Skip to main content Accessibility help
×
Home

Increased Plasma Catecholamines in Patients with Friedreich’s Ataxia

  • A. Pasternac (a1), P. Wagniart (a1), R. Olivenstein (a1), R. Petitclerc (a1), R. Krol (a1), E. Andermann (a1), S. Melancon (a1), G. Geoffroy (a1), J. de Champlain (a1) and A. Barbeau (a1)...

Summary:

We studied free plasma catecholamines in 23 patients with Friedreich’s ataxia, having a mean age of 22 ± 9.6 (SD) years. Conjugated catecholamines were also studied in 10 patients. Mean plasma norepinephrine and epinephrine were significantly higher than controls both in the supine and standing positions. In total 15 out of 23 patients (65%) had increased free and/or conjugated plasma catecholamines. The increase in plasma catecholamines was more marked in patients with severe neuromotor impairment. Among the patients with left ventricular concentric hypertrophy (wall thickness >12 mm), only 3 had no demonstrable sympathetic hyperfunction.

Since the high local concentrations of norepinephrine at the site of release from sympathetic nerve terminals may serve as a trigger for the hypertrophic response of the myocardial cell, it is suggested that early pharmacological intervention could prevent or limit the cardiomyopathic process or its clinical consequences.

    • Send article to Kindle

      To send this article to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about sending to your Kindle. Find out more about sending to your Kindle.

      Note you can select to send to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

      Find out more about the Kindle Personal Document Service.

      Increased Plasma Catecholamines in Patients with Friedreich’s Ataxia
      Available formats
      ×

      Send article to Dropbox

      To send this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Dropbox.

      Increased Plasma Catecholamines in Patients with Friedreich’s Ataxia
      Available formats
      ×

      Send article to Google Drive

      To send this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Google Drive.

      Increased Plasma Catecholamines in Patients with Friedreich’s Ataxia
      Available formats
      ×

Copyright

Corresponding author

Clinical Research Institute of Montreal, 110 West Pine Avenue West, Montréal, Quebec, Canada H2W 1R7

References

Hide All
Alousi, A.A. and Beards, J.A. (1972). Catecholamine, protein and RNA content in advanced congestive heart failure in the Syrian hamster. In: Recent Advances in Studies on Cardiac Structure and Metabolism. Bajusz, E. and Rona, G., editors. University Park Press, Baltimore. Vol. 1, pp. 279288.
Angelakos, E.T.Carballo, L.C., Daniels, J.B., King, M.B. and Bajusz, E. (1972). Adrenergic neurohumors in the heart of hamsters with hereditary myopathy during cardiac hypertrophy and failure. In: Recent Advances in Studies on Cardiac Structure and Metabolism. Bajusz, E. and Rona, G., editors. University Park Press, Baltimore. Vol. 1, pp. 288299.
Angelakos, E.T.King, M.B. and Carballo, L. (1973). Cardiac adrenergic innervation in hamsters with hereditary myocardiopathy: chemical and histochemical studies. In: Recent Advances in Studies on Cardiac Structure and Metabolism. Bajusz, E. and Rona, G., editors. University Park Press, Baltimore. Vol. 2, pp. 519531.
Azari, J., Reiusine, T., Barbeau, A., Yamamura, H.I. and Huxtable, R. (1979). The Syrian Golden hamster: A model for the cardiomyopathy of Friedreich’s ataxia. Can. J. Neurol. Sci. 6: 223226.
Bajusz, E., Homburger, F., Baker, J.R. and Opie, L.H. (1966). The heart muscle in muscular dystrophy with special reference to involvement of the cardiovascular system in the hereditary myopathy of the hamster. Ann. N.Y. Acad. Sci. 138:213229.
Cohen, J. (1974). Role of endocrine factors in the pathogenesis of cardiac hypertrophy. Circ. Res. 35 (Suppl II): 4957.
Cote, M., Davignon, A., Peckodrouin, K., Solignac, A., Geoffroy, G., Lemieux, B. and Barbeau, A. (1976). Cardiological signs and symptoms in Friedreich’s ataxia. Can. J. Neurol. Sci. 3:319321.
Davies, A.O., De Lean, A. and Lefkowitz, R.J. (1981). Myocardial beta-adrenergic receptors from adrenalectomized rats: impaired formation of high-affinity agonist-receptor complexes. Endocrinology 108:720722.
Goldstein, D.S. (1981). Plasma norepinephrine activity in clinical cardiology. Am. J. Cardiol. 48: 11471154.
Goodwin, J.F. (1974). Prospects and predictions for the cardiomyopathies. Circulation 50:210219.
Goodwin, J.F. and Krikler, D.M. (1976). Hypothesis: arrhythmia as a cause of sudden death in hypertrophic cardiomyopathy. Lancet 2: 937940.
Gottdiener, J.S., Hawley, R.J.. Marón, B.J., Bertorini, T.F. and Engel, W.K. (1979). Hypertrophic cardiomyopathy in Friedreich’s ataxia. Circulation 60 (Suppl II): 242.
Hartman, J.M. and Booth, R.W. (1960). Friedreich’s ataxia: a neurocardiac disease. Am. Heart J. 60: 716720.
Johnson, G.A., Baker, C.A. and Smith, R.T. (1980). Radioenzymatic assay of sulfate conjugates of catecholamines and dopa in plasma. Life Sci. 26: 15911598.
Kafka, M., Tallman, J.F., Smith, C.C. and Costa, J.L. (1977). Alpha-adrenergic receptors in human platelets. Life Sci. 21: 14291438.
Karliner, J.S., Alabaster, C., Stephens, H., Barnes, P. and Dollery, C. (1981). Enhanced noradrenaline response in cardiomyopathic hamsters: possible relation to changes in adrenoceptors studied by radioligand binding. Cardiovasc. Res. 15:296304.
Kopin, I., Mccarty, R., Torda, T. and Yamaguchi, I. (1980). Catecholamines in plasma and response to stress. In: Catecholamines and Stress: recent advances. Usdin, Koetnansky and Kopin, editors. Elsevier North Holland, pp. 197204.
Kuchel, O., Buu, N.T., Unger, T. and Genest, J. (1978). Free and conjugated catecholamines in human hypertension. Clin. Sci. Mol. Med. 55 (Suppl 4): 77S–80S.
Lake, C.R., Ziegler, M., Coleman, M. and Kopin, I.J. (1980). Effect of clonidine on sympathetic nervous system function in hypertensive patients (abs). The Pharmacologist 20: 3.
Laks, M.M., Morady, F. and Swan, H. J.C. (1973). Myocardial hypertrophy produced by chronic infusion of subhypertensive doses of norepinephrine in the dog. Chest 65: 7578.
Ledda, F., Marchetti, P. and Mucelli, A. (1975). Studies on the positive inotropic effect of phenylephrine: a comparison with isoprenaline. Br. J. Pharmacol. 54:8390.
Loiseau, J. (1938). Les troubles cardiaques dans la maladie de Friedreich. Thèse, Paris.
Lorell, B.H., Paulus, W.J., Grossman, W., Wynne, J. and Cohn, P.F. (1982). Modification of abnormal left ventricular diastolic properties by nifedipine in patients with hypertrophic cardiomyopathy. Circulation 65: 499507.
Lossnitzer, K., Janke, J., Hein, B., Stauch, M. and Fleckenstein, A. (1975). Disturbed myocardial calcium metabolism: a possible pathogenetic factor in the hereditary cardiomyopathy of the Syrian hamster. In: Recent Advances in Studies on Cardiac Structure and Metabolism. Fleckenstein, A. and Rona, G., editors. University Park Press, Baltimore. Vol.6.
Mallani, A., Recordati, G. and Schwartz, P.J. (1973). Nervous activity of afferent cardiac sympathetic fibers with atrial and ventricular endings. J. Physiol. (London) 229: 457469.
Micalizzi, E.R. and Pals, D.T. (1979). Evaluation of plasma norepinephrine as an index of sympathetic neuron function in the conscious, unstrained rat. Life Sci. 24: 20712076.
Ostman-Smith, I. (1976). Prevention of exercise-induced cardiac hypertrophy in rats by chemical sympathectomy (guanethidine treatment). Neuroscience 1: 497507.
Ostman-Smith, I. (1979). Adaptive changes in the sympathetic nervous system and some effector organs of the rat following long-term exercise or cold acclimation and the role of cardiac sympathetic nerves in the genesis of compensatory hypertrophy. Acta Physiol. Scand. Suppl. 477: 1118.
Ostman-Smith, I. (1981). Cardiac sympathetic nerves as the final common pathway in the induction of adaptive cardiac hypertrophy. Clin. Sci. 61: 265272.
Pasternac, A., Król, R., Petitclerc, R., Harvey, C., Andermann, E. and Barbeau, A. (1980). Hypertrophic cardiomyopathy in Friedreich’s ataxia: symmetric or asymmetric? Can. J. Neurol. Sci. 7:379382.
Pasternac, A., Noble, J., Streulens, Y., Elie, R., Henschke, C. and Bourassa, M.G. (1982). Pathophysiology of chest pain in patients with cardiomyopathies and normal coronary arteries. Circulation 65: 201213.
Perloff, J.K. (1981). Pathogenesis of hypertrophic cardiomyopathy: hypotheses and speculation. Am. Heart J. 101: 219226.
Peuler, J.D. and Johnson, G.A. (1977). Simultaneous single isotope radioenzymatic assay of plasma norepinephrine, epinephrine and dopamine. Life Sci. 21: 625636.
Pourcher, E. and Barbeau, A. (1980). Field testing of an ataxia scoring and staging system. J. Can. Neurol. Sci. 7: 339344.
Rosing, D.R., Kent, K.M., Borer, J.S., Seides, S.F., Maron, B.J. and Epstein, S.E. (1979). Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy. I. Hemodynamic effects. Circulation 60: 12011207.
Sauebier, I. and Von Mayers Bach, H. (1977). Circadian variation of catecholamines in human blood. Horm. Metab. Res. 9: 529530.
Silverberg, A.B., Shah, S.D., Haymond, M.W. and Cryer, P.E. (1978). Norepinephrine: hormone and neurotransmitter in man. Am. J. Physiol. 234: E252E256.
Sole, M.J., Wurtman, R.J., Lo, C.M., Kamble, A.B. and Sonnenblick, E.H. (1977). Tyrosine hydroxylase activity in the heart of the cardiomyopathic Syrian hamster. J. Mol. Cell. Cardiol. 9: 225233.
St-John Sutton, M.G.. Olukotun, A.Y., Tajik, A.J., Lovett, J.L. and Giuliani, A. (1980). Left ventricular function in Friedreich’s ataxia. Br. Heart J. 44:309316.
Stanton, H.C., Brenner, G. and Mayfield, E.D. (1969). Studies on isoproterenol-induced cardiomegaly in rats. Am. Heart J. 77: 7280.
Thoren, C. (1964). Cardiomyopathy in Friedreich’s ataxia: with studies of cardiovascular and respiratory functions. Acta Paediat. 53 (Suppl 153): 3136.
Vaughan Williams, E.M., Raine, A.E.G., Cabrera, A.A., and Whyte, J.M. (1975). The effects of prolonged β-adrenoreceptor blockade on heart weight and cardiac intracellular potentials in rabbits. Cardiovasc. Res. 9: 579592.
Williams, L.T., Snyderman, R. and Lefkowitz, R.J. (1976). Identification of β-adrenergic receptors in human lymphocytes by (-) [3H] alprenolol binding. J. Clin. Invest. 57: 149155.

Increased Plasma Catecholamines in Patients with Friedreich’s Ataxia

  • A. Pasternac (a1), P. Wagniart (a1), R. Olivenstein (a1), R. Petitclerc (a1), R. Krol (a1), E. Andermann (a1), S. Melancon (a1), G. Geoffroy (a1), J. de Champlain (a1) and A. Barbeau (a1)...

Metrics

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed