https://openalex.org/I153297377","display_name":"Medical University of South Carolina","ror":"https://ror.org/012jban78","country_code":"US","type":"education","lineage":["https://openalex.org/I153297377"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Russell A. Norris","raw_affiliation_strings":["Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, United States","Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, United States","institution_ids":["https://openalex.org/I153297377"]},{"raw_affiliation_string":"Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States","institution_ids":["https://openalex.org/I153297377"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5013120513"],"corresponding_institution_ids":["https://openalex.org/I153297377"],"apc_list":{"value":3295,"currency":"USD","value_usd":3295},"apc_paid":{"value":3295,"currency":"USD","value_usd":3295},"fwci":1.4121,"has_fulltext":true,"cited_by_count":22,"citation_normalized_percentile":{"value":0.8132547,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":100},"biblio":{"volume":"15","issue":null,"first_page":"1452409","last_page":"1452409"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11310","display_name":"Connective tissue disorders research","score":0.3449999988079071,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T11310","display_name":"Connective tissue disorders research","score":0.3449999988079071,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T12622","display_name":"Neurosurgical Procedures and Complications","score":0.1509000062942505,"subfield":{"id":"https://openalex.org/subfields/2728","display_name":"Clinical 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/T11872","display_name":"Barrier Structure and Function Studies","score":0.10010000318288803,"subfield":{"id":"https://openalex.org/subfields/2808","display_name":"Neurology"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/hypermobile-ehlers-danlos-syndrome","display_name":"hypermobile Ehlers-Danlos syndrome","score":0.996999979019165},{"id":"https://openalex.org/keywords/spontaneous-cerebrospinal-fluid-leak","display_name":"spontaneous cerebrospinal fluid leak","score":0.902999997138977},{"id":"https://openalex.org/keywords/connective-tissue-diseases","display_name":"connective tissue diseases","score":0.9649999737739563},{"id":"https://openalex.org/keywords/collagen","display_name":"collagen","score":0.8640000224113464},{"id":"https://openalex.org/keywords/extracellular-matrix","display_name":"extracellular matrix","score":0.9980000257492065},{"id":"https://openalex.org/keywords/blood-brain-barrier","display_name":"blood-brain barrier","score":0.968999981880188},{"id":"https://openalex.org/keywords/neuroinflammation","display_name":"neuroinflammation","score":0.9959999918937683},{"id":"https://openalex.org/keywords/cerebrospinal-fluid","display_name":"cerebrospinal fluid","score":0.6990000009536743},{"id":"https://openalex.org/keywords/chronic-headache","display_name":"chronic headache","score":0.5690000057220459},{"id":"https://openalex.org/keywords/autonomic-dysfunction","display_name":"autonomic dysfunction","score":0.8019999861717224},{"id":"https://openalex.org/keywords/mast-cell-activation","display_name":"mast cell activation","score":0.8679999709129333},{"id":"https://openalex.org/keywords/chronic-pain","display_name":"chronic pain","score":0.7799999713897705}],"concepts":[{"id":"https://openalex.org/C2778333328","wikidata":"https://www.wikidata.org/wiki/Q1141499","display_name":"Ehlers\u2013Danlos syndrome","level":2,"score":0.879997730255127},{"id":"https://openalex.org/C518705261","wikidata":"https://www.wikidata.org/wiki/Q25615","display_name":"Connective tissue","level":2,"score":0.6321781277656555},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.5102999210357666},{"id":"https://openalex.org/C2910325231","wikidata":"https://www.wikidata.org/wiki/Q1779300","display_name":"Connective Tissue Disorder","level":2,"score":0.49027326703071594},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.34574592113494873}],"mesh":[],"locations_count":4,"locations":[{"id":"doi:10.3389/fneur.2024.1452409","doi":"https://doi.org/10.3389/fneur.2024.1452409","is_oa":true,"landing_page_url":"https://doi.org/10.3389/fneur.2024.1452409","pdf_url":"https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2024.1452409/pdf","source":{"id":"https://openalex.org/S96975567","display_name":"Frontiers in Neurology","issn_l":"1664-2295","issn":["1664-2295"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","jufo-1","medline","norway-1","poland-100","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310320527","host_organization_name":"Frontiers Media","host_organization_lineage":["https://openalex.org/P4310320527"],"host_organization_lineage_names":["Frontiers Media"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Frontiers in Neurology","raw_type":"journal-article"},{"id":"pmh:oai:pubmedcentral.nih.gov:11289524","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/11289524","pdf_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC11289524/pdf/fneur-15-1452409.pdf","source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"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":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Front Neurol","raw_type":"Text"},{"id":"pmid:39087003","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/39087003","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,"listed_in":[],"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":"Frontiers in neurology","raw_type":"Journal Article"},{"id":"pmh:oai:doaj.org/article:453107f7fbc8435bbb65f251325a95a5","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/453107f7fbc8435bbb65f251325a95a5","pdf_url":null,"source":{"id":"https://openalex.org/S4306401280","display_name":"DOAJ (DOAJ: Directory of Open Access Journals)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-sa","license_id":"https://openalex.org/licenses/cc-by-sa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Frontiers in Neurology, Vol 15 (2024)","raw_type":"article"}],"best_oa_location":{"id":"doi:10.3389/fneur.2024.1452409","doi":"https://doi.org/10.3389/fneur.2024.1452409","is_oa":true,"landing_page_url":"https://doi.org/10.3389/fneur.2024.1452409","pdf_url":"https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2024.1452409/pdf","source":{"id":"https://openalex.org/S96975567","display_name":"Frontiers in Neurology","issn_l":"1664-2295","issn":["1664-2295"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","jufo-1","medline","norway-1","poland-100","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310320527","host_organization_name":"Frontiers Media","host_organization_lineage":["https://openalex.org/P4310320527"],"host_organization_lineage_names":["Frontiers Media"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Frontiers in Neurology","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.9476000070571899,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[{"id":"https://openalex.org/G9000896626","display_name":"Genetic and Molecular Mechanisms of hypermobile Ehlers Danlos Syndrome","funder_award_id":"F32AI181339","funder_id":"https://openalex.org/F4320337355","funder_display_name":"National Institute of Allergy and Infectious Diseases"}],"funders":[{"id":"https://openalex.org/F4320309312","display_name":"Fullerton College Foundation","ror":"https://ror.org/057bq1s94"},{"id":"https://openalex.org/F4320332161","display_name":"National Institutes of Health","ror":"https://ror.org/01cwqze88"},{"id":"https://openalex.org/F4320337355","display_name":"National Institute of Allergy and Infectious Diseases","ror":"https://ror.org/043z4tv69"}],"has_content":{"grobid_xml":true,"pdf":true},"content_urls":{"pdf":"https://content.openalex.org/works/W4400740258.pdf","grobid_xml":"https://content.openalex.org/works/W4400740258.grobid-xml"},"referenced_works_count":46,"referenced_works":["https://openalex.org/W410379109","https://openalex.org/W1578144316","https://openalex.org/W1973939411","https://openalex.org/W1994171714","https://openalex.org/W2019252616","https://openalex.org/W2021662231","https://openalex.org/W2043979674","https://openalex.org/W2046057151","https://openalex.org/W2063219307","https://openalex.org/W2065890481","https://openalex.org/W2067935721","https://openalex.org/W2094815411","https://openalex.org/W2100968062","https://openalex.org/W2143156113","https://openalex.org/W2163204421","https://openalex.org/W2405367022","https://openalex.org/W2414741881","https://openalex.org/W2502367267","https://openalex.org/W2505025059","https://openalex.org/W2590724600","https://openalex.org/W2597004434","https://openalex.org/W2743022328","https://openalex.org/W2761246502","https://openalex.org/W2887308143","https://openalex.org/W2904598213","https://openalex.org/W2911609224","https://openalex.org/W2911889662","https://openalex.org/W2968654896","https://openalex.org/W2986786777","https://openalex.org/W2987538608","https://openalex.org/W3033104124","https://openalex.org/W3041452159","https://openalex.org/W3116107586","https://openalex.org/W3155560047","https://openalex.org/W3183553035","https://openalex.org/W3183974162","https://openalex.org/W3200709465","https://openalex.org/W4221129484","https://openalex.org/W4224222114","https://openalex.org/W4285040864","https://openalex.org/W4320076980","https://openalex.org/W4327545334","https://openalex.org/W4381954552","https://openalex.org/W4390698853","https://openalex.org/W4396929563","https://openalex.org/W4402464530"],"related_works":["https://openalex.org/W3106789862","https://openalex.org/W4415722440","https://openalex.org/W2590724600","https://openalex.org/W3161259866","https://openalex.org/W4387672656","https://openalex.org/W2167604858","https://openalex.org/W7143394354","https://openalex.org/W7097374056","https://openalex.org/W2270661542","https://openalex.org/W3155560047"],"abstract_inverted_index":{"Collagen,":[0],"the":[1,6,13,23,45,48,57,62,67,133,172,179],"most":[2,136],"abundant":[3],"protein":[4],"in":[5,22,31,66,73,130],"body,":[7],"is":[8],"a":[9,19,52,120],"key":[10],"component":[11],"of":[12,27,47,85,122],"extracellular":[14,59],"matrix":[15],"(ECM),":[16],"which":[17],"plays":[18],"crucial":[20],"role":[21],"structure":[24],"and":[25,43,82,95,112,132,166,178,192],"support":[26],"connective":[28,35,189],"tissues.":[29],"Abnormalities":[30],"collagen":[32,131],"associated":[33,187],"with":[34,188],"tissue":[36,190],"disorders":[37],"(CTD)":[38],"can":[39,69],"lead":[40],"to":[41,77,101],"neuroinflammation":[42],"weaken":[44],"integrity":[46],"blood-brain":[49],"barrier":[50],"(BBB),":[51],"semi-permeable":[53],"membrane":[54],"that":[55,125,145],"separates":[56],"brain's":[58],"fluid":[60,168],"from":[61,71,127],"bloodstream.":[63],"This":[64],"compromise":[65],"BBB":[68,180],"result":[70,126],"disruptions":[72],"ECM":[74],"components,":[75],"leading":[76],"neuroinflammatory":[78],"responses,":[79],"neuronal":[80],"damage,":[81],"increased":[83],"risks":[84],"neurological":[86,98,161,185],"disorders.":[87],"These":[88],"changes":[89],"impact":[90],"central":[91],"nervous":[92],"system":[93],"homeostasis":[94],"may":[96],"exacerbate":[97],"conditions":[99],"linked":[100],"CTD,":[102],"manifesting":[103],"as":[104,158,160],"cognitive":[105],"impairment,":[106],"sensory":[107],"disturbances,":[108],"headaches,":[109],"sleep":[110],"issues,":[111],"psychiatric":[113],"symptoms.":[114],"The":[115,135],"Ehlers-Danlos":[116],"syndromes":[117],"(EDS)":[118],"are":[119],"group":[121],"heritable":[123],"CTDs":[124],"varying":[128],"defects":[129],"ECM.":[134],"prevalent":[137],"subtype,":[138],"hypermobile":[139],"EDS":[140],"(hEDS),":[141],"involves":[142],"clinical":[143],"manifestations":[144,162],"include":[146],"joint":[147],"hypermobility,":[148],"skin":[149],"hyperextensibility,":[150],"autonomic":[151],"dysfunction,":[152],"mast":[153],"cell":[154],"activation,":[155],"chronic":[156,164],"pain,":[157],"well":[159],"like":[163],"headaches":[165],"cerebrospinal":[167],"(CSF)":[169],"leaks.":[170],"Understanding":[171],"connections":[173],"between":[174],"collagen,":[175],"CSF,":[176],"inflammation,":[177],"could":[181],"provide":[182],"insights":[183],"into":[184],"diseases":[186],"abnormalities":[191],"guide":[193],"future":[194],"research.":[195]},"counts_by_year":[{"year":2026,"cited_by_count":11},{"year":2025,"cited_by_count":10},{"year":2024,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2024-07-18T00:00:00"}