{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T19:31:46Z","timestamp":1780947106312,"version":"3.54.1"},"reference-count":30,"publisher":"Cambridge University Press (CUP)","issue":"2","license":[{"start":{"date-parts":[[2015,9,18]],"date-time":"2015-09-18T00:00:00Z","timestamp":1442534400000},"content-version":"unspecified","delay-in-days":13289,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Can. j. neurol. sci."],"published-print":{"date-parts":[[1979,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Friedreich's Ataxia (F.A.) is a degenerative disease which commonly leads to premature death of cardio-respiratory origin. To explain the early death of these patients, previous investigations have established the existence of I) a cardiomyopathy in nearly 100% of cases, 2) a restrictive pulmonary syndrome of scoliotic origin and 3) a mild hypoxemia associated with slight respiratory alkalosis and a normal oxyhemoglobin dissociation curve. To further assess the cause of early death in patients with such neuromyo-pathy, we evaluated, in eleven FA. patients, the sensitivity of the respiratory centers to hypercapnia, hypoxia, and hyperoxia.<\/jats:p><jats:p>Ventilatory (V<jats:sub>E<\/jats:sub>, V<jats:sub>T<\/jats:sub>, F, V<jats:sub>T<\/jats:sub>\/T<jats:sub>i<\/jats:sub>) and occlusion pressure (P<jats:sub>0.1<\/jats:sub>) responses were taken as indices of the respiratory centers output during progressive hypercapnia (Read's method) and isocarhic hypoxia (Weil's method). We studied l\u00ec Friedreich's Ataxia patients and II age, sex, and arm-span matched controls. The responses of patients to hypercapnia were significantly greater than controls hut their responses to hypoxia were similar to controls.<\/jats:p><jats:p>Our study establishes that the respiratory centers are functioning adequately in early Friedreich's Ataxia and do not contribute to cardio-respiratory insufficiency in such neuromyopathy.<\/jats:p>","DOI":"10.1017\/s0317167100119572","type":"journal-article","created":{"date-parts":[[2016,7,28]],"date-time":"2016-07-28T11:33:04Z","timestamp":1469705584000},"page":"159-165","source":"Crossref","is-referenced-by-count":8,"title":["Regulation of Respiration in Friedreich's Ataxia"],"prefix":"10.1017","volume":"6","author":[{"given":"R.","family":"Begin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Lupien","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. A.","family":"Bureau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Labbe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Lemieux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2015,9,18]]},"reference":[{"key":"S0317167100119572_ref007","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100025579"},{"key":"S0317167100119572_ref024","doi-asserted-by":"publisher","DOI":"10.1378\/chest.42.5.558"},{"key":"S0317167100119572_ref002","first-page":"199","volume":"38","author":"Begin","year":"1975"},{"key":"S0317167100119572_ref009","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.26.2.140"},{"key":"S0317167100119572_ref028","doi-asserted-by":"publisher","DOI":"10.1172\/JCI106322"},{"key":"S0317167100119572_ref021","doi-asserted-by":"publisher","DOI":"10.1378\/chest.70.1_Supplement.131"},{"key":"S0317167100119572_ref025","doi-asserted-by":"publisher","DOI":"10.1111\/imj.1967.16.1.20"},{"key":"S0317167100119572_ref005","first-page":"641","article-title":"Maximal static respiratory pressures in generalized neuromuscular disease.","volume":"103","author":"Black","year":"1971","journal-title":"Am. 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Dis."},{"key":"S0317167100119572_ref006","first-page":"947","article-title":"Impaired peripheral chemosensitivity and acute respiratory failure in Arnold-Chiari malformation and syringomyelia.","volume":"288","author":"Bokinsky","year":"1973"},{"key":"S0317167100119572_ref008","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1017\/S0317167100024847","article-title":"Oxygen transport in patients with Friedreich's Ataxia.","volume":"5","author":"Bureau","year":"1978","journal-title":"Can. J. Neurol. Sci."},{"key":"S0317167100119572_ref010","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.27.12.1125"},{"key":"S0317167100119572_ref012","first-page":"115","article-title":"Etude de la r\u00e9gulation de la ventilation de repos chez l'homme en haute altitude.","volume":"4","author":"Dejours","year":"1959","journal-title":"Rev. Franc. Etudes Clin"},{"key":"S0317167100119572_ref013","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.1962.42.3.335"},{"key":"S0317167100119572_ref014","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1963.tb13497.x"},{"key":"S0317167100119572_ref015","first-page":"317","article-title":"Respiratory failure as a \u2018complication of neuromuscular disease.","volume":"17","author":"Hall","year":"1977","journal-title":"Adv. Neurol."},{"key":"S0317167100119572_ref017","first-page":"379","author":"Kellogg","year":"1963"},{"key":"S0317167100119572_ref020","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(68)90088-0"},{"key":"S0317167100119572_ref022","first-page":"1029","volume":"293","author":"Milic-Emili","year":"1975"},{"key":"S0317167100119572_ref023","doi-asserted-by":"crossref","first-page":"119","DOI":"10.7326\/0003-4819-46-1-119","article-title":"Exertional dyspnea: a primary complaint in unusual cases of progressive muscular atrophy and amyotrophic lateral sclerosis.","volume":"46","author":"Miller","year":"1957","journal-title":"Ann. Int. Med."},{"key":"S0317167100119572_ref026","article-title":"Statistical methods (6th ed.) Arnes, Iowa: Iowa State U niv.","author":"Snedecor","year":"1967"},{"key":"S0317167100119572_ref029","doi-asserted-by":"publisher","DOI":"10.1016\/0034-5687(75)90059-6"},{"key":"S0317167100119572_ref030","doi-asserted-by":"publisher","DOI":"10.1152\/jappl.1969.26.3.308"},{"key":"S0317167100119572_ref027","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-83-4-476"},{"key":"S0317167100119572_ref018","first-page":"235","article-title":"Maximal oxygen uptake lung volume and ventil\u00e1tory response to carbon dioxide and hypoxia in a pair of identical twin athletes.","volume":"48","author":"Leitch","year":"1975","journal-title":"Clin. Sci. Mol. 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