{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T20:04:43Z","timestamp":1789502683124,"version":"build-2803163510"},"reference-count":52,"publisher":"Georg Thieme Verlag KG","issue":"05","funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["NS065736\u201307"],"award-info":[{"award-number":["NS065736\u201307"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100001594","name":"Dysautonomia Foundation Inc.","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100001594","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Neurol"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Head and neck tumors can affect afferent baroreceptor neurons and either interrupt or intermittently increase their signaling, causing blood pressure to become erratic. When the afferent fibers of the baroreflex are injured by surgery or radiotherapy or fail to develop as in familial dysautonomia, their sensory information is no longer present to regulate arterial blood pressure, resulting in afferent baroreflex failure. When the baroreflex afferents are abnormally activated, such as by paragangliomas in the neck, presumably by direct compression, they trigger acute hypotension and bradycardia and frequently syncope, by a mechanism similar to the carotid sinus syndrome. We describe our observations in a large series of 23 patients with afferent baroreflex dysfunction and the cardiovascular autonomic features that arise when the sensory baroreceptor neurons are injured or compressed. The management of afferent baroreceptor dysfunction is limited, but pharmacological strategies can mitigate blood pressure swings, improve symptoms, and may reduce hypertensive organ damage. Although rare, the prevalence of afferent baroreflex dysfunction appears to be increasing in middle-aged men due to human papillomavirus related oropharyngeal cancer.<\/jats:p>","DOI":"10.1055\/s-0040-1713892","type":"journal-article","created":{"date-parts":[[2020,9,9]],"date-time":"2020-09-09T23:03:41Z","timestamp":1599692621000},"page":"540-549","source":"Crossref","is-referenced-by-count":13,"title":["Afferent Baroreflex Dysfunction: Decreased or Excessive Signaling Results in Distinct Phenotypes"],"prefix":"10.1055","volume":"40","author":[{"given":"Lucy","family":"Norcliffe-Kaufmann","sequence":"additional","affiliation":[{"name":"Department of Neurology, New York University School of Medicine, New York, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricio","family":"Millar Vernetti","sequence":"additional","affiliation":[{"name":"Department of Neurology, New York University School of Medicine, New York, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose-Alberto","family":"Palma","sequence":"additional","affiliation":[{"name":"Department of Neurology, New York University School of Medicine, New York, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bhumika J.","family":"Balgobin","sequence":"additional","affiliation":[{"name":"Department of Neurology, New York University School of Medicine, New York, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Horacio","family":"Kaufmann","sequence":"additional","affiliation":[{"name":"Department of Neurology, New York University School of Medicine, New York, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2020,9,9]]},"reference":[{"issue":"20","key":"ref1","doi-asserted-by":"crossref","first-page":"1449","DOI":"10.1056\/NEJM199311113292003","article-title":"The diagnosis and treatment of baroreflex failure","volume":"329","author":"D Robertson","year":"1993","journal-title":"N Engl J Med"},{"issue":"03","key":"ref2","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1212\/WNL.0000000000000605","article-title":"Neural control of the heart: recent concepts and clinical correlations","volume":"83","author":"J A Palma","year":"2014","journal-title":"Neurology"},{"issue":"21","key":"ref3","doi-asserted-by":"crossref","first-page":"1904","DOI":"10.1212\/WNL.0b013e3181feb283","article-title":"Afferent baroreflex failure in familial dysautonomia","volume":"75","author":"L Norcliffe-Kaufmann","year":"2010","journal-title":"Neurology"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1126\/science.aau6324","article-title":"PIEZOs mediate neuronal sensing of blood pressure and the baroreceptor reflex","volume":"362","author":"W Z Zeng","year":"2018","journal-title":"Science"},{"issue":"02","key":"ref5","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/S0301-0082(97)00003-8","article-title":"Neurochemistry of the nodose ganglion","volume":"52","author":"H Zhuo","year":"1997","journal-title":"Prog Neurobiol"},{"issue":"01","key":"ref6","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1038\/jhh.2011.107","article-title":"Developmental abnormalities, blood pressure variability and renal disease in Riley Day syndrome","volume":"27","author":"L Norcliffe-Kaufmann","year":"2013","journal-title":"J Hum Hypertens"},{"issue":"02","key":"ref7","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1113\/jphysiol.1972.sp009861","article-title":"The role of the solitary and paramedian reticular nuclei in mediating cardiovascular reflex responses from carotid baro- and chemoreceptors","volume":"223","author":"M Miura","year":"1972","journal-title":"J Physiol"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1111\/j.1749-6632.2001.tb03681.x","article-title":"Excitatory inputs to the RVLM in the context of the baroreceptor reflex","volume":"940","author":"A F Sved","year":"2001","journal-title":"Ann N Y Acad Sci"},{"issue":"03","key":"ref9","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1152\/physrev.1991.71.3.617","article-title":"Reflexes from the heart","volume":"71","author":"R Hainsworth","year":"1991","journal-title":"Physiol Rev"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1113\/jphysiol.1979.sp012612","article-title":"Carotid and cardiopulmonary baroreceptor control of splanchnic and forearm vascular resistance during venous pooling in man","volume":"286","author":"F M Abboud","year":"1979","journal-title":"J Physiol"},{"key":"ref11","first-page":"384","article-title":"Aortic baroreceptors display higher mechanosensitivity than carotid baroreceptors","volume":"7","author":"E O Lau","year":"2016","journal-title":"Front Physiol"},{"issue":"02","key":"ref12","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1113\/expphysiol.2013.072637","article-title":"Cardiovascular control from cardiac and pulmonary vascular receptors","volume":"99","author":"R Hainsworth","year":"2014","journal-title":"Exp Physiol"},{"issue":"06","key":"ref13","doi-asserted-by":"crossref","first-page":"R1807","DOI":"10.1152\/ajpregu.00082.2008","article-title":"Role of cardiopulmonary and carotid sinus baroreceptors in regulating renal sympathetic nerve activity during water immersion in conscious dogs","volume":"296","author":"K Miki","year":"2009","journal-title":"Am J Physiol Regul Integr Comp Physiol"},{"issue":"03","key":"ref14","first-page":"H299","article-title":"Cardiopulmonary receptors with vagal afferents tonically inhibit ADH release in the dog","volume":"237","author":"M D Thames","year":"1979","journal-title":"Am J Physiol"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/0002-9343(88)90212-4","article-title":"Cardiopulmonary receptor regulation of renin release","volume":"84","author":"G Grassi","year":"1988","journal-title":"Am J Med"},{"issue":"04","key":"ref16","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1161\/01.RES.50.4.554","article-title":"Differential baroreflex control of heart rate and vascular resistance in rabbits. Relative role of carotid, aortic, and cardiopulmonary baroreceptors","volume":"50","author":"G B Guo","year":"1982","journal-title":"Circ Res"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1093\/brain\/108.2.423","article-title":"Sympathetic outflow in man after anaesthesia of the glossopharyngeal and vagus nerves","volume":"108","author":"J Fagius","year":"1985","journal-title":"Brain"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/j.pneurobio.2016.06.003","article-title":"Familial dysautonomia: history, genotype, phenotype and translational research","volume":"152","author":"L Norcliffe-Kaufmann","year":"2017","journal-title":"Prog Neurobiol"},{"issue":"02","key":"ref19","first-page":"164","article-title":"Convulsive syncope as presenting symptom of carotid body tumors: case series","volume":"18","author":"A A Beigi","year":"2013","journal-title":"J Res Med Sci"},{"issue":"02","key":"ref20","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1007\/s10286-004-0165-3","article-title":"Long-term effects of unilateral carotid endarterectomy on arterial baroreflex function","volume":"14","author":"H J Timmers","year":"2004","journal-title":"Clin Auton Res"},{"issue":"01","key":"ref21","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1007\/s10286-015-0323-9","article-title":"Mother-induced hypertension in familial dysautonomia","volume":"26","author":"L Norcliffe-Kaufmann","year":"2016","journal-title":"Clin Auton Res"},{"issue":"11","key":"ref22","doi-asserted-by":"crossref","first-page":"1269","DOI":"10.1113\/EP085340","article-title":"Chewing-induced hypertension in afferent baroreflex failure: a sympathetic response?","volume":"100","author":"C Fuente Mora","year":"2015","journal-title":"Exp Physiol"},{"issue":"02","key":"ref23","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1097\/MCG.0b013e3182582cbf","article-title":"Cyclic vomiting associated with excessive dopamine in Riley-day syndrome","volume":"47","author":"L J Norcliffe-Kaufmann","year":"2013","journal-title":"J Clin Gastroenterol"},{"issue":"17","key":"ref24","doi-asserted-by":"crossref","first-page":"1611","DOI":"10.1212\/WNL.0b013e31828f18f0","article-title":"Hyperdopaminergic crises in familial dysautonomia: a randomized trial of carbidopa","volume":"80","author":"L Norcliffe-Kaufmann","year":"2013","journal-title":"Neurology"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1113\/jphysiol.2003.052415","article-title":"Denervation of carotid baro- and chemoreceptors in humans","volume":"553","author":"H J Timmers","year":"2003","journal-title":"J Physiol"},{"issue":"01","key":"ref26","first-page":"14","article-title":"Fatal hypertensive crisis following denervation of the carotid sinus for the relief of repeated attacks of syncope; case history","volume":"100","author":"F R Ford","year":"1957","journal-title":"Bull Johns Hopkins Hosp"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1196\/annals.1296.063","article-title":"Cardiovascular responses to stress after carotid baroreceptor denervation in humans","volume":"1018","author":"H J Timmers","year":"2004","journal-title":"Ann N Y Acad Sci"},{"issue":"03","key":"ref28","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1002\/ajmg.a.20686","article-title":"Severe congenital myopathy with M\u00f6bius, Robin, and Poland sequences: new aspects of the Carey-Fineman-Ziter syndrome","volume":"127A","author":"A Dufke","year":"2004","journal-title":"Am J Med Genet A"},{"issue":"09","key":"ref29","doi-asserted-by":"crossref","first-page":"3226","DOI":"10.1002\/hed.25810","article-title":"Projected oropharyngeal carcinoma incidence among middle-aged US men","volume":"41","author":"L Xu","year":"2019","journal-title":"Head Neck"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.autneu.2019.02.003","article-title":"Chemoreflex failure and sleep-disordered breathing in familial dysautonomia: implications for sudden death during sleep","volume":"218","author":"J A Palma","year":"2019","journal-title":"Auton Neurosci"},{"issue":"05","key":"ref32","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1161\/01.HYP.0000160355.93303.72","article-title":"Baroreflex failure","volume":"45","author":"K Heusser","year":"2005","journal-title":"Hypertension"},{"issue":"05","key":"ref33","doi-asserted-by":"crossref","first-page":"1072","DOI":"10.1161\/01.HYP.30.5.1072","article-title":"Malignant vagotonia due to selective baroreflex failure","volume":"30","author":"J Jordan","year":"1997","journal-title":"Hypertension"},{"issue":"07","key":"ref34","doi-asserted-by":"crossref","first-page":"2414","DOI":"10.1177\/0271678X16667524","article-title":"Cerebral autoregulation and symptoms of orthostatic hypotension in familial dysautonomia","volume":"37","author":"C Fuente Mora","year":"2017","journal-title":"J Cereb Blood Flow Metab"},{"issue":"01","key":"ref35","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s10286-010-0113-3","article-title":"Afferent baroreflex failure and tako-tsubo cardiomyopathy","volume":"21","author":"L J Norcliffe-Kaufmann","year":"2011","journal-title":"Clin Auton Res"},{"issue":"03","key":"ref36","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1002\/ana.25170","article-title":"Orthostatic heart rate changes in patients with autonomic failure caused by neurodegenerative synucleinopathies","volume":"83","author":"L Norcliffe-Kaufmann","year":"2018","journal-title":"Ann Neurol"},{"issue":"06","key":"ref37","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1002\/mds.25482","article-title":"Relationship between proprioception at the knee joint and gait ataxia in HSAN III","volume":"28","author":"V G Macefield","year":"2013","journal-title":"Mov Disord"},{"issue":"18","key":"ref38","doi-asserted-by":"crossref","first-page":"2653","DOI":"10.1517\/14656566.2014.970530","article-title":"Current treatments in familial dysautonomia","volume":"15","author":"J A Palma","year":"2014","journal-title":"Expert Opin Pharmacother"},{"issue":"01","key":"ref39","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1161\/01.HYP.0000077441.45309.08","article-title":"Baroreflex failure as a late sequela of neck irradiation","volume":"42","author":"Y Sharabi","year":"2003","journal-title":"Hypertension"},{"issue":"04","key":"ref40","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1111\/joim.12042","article-title":"At the heart of the arterial baroreflex: a physiological basis for a new classification of carotid sinus hypersensitivity","volume":"273","author":"W Wieling","year":"2013","journal-title":"J Intern Med"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1016\/j.avsg.2015.12.029","article-title":"Management of carotid bifurcation tumors: 30-year experience","volume":"34","author":"J I Torrealba","year":"2016","journal-title":"Ann Vasc Surg"},{"issue":"09","key":"ref42","doi-asserted-by":"crossref","first-page":"2403","DOI":"10.1007\/s00405-014-3141-0","article-title":"Management of vagal paragangliomas: review of 17 patients","volume":"272","author":"R Gonz\u00e1lez-Or\u00fas \u00c1lvarez-Morujo","year":"2015","journal-title":"Eur Arch Otorhinolaryngol"},{"issue":"03","key":"ref43","doi-asserted-by":"crossref","first-page":"122","DOI":"10.4103\/0975-3583.70905","article-title":"Diagnosis and surgical treatment of carotid body tumor: a report of 18 cases","volume":"1","author":"D Ma","year":"2010","journal-title":"J Cardiovasc Dis Res"},{"issue":"08","key":"ref44","doi-asserted-by":"crossref","first-page":"1068","DOI":"10.1007\/s00246-009-9497-z","article-title":"Echocardiographic abnormalities in familial dysautonomia","volume":"30","author":"U Nussinovitch","year":"2009","journal-title":"Pediatr Cardiol"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"1704","DOI":"10.1016\/S0140-6736(05)77741-2","article-title":"Intraocular pressure and baroreflex failure","volume":"351","author":"K M Joos","year":"1998","journal-title":"Lancet"},{"issue":"04","key":"ref46","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1016\/S0002-8703(77)80044-6","article-title":"Myocardial infarction association with the Riley-Day syndrome","volume":"94","author":"R Reshef","year":"1977","journal-title":"Am Heart J"},{"issue":"01","key":"ref47","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1007\/s10286-016-0383-5","article-title":"Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia","volume":"27","author":"R C Dillon","year":"2017","journal-title":"Clin Auton Res"},{"issue":"23","key":"ref48","doi-asserted-by":"crossref","first-page":"2939","DOI":"10.1016\/j.jacc.2019.10.027","article-title":"Blood pressure management in afferent baroreflex failure: JACC Review Topic of the Week","volume":"74","author":"I Biaggioni","year":"2019","journal-title":"J Am Coll Cardiol"},{"issue":"08","key":"ref49","first-page":"zsx083","article-title":"Sudden unexpected death during sleep in familial dysautonomia: a case-control study","volume":"40","author":"J A Palma","year":"2017","journal-title":"Sleep (Basel)"},{"issue":"01","key":"ref50","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1159\/000329213","article-title":"Management of vagal paragangliomas including application of internal carotid artery stenting","volume":"17","author":"S H Shin","year":"2012","journal-title":"Audiol Neurotol"},{"issue":"02","key":"ref51","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.ejvs.2007.01.015","article-title":"A multicenter review of carotid body tumour management","volume":"34","author":"M S Sajid","year":"2007","journal-title":"Eur J Vasc Endovasc Surg"},{"issue":"02","key":"ref52","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1097\/MOO.0b013e328342b9b4","article-title":"Update on the management of parapharyngeal tumours","volume":"19","author":"P J Bradley","year":"2011","journal-title":"Curr Opin Otolaryngol Head Neck Surg"},{"key":"ref53","first-page":"47","article-title":"An evaluation on management of carotid body tumour (CBT). A twelve years experience","volume":"35","author":"G Boscarino","year":"2014","journal-title":"G Chir"}],"container-title":["Seminars in Neurology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0040-1713892.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,3,31]],"date-time":"2021-03-31T14:41:22Z","timestamp":1617201682000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0040-1713892"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9,9]]},"references-count":52,"journal-issue":{"issue":"05","published-online":{"date-parts":[[2020,10,27]]},"published-print":{"date-parts":[[2020,10]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0040-1713892","relation":{},"ISSN":["0271-8235","1098-9021"],"issn-type":[{"value":"0271-8235","type":"print"},{"value":"1098-9021","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,9,9]]}}}