Evidence of cardiovascular autonomic impairment in mitochondrial disorders

R. Di Leo, O. Musumeci, C. De Gregorio, A. Recupero, P. Grimaldi, C. Messina, S. Coglitore, G. Vita, A. Toscano

Research output: Contribution to journalArticlepeer-review


Objective: To investigate autonomic nervous system (ANS) function in mitochondrial disorders (MD). Background: MD are characterized by a wide range of clinical features, including heart abnormalities and peripheral and central nervous systems involvement. Rarely autonomic symptoms have been reported. Methods: 22 patients with MD underwent a battery of cardiovascular reflex tests including five tests of parasympathetic function and four tests of sympathetic function. Power spectral analyses (PSA) of heart rate variability in the supine and upright positions were also evaluated. Plasma levels of adrenaline, noradrenaline and dopamine were determined in the standing and lying positions. Results: Only 4/22 patients referred symptoms related to ANS dysfunction. 46% of patients had a definite autonomic damage (i. e. an autonomic score ≥ 4). 36% showed moderate alterations with an autonomic score in the range 2-3 and 18 % had a normal autonomic function. MD patients had a significantly (p

Original languageEnglish
Pages (from-to)1498-1503
Number of pages6
JournalJournal of Neurology
Issue number11
Publication statusPublished - Nov 2007


  • Autonomic nervous system
  • Cardiovascular reflex tests
  • Mitochondrial encephalomyopathies
  • Plasma catecholamines
  • Power spectral analysis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology


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