Abstract
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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Lugaresi E, Medori R, Montagna P,et al. Fatal Familial Insomnia and dysautonomia with selective degeneration of thalamic nuclei.NEJM 1986;315: 997–1003.
Lugaresi E, Montagna P, Cortelli P, Gambetti PL, Gambetti PL. Fata Familial Insomnia: role of the thalamus in endocrine and autonomic regulation. In: Mancia M, Marini G. eds.The Diencephalon and Sleep. New York, Raven Press, 1990, 293–9.
UAnton AH, Sayre DF. A study of the factors affecting the aluminum oxidetrihydroxyindole procedure for the analysis of catecholamines.J Pharmacol Exp Ther 1962;138: 360–75.
Bannister R, Mathias CJ. Testing autonomic reflexes. In: Bannister R ed.Autonomic Failure—A Textbook of Clinical Disorders of the Autonomic Nervous System, 2nd edn. Oxford: Oxford University Press, 1988: 289–307.
Goldstein DS, Horwitz D, Keiser HR. Comparison of techniques for measuring baroreflex sensitivity in man.Circulation 1982;66: 432–9.
Goldstein DS, McCarty R, Polinsky RJ, Kopin IJ. Relationship between plasma norepinephrine and sympathetic neural activity.Hypertension 1983;5: 552–9.
Kobinger W. Central alpha-adrenergic systems as targets for hypotensive drugs.Rev Physiol Biochem Pharmacol 1978;81: 39–100.
Nutt DJ. Interaction between diazepam and noradrenergic function in man. HumansPsychopharmacology 1986;1: 35–40.
Spyer KM. Central nervous system control of the cardiovascular system. In: Bannister R, ed.Autonomic Failure—A Textbook of Clinical Disorders of the Autonomic Nervous System, 2nd edn. Oxford: Oxford University Press, 1988: 56–79.
Jones EG. The medial nuclei. The anterior nuclei. In:The Thalamus. New York: Plenum, 1985: 648–96.
Room P, Russchen FT, Groenewegen HJ, Lohman AHM. Efferent connections of the prelimbic (area 32) and the infralimbic (area 25) cortices: an anterograde tracing study in the cat.J Comparative Neurol 1985;242: 40–55.
Cechetto DF, Saper CB. Role of the cerebral cortex in autonomic function. In: Loewy AD, Spyer KM, eds.Central Regulation of Autonomic Functions. New York, Oxford: Oxford University Press, 1990: 208–23.
Terreberry RR, Neafsey EJ. Rat medial frontal cortex: a visceral motor region with a direct projection to the solitary nucleus.Brain Res. 1983;278: 245–9.
Yasui Y, Itoh K, Sugimoto T, Kaneko T, Mizuno N. Thalamocortical and thalamo-amygdaloid projections from the parvicellular division of the posteromedial ventral nucleus in the cat. 1987;257: 253–68.
Hess WR.Hypothalamus and Thalamus: experimental documentation. Stuttgart: Georg Thieme, 1969.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Cortelli, P., Parchi, P., Contin, M. et al. Cardiovascular dysautonomia in fatal familial insomnia. Clinical Autonomic Research 1, 15–21 (1991). https://doi.org/10.1007/BF01826053
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01826053