Clinical Autonomic Research

, Volume 5, Issue 3, pp 165–169 | Cite as

Hyposensitivity of peripheral α-adrenoceptors in respiratordependent amyotrophic lateral sclerosis assessed by intravenous norepinephrine infusion

  • T. Shimizu
  • H. Hayashi
  • M. Hayashi
  • S. Kato
  • H. Tanabe
Research Paper


Intravenous norepinephrine infusion test was performed in eight patients with amyotrophic lateral sclerosis (ALS) supported by respirators and nine control subjects, to examine α-adrenoceptor function of peripheral resistant blood vessels. Baseline plasma norepinephrine concentrations in ALS patients were significantly higher than those in control subjects, indicating basal sympathetic hyperactivity (normal 218.2 ± 59.7 pg/ml; ALS 450.0 ± 288.4 pg/ml). The stimulus-response curves in the patients were similar to those in control subjects, and there were no significant differences between mean gains of the stimulus—response curves in both groups (normal 18.7 ± 5.5; ALS 15.2 ± 11.2). However, three ALS patients, two of whom had circulatory fluctuation and sympathetic hyperactivity, revealed lower gain levels than the mean minus 2 SD in control subjects (4.7, 1.1 and 3.7). This indicates hyposensitivity or down-regulation of the α-adrenoceptor function of peripheral blood vessels in these ALS patients. For early detection of sympathetic hyperactivity and prediction of circulatory collapse, it would be useful to measure the plasma norepinephrine concentration and the gain of the norepinephrine infusion curve in respirator-dependent ALS patients.


amyotrophic lateral sclerosis down-regulation adrenoceptors sympathetic hyperactivity circulatory collapse norepinephrine 


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  1. 1.
    Hayashi H, Kato S, Kawada A. Amyotrophic lateral sclerosis patients living beyond respiratory failure.J Neurol Sci 1991;105: 73–78.PubMedGoogle Scholar
  2. 2.
    Bogucki A, Salvesen R. Sympathetic iris function in amyotrophic lateral sclerosis.J Neurol 1987;234: 185–186.PubMedGoogle Scholar
  3. 3.
    Jokelainen M, Palo J. Amyotrophic lateral sclerosis and autonomic nervous system.Lancet 1976;i: 1246.Google Scholar
  4. 4.
    Sachs C, Conradi S, Lennart K. Autonomic function in amyotrophic lateral sclerosis: a study of cardiovascular responses.Acta Neurol Scand 1985;71: 373–378.PubMedGoogle Scholar
  5. 5.
    Shimizu T, Hayashi H, Kato S, Hayashi M, Tanabe H, Oda M. Circulatory collapse and sudden death in respirator-dependent amyotrophic lateral sclerosis.J Neurol Sci 1994;124: 45–55.PubMedGoogle Scholar
  6. 6.
    Polinsky RJ, Kopin IJ, Ebert MH, Weise V. Pharmacologic distinction of different orthostatic hypotension syndromes.Neurology 1981;31: 1–7.Google Scholar
  7. 7.
    Sutoo D, Akiyama K, Takita H. Behavioral changes in cold-stressed mice related to a central calcium-dependent-catecholamine synthesizing system.Pharmacol Biochem Behav 1991;40: 423–428.PubMedGoogle Scholar
  8. 8.
    Chida K, Sakamaki S, Takasu T. Alteration in autonomic function and cardiovascular regulation in amyotrophic lateral sclerosis.J Neurol 1989;236: 127–130.PubMedGoogle Scholar
  9. 9.
    Shindo K, Tsunoda S, Shiozawa Z. Microneurographic analysis of muscle sympathetic nerve activity in amyotrophic lateral sclerosis.Clin Auton Res 1993;3: 131–135.PubMedGoogle Scholar
  10. 10.
    Tamura N, Shimazu K, Hienuki M, Maruki Y, Oiwa K, Kim Het al. Sympathetic nervous function in motor neuron disease and spinocerebellar degeneration.J Auton Nerv Syst 1986; Suppl: 435–440.Google Scholar
  11. 11.
    Tamura N, Shimazu K, Yamamoto T, Watanabe T, Onoda A, Itokawa K, Hamaguchi K. A supplementary study of sympathetic nervous hyperfunction in motor neuron disease.Auton Nerv Syst (Jpn) 1991;28: 357–363.Google Scholar
  12. 12.
    Ziegler MG, Brooks BR, Lake R, Wood JH, Enna SJ. Norepinephrine and gamma-aminobutyric acid in amyotrophic lateral sclerosis.Neurology 1980;30: 98–101PubMedGoogle Scholar
  13. 13.
    Spyer KM. Neural mechanisms involved in cardiovascular control during affective behaviour.Trends Neurosci 1989;12: 506–513.PubMedGoogle Scholar
  14. 14.
    Cryer PE. Physiology and pathophysiology of the human sympathoadrenal neuroendocrine system.N Engl J Med 1980;303: 436–444.PubMedGoogle Scholar

Copyright information

© Rapid Communications of Oxford Ltd 1995

Authors and Affiliations

  • T. Shimizu
    • 1
  • H. Hayashi
    • 2
  • M. Hayashi
    • 1
  • S. Kato
    • 1
  • H. Tanabe
    • 1
  1. 1.Department of NeurologyTokyo Metropolitan Neurological HospitalFuchu, TokyoJapan
  2. 2.Department of NeurologyTokyo Metropolitan Komagome HospitalTokyoJapan

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