{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T01:42:53Z","timestamp":1778895773239,"version":"3.51.4"},"reference-count":28,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,11,8]],"date-time":"2024-11-08T00:00:00Z","timestamp":1731024000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Upper cervical instability (UCI) is a potentially disabling complication of the connective tissue disorders hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders (hEDS\/HSD). UCI can impact various neurological structures, including the brainstem, spinal cord, cranial nerves, and blood supply to and from the brain, resulting in complex neurological signs and symptoms in this population. The current study was an observational study applying recent expert consensus recommendations for physical therapy assessment and management of patients with UCI associated with hEDS\/HSD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This was a retrospective observational study describing how the clinical decision-making model was used to screen, examine, and treat three patients with highly irritable hEDS\/HSD-related UCI, resulting in complex neurological presentation. The treatment used a neuroplasticity approach, including proprioception and motor control training emphasizing patient education and biofeedback. Outcome measures tracked progress.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All patients started with significant disability associated with UCI. One patient returned to full function with intermittent flares that he was able to manage. The second patient continued to have mild-moderate irritability but returned to parenting responsibilities and full-time work. The third patient required cervical fusion and remained disabled but was better able to minimize flares. The number of initial red and yellow flags was associated with the final outcomes, suggesting that the decision-making model might be useful for predicting patient prognosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This brief report applies recent recommendations for safely evaluating and managing hypermobility-related UCI and provides a first step in experimental studies to test both the assessment and physical therapy treatment approaches.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fneur.2024.1459115","type":"journal-article","created":{"date-parts":[[2024,11,8]],"date-time":"2024-11-08T05:10:17Z","timestamp":1731042617000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Presentation and physical therapy management using a neuroplasticity approach for patients with hypermobility-related upper cervical instability: a brief report"],"prefix":"10.3389","volume":"15","author":[{"given":"Susan","family":"Chalela","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leslie N.","family":"Russek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,11,8]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"2902","DOI":"10.1002\/ajmg.a.61873","article-title":"Headaches in hypermobility syndromes: A pain in the neck?","volume":"182","author":"Malhotra","year":"2020","journal-title":"Am J Med Genet A."},{"key":"B2","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1002\/ajmg.c.31549","article-title":"Neurological and spinal manifestations of the Ehlers-Danlos syndromes","volume":"175","author":"Henderson","year":"2017","journal-title":"Am J Med Genet C Semin Med Genet."},{"key":"B3","doi-asserted-by":"publisher","first-page":"1072764","DOI":"10.3389\/fmed.2022.1072764","article-title":"Presentation and physical therapy management of upper cervical instability in patients with symptomatic generalized joint hypermobility: International expert consensus recommendations","volume":"9","author":"Russek","year":"2022","journal-title":"Front Med (Lausanne)."},{"key":"B4","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1007\/s10143-023-02249-0","article-title":"Craniocervical instability in patients with Ehlers-Danlos syndromes: outcomes analysis following occipito-cervical fusion","volume":"47","author":"Henderson","year":"2024","journal-title":"Neurosurg Rev."},{"key":"B5","doi-asserted-by":"publisher","first-page":"1944","DOI":"10.1016\/j.spinee.2022.08.008","article-title":"Craniocervical instability in patients with ehlers-danlos syndrome: controversies in diagnosis and management","volume":"22","author":"Mao","year":"2022","journal-title":"Spine J."},{"key":"B6","doi-asserted-by":"publisher","first-page":"21925682211068520","DOI":"10.1177\/21925682211068520","article-title":"Craniocervical instability in ehlers-danlos syndrome-a systematic review of diagnostic and surgical treatment criteria","volume":"2022","author":"Lohkamp","year":"2022","journal-title":"Global Spine J."},{"key":"B7","doi-asserted-by":"publisher","first-page":"24","DOI":"10.4103\/jcvjs.jcvjs_127_22","article-title":"Neurosurgical management of patients with Ehlers-Danlos syndrome: a descriptive case series","volume":"14","author":"Rock","year":"2023","journal-title":"J Craniovertebr Junction Spine."},{"key":"B8","doi-asserted-by":"publisher","first-page":"21925682231151924","DOI":"10.1177\/21925682231151924","article-title":"Outcomes following anterior cervical discectomy and fusion in patients with ehlers-danlos syndrome","volume":"2023","author":"Chi","year":"2023","journal-title":"Global Spine J."},{"key":"B9","doi-asserted-by":"publisher","first-page":"1365712","DOI":"10.3389\/fpubh.2024.1365712","article-title":"Estimates of the excess cost burden of Ehlers-Danlos syndromes: a United States MarketScan\u00ae claims database analysis","volume":"12","author":"Schubart","year":"2024","journal-title":"Front Public Health."},{"key":"B10","doi-asserted-by":"publisher","first-page":"2709","DOI":"10.1007\/s00296-013-2790-4","article-title":"Do people with benign joint hypermobility syndrome (BJHS) have reduced joint proprioception? 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