Cardiovascular dysautonomia in fatal familial insomnia

Pietro Cortelli, Piero Parchi, Manuela Contin, Giulia Pierangeli, Patrizia Avoni, Paolo Tinuper, Pasquale Montagna, Agostino Baruzzi, Pier Luigi Gambetti, Elio Lugaresi

Research output: Contribution to journalArticlepeer-review


Autonomic control of the cardiovascular system was assessed in two patients with Fatal Familial Insomnia. The diagnosis was confirmed at autopsy in patient 1. In the resting state blood pressure and heart rate were higher than controls in patient 1; plasma noradrenaline levels were elevated in both patients. Evaluation of cardiovascular reflexes indicated intact baroreflex pathways but with exaggerated blood pressure and biochemical responses to certain stimuli (postural change, Valsalva manoeuvre, isometric handgrip). There was no pressor response to intravenously infused noradrenaline, an increased response to atropine and diminished depressor and sedative effects to clonidine. Overall these results are indicative of an unbalanced autonomic control with preserved parasympathetic and higher background and stimulated sympathetic activity. These physiological, biochemical and pharmacological data, together with known neuro-pathological findings in this disorder, emphasize the possible role played by the thalamus in regulating autonomic control of cardiovascular function in man.

Original languageEnglish
Pages (from-to)15-21
Number of pages7
JournalClinical Autonomic Research
Issue number1
Publication statusPublished - Mar 1991


  • Cardiovascular system
  • Dysautonomia
  • Sleep
  • Thalamic Degeneration

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology


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