Disorders of the autonomic nervous system: Part 2. investigation and treatment

Autonomic function may be adequately tested with noninvasive tests of sympathetic and parasympathetic pathways, including: the response of blood pressure to change in posture and isometric contraction, heart rate response to standing, variation in heart rate with respiration, Valsalva ratio, sweat tests, and plasma noradrenaline measurements. Abnormal results in two or more of these tests indicate autonomic dysfunction. Intraarterial catheterization and tests of vasomotor function are usually required only in doubtful cases or for research purposes. Treatment of autonomic dysfunction is focused primarily on bladder control and control of orthostatic hypotension. Orthostatic hypotension is best treated with physical measures, pharmacologically with 9‐alpha‐fluorohydrocortisone and dihydroergotamine mesylate. A number of other agents may be tried but results have been less effective.

[1]  K. Lübke A controlled study with Dihydergot on patients with orthostatic dysregulation. , 1976, Cardiology.

[2]  SWEAT SECRETION IN MAN: II. ANATOMIC DISTRIBUTION OF DISTURBANCES IN SWEATING ASSOCIATED WITH LESIONS OF THE SYMPATHETIC NERVOUS SYSTEM , 1938 .

[3]  P. Sever,et al.  Cardiovascular reflexes and biochemical responses in progressive autonomic failure. , 1977, Brain : a journal of neurology.

[4]  F. Abboud,et al.  Carotid and cardiopulmonary baroreceptor control of splanchnic and forearm vascular resistance during venous pooling in man , 1979, The Journal of physiology.

[5]  D. Ewing,et al.  Cardiovascular responses to sustained handgrip in normal subjects and in patients with diabetes mellitus: a test of autonomic function. , 1974, Clinical science and molecular medicine.

[6]  E. Sharpey-Schafer,et al.  Postural changes in the peripheral blood-flow of normal subjects with observations on vasovagal fainting reactions as a result of tilting, the lordotic posture, pregnancy and spinal anaesthesia. , 1950, Clinical science.

[7]  N. Christensen,et al.  Treatment of diabetic orthostatic hypotension with Pindolol , 1982, Acta Neurologica Scandinavica.

[8]  George E. Brown,et al.  The cold pressor test for measuring the reactibility of the blood pressure: Data concerning 571 normal and hypertensive subjects , 1936 .

[9]  R. Bedrossian Silastic intubation of the nasolacrimal passages. , 1978, Ophthalmology.

[10]  W. Cannon,et al.  A LAW OF DENERVATION , 1939 .

[11]  K. Shimada,et al.  Age-related changes of baroreflex function, plasma norepinephrine, and blood pressure. , 1985, Hypertension.

[12]  H. Kirchheim Systemic arterial baroreceptor reflexes. , 1976, Physiological reviews.

[13]  J. Ludbrook,et al.  Effects of mental arithmetic on arterial pressure and hand blood flow. , 1975, Clinical and Experimental Pharmacology and Physiology.

[14]  J. Brown,et al.  Plasma renin in idiopathic orthostatic hypotension: differential response in subjects with probable afferent and efferent autonomic failure. , 1971, Clinical science.

[15]  C. F. List,et al.  SWEAT SECRETION IN MAN: III. CLINICAL OBSERVATIONS ON SWEATING PRODUCED BY PILOCARPINE AND MECHOLYL , 1938 .

[16]  R. Edwards,et al.  Postural hypotension--pathophysiology and management. , 1986, The Quarterly journal of medicine.

[17]  D Robertson,et al.  Management of chronic orthostatic hypotension. , 1986, American Journal of Medicine.

[18]  D J Ewing,et al.  Autonomic function in patients with chronic renal failure on intermittent haemodialysis. , 1975, Nephron.

[19]  W. Kennedy,et al.  Collateral reinnervation of sweat glands , 1984, Annals of neurology.

[20]  B. Wallin,et al.  Effect of lower body negative pressure on human muscle nerve sympathetic activity , 1978, The Journal of physiology.

[21]  R Peto,et al.  Effect of Age and High Blood Pressure on Barorefiex Sensitivity in Man , 1971, Circulation research.

[22]  D. Eckberg Adaptation of the human carotid baroreceptor‐cardiac reflex. , 1977, The Journal of physiology.

[23]  P. Low,et al.  The sympathetic nervous system in diabetic neuropathy. A clinical and pathological study. , 1975, Brain : a journal of neurology.

[24]  R. Maclennan MORE ABOUT METHYSERGIDE. , 1964, Lancet.

[25]  S. Sheps,et al.  Midodrine. A new agent in the management of idiopathic orthostatic hypotension and Shy-Drager syndrome. , 1981, Mayo Clinic proceedings.

[26]  P. Sever,et al.  Defective cardiovascular reflexes and supersensitivity to sympathomimetic drugs in autonomic failure. , 1979, Brain : a journal of neurology.

[27]  J. Halperin,et al.  Sympathetic skin response--a method of assessing unmyelinated axon dysfunction in peripheral neuropathies. , 1984, Journal of neurology, neurosurgery, and psychiatry.

[28]  G. Sundkvist,et al.  Respiratory influence on heart rate in diabetes mellitus. , 1979, British medical journal.

[29]  S. L. Nielsen,et al.  Measurement of blood flow in adipose tissue from the washout of Xenon-133 after atraumatic labelling. , 1972, Acta physiologica Scandinavica.

[30]  Y Toyokura,et al.  Postural hypotension and low R‐R interval variability in parkinsonism, spino‐cerebellar degeneration, and Shy‐Drager syndrome , 1983, Neurology.

[31]  H. Scheie SITE OF DISTURBANCE IN ADIE'S SYNDROME , 1940 .

[32]  M. Lader,et al.  THE PSYCHO-GALVANIC REFLEX: A PHARMACOLOGICAL STUDY OF THE PERIPHERAL MECHANISM , 1962, Journal of neurology, neurosurgery, and psychiatry.

[33]  F. Mcdowell,et al.  Release of antidiuretic hormone in quadriplegic subjects in response to head‐up tilt , 1985, Neurology.

[34]  R. Khurana,et al.  Shy‐Drager syndrome , 1980, Neurology.

[35]  O. Appenzeller,et al.  Autonomic deficits in Parkinson's syndrome. , 1971, Archives of neurology.

[36]  R. Murray,et al.  Idiopathic postural hypotension: physiologic observations and report of a new mode of therapy. , 1970, The Journal of clinical investigation.

[37]  L. Rowell,et al.  Human Splanchnic and Forearm Vasoconstrictor Responses to Reductions of Right Atrial and Aortic Pressures , 1974, Circulation research.

[38]  P. Dyck,et al.  Evaluation of skin vasomotor reflexes by using laser Doppler velocimetry. , 1983, Mayo Clinic proceedings.

[39]  P. Sever,et al.  PRESSOR AMINES AND MONOAMINE-OXIDASE INHIBITORS FOR TREATMENT OF POSTURAL HYPOTENSION IN AUTONOMIC FAILURE Limitations and Hazards , 1978, The Lancet.

[40]  D. A. Blair,et al.  The increase in tone in forearm resistance blood vessels exposed to increased transmural pressure , 1959, The Journal of physiology.

[41]  G. Jennings,et al.  Low oral bioavailability of dihydroergotamine and first‐pass extraction in patients with Orthostatic hypotension , 1981, Clinical pharmacology and therapeutics.

[42]  P. Low,et al.  The sympathetic nervous system in alcoholic neuropathy. A clinical and pathological study. , 1975, Brain : a journal of neurology.

[43]  R HARRIS,et al.  Correlation of skin temperature and circulatory changes in muscle and subcutaneous tissue of the hand during trunk heating. , 1952, Clinical science.

[44]  E. Sharpey-Schafer Circulatory reflexes in chronic disease of the afferent nervous system , 1956, The Journal of physiology.

[45]  B. Robinson,et al.  Do elderly patients with an excessive fall in blood pressure on standing have evidence of autonomic failure? , 1983, Clinical science.

[46]  J. Ludbrook,et al.  Cold Hypersensitivity in Raynaud's Phenomenon , 1971 .

[47]  P. W. Humphreys,et al.  THE CIRCULATIORY EFFECTS OF SUSTAINED VOLUNTARY MUSCLE CONTRACTION. , 1964, Clinical science.

[48]  I. Kopin,et al.  SYMPATHETIC NEURAL PROSTHESIS FOR MANAGING ORTHOSTATIC HYPOTENSION , 1983, The Lancet.

[49]  J. Uther,et al.  ‘Steady‐state’ properties of the baroreceptor‐heart rate reflex in essential hypertension in man , 1974, Clinical and experimental pharmacology & physiology.

[50]  P. Watkins,et al.  Cardiac Denervation in Diabetes , 1973, British medical journal.

[51]  J. Webster,et al.  Influence of arm position on measurement of blood pressure. , 1984, British medical journal.

[52]  R. Hellon,et al.  Use of the strain gauge plethysmograph in assessing the effect of certain drugs on the blood flow through the skin and muscle of the human forearm , 1958, The Journal of physiology.

[53]  Circulatory reflexes in cerebral ischaemia involving different vascular territories. , 1970, Clinical science.

[54]  T. Schwartz,et al.  Impaired response of pancreatic polypeptide to hypoglycaemia: an early sign of autonomic neuropathy in diabetics. , 1979, British medical journal.

[55]  R. Bannister,et al.  Methacholine pupillary responses in third nerve palsy and Adie's syndrome. , 1982, Brain : a journal of neurology.

[56]  L. Guttmann TOPOGRAPHIC STUDIES OF DISTURBANCES OF SWEAT SECRETION AFTER COMPLETE LESIONS OF PERIPHERAL NERVES , 1940, Journal of neurology and psychiatry.

[57]  D. Ewing,et al.  Plasma Renin Activity in Diabetic Autonomic Neuropathy , 1976, European journal of clinical investigation.

[58]  D. Ferguson,et al.  Effects of Propranolol on Reflex Vascular Responses to Orthostatic Stress in Humans: Role of Ventricular Baroreceptors , 1983, Circulation.

[59]  G. Jennings,et al.  Treatment of orthostatic hypotension with dihydroergotamine. , 1979, British medical journal.

[60]  I. Kopin,et al.  The sympathetic-nervous-system defect in primary orthostatic hypotension. , 1977, The New England journal of medicine.

[61]  A. Paintal,et al.  Vagal sensory receptors and their reflex effects. , 1973, Physiological reviews.

[62]  R Bannister,et al.  Degenerative diseases of the nervous system associated with autonomic failure. , 1972, Brain : a journal of neurology.

[63]  S. Smith Reduced sinus arrhythmia in diabetic autonomic neuropathy: diagnostic value of an age-related normal range. , 1982, British medical journal.

[64]  R. F. Whelan,et al.  The vasomotor nerve supply to the skin and muscle of the human forearm. , 1957, Clinical science.

[65]  D. McCloskey,et al.  Cardiovascular and respiratory responses to changes in central command during isometric exercise at constant muscle tension , 1972, The Journal of physiology.

[66]  D. Richardson,et al.  Norepinephrine depletion in idiopathic orthostatic hypotension. , 1975, Annals of internal medicine.

[67]  H. T. Howat,et al.  Rapid evaluation of creatinine clearance. , 1971, Lancet.

[68]  S. Mellander,et al.  Comparative effects of dihydroergotamine and noradrenaline on resistance, exchange and capacitance functions in the peripheral circulation. , 1970, Clinical science.

[69]  I. Kopin,et al.  Pharmacologic distinction of different orthostatic hypotension syndromes , 1981, Neurology.

[70]  R. Penhall,et al.  PINDOLOL IN ORTHOSTATIC HYPOTENSION, POSSIBLE THERAPY? , 1980, The Medical journal of Australia.

[71]  R. Hoeldtke,et al.  Treatment of orthostatic hypotension with dihydroergotamine and caffeine. , 1986, Annals of internal medicine.

[72]  I. Schatz Orthostatic hypotension. II. Clinical diagnosis, testing, and treatment. , 1984, Archives of internal medicine.

[73]  P. Bennett,et al.  Autonomic Neuropathy in Rheumatoid Arthritis , 1965, Annals of the rheumatic diseases.

[74]  J. Holst,et al.  Vagal, cholinergic regulation of pancreatic polypeptide secretion. , 1978, The Journal of clinical investigation.

[75]  P. Boyle,et al.  Autonomic neuropathy in acute intermittent porphyria. , 1985, Journal of neurology, neurosurgery, and psychiatry.

[76]  D. Ewing,et al.  Immediate heart-rate response to standing: simple test for autonomic neuropathy in diabetes. , 1978, British medical journal.

[77]  D L Eckberg,et al.  Baroreflex inhibition of the human sinus node: importance of stimulus intensity, duration, and rate of pressure change. , 1977, The Journal of physiology.

[78]  R. Bannister,et al.  An assessment of various methods of treatment of idiopathic orthostatic hypotension. , 1969, The Quarterly journal of medicine.

[79]  A. Levin,et al.  A simple test of cardiac function based upon the heart rate changes induced by the Valsalva maneuver. , 1966, The American journal of cardiology.

[80]  J. Gutkowska,et al.  Treatment of severe orthostatic hypotension by metoclopramide. , 1980, Annals of internal medicine.

[81]  D. Agarwal,et al.  Aldehyde Dehydrogenase Deficiency as Cause of Facial Flushing Reaction to Alcohol in Japanese , 1995, Alcohol health and research world.

[82]  S. Bajada Dihydroergotamine therapy in symptomatic postural hypotension. , 1979, Australian and New Zealand journal of medicine.

[83]  S. Skinner,et al.  Sympathomimetic drugs in orthostatic hypotension. , 1961, Lancet.

[84]  W. Delius,et al.  Reactions of arterial and venous vessels in the human forearm and hand to deep breath or mental strain. , 1971, Clinical science.

[85]  R. Johnson,et al.  TREATMENT OF NEUROGENIC ORTHOSTATIC HYPOTENSION WITH A MONOAMINE OXIDASE INHIBITOR AND TYRAMINE , 1976, The Lancet.

[86]  J. Hampton,et al.  Cardiovascular responses to graded reductions of central blood volume in normal subjects and in patients with diabetes mellitus. , 1980, Clinical science.

[87]  C. Mathias,et al.  PINDOLOL IN POSTURAL HYPOTENSION: THE CASE FOR CAUTION , 1981, The Lancet.

[88]  Cooper Eh,et al.  Pilomotor and sudomotor innervation in diabetes. , 1956 .

[89]  C. F. List,et al.  SWEAT SECRETION IN MAN: I. SWEATING RESPONSES IN NORMAL PERSONS , 1938 .

[90]  G. Solders,et al.  R‐R variations in Guillain‐Barré syndrome: a test of autonomic dysfunction , 1983, Acta neurologica Scandinavica.

[91]  P. Cryer,et al.  Cholinergic stimulation of norepinephrine release in man. Evidence of a sympathetic postganglionic axonal lesion in diabetic adrenergic neuropathy. , 1979, The Journal of clinical investigation.

[92]  E. Topol,et al.  Atrial tachypacing in the treatment of a patient with primary orthostatic hypotension. , 1980, The New England journal of medicine.

[93]  J. Shaver,et al.  Reflex heart rate control in man. , 1970, American heart journal.

[94]  Wally Knezevic,et al.  Peripheral autonomic surface potential A quantitative technique for recording sympathetic conduction in man , 1985, Journal of the Neurological Sciences.

[95]  A. Tonkin,et al.  Reflex and mechanical circulatory effects of graded Valsalva maneuvers in normal man. , 1976, Journal of applied physiology.

[96]  A. D. Smith MECHANISMS INVOLVED IN THE RELEASE OF NORADRENALINE FROM SYMPATHETIC NERVES , 1973 .

[97]  O. Appenzeller,et al.  NEUROGENIC PATHWAYS CONCERNED IN REFLEX VASODILATATION IN THE HAND WITH ESPECIAL REFERENCE TO STIMULI AFFECTING THE AFFERENT PATHWAY. , 1963, Clinical science.

[98]  A. Macmillan,et al.  Human sweating response to electrophoresed acetylcholine: a test of postganglionic sympathetic function. , 1969, Journal of neurology, neurosurgery, and psychiatry.

[99]  L. Kaijser,et al.  Autonomic cardiovascular responses in old age. , 1985, Clinical physiology.

[100]  R. Gordon,et al.  Role of the sympathetic nervous system in regulating renin and aldosterone production in man. , 1967, The Journal of clinical investigation.

[101]  P. Sever,et al.  The pressor actions of noradrenaline and angiotension II in chronic autonomic failure treated with indomethacin. , 1980, British journal of clinical pharmacology.

[102]  B. Wallin,et al.  Sympathetic reflex latencies and conduction velocities in normal man , 1980, Journal of the Neurological Sciences.

[103]  E. Braunwald,et al.  Control of Heart Rate by the Autonomic Nervous System: Studies in Man on the Interrelation Between Baroreceptor Mechanisms and Exercise , 1966, Circulation research.

[104]  R. Gilliatt,et al.  Vaso‐constriction in the finger after deep inspiration , 1948, The Journal of physiology.

[105]  J. Onrot,et al.  Hemodynamic and humoral effects of caffeine in autonomic failure. Therapeutic implications for postprandial hypotension. , 1985, The New England journal of medicine.

[106]  S. Rothman,et al.  Sweat Response to Acetylcholine1 , 1942 .

[107]  G. Solders,et al.  Autonomic dysfunction in non-diabetic terminal uraemia. , 2009, Acta neurologica Scandinavica.

[108]  W. Kennedy,et al.  Quantitation of the sweating deficiency in diabetes mellitus , 1984, Annals of neurology.

[109]  P. Dyck,et al.  Quantitative sudomotor axon reflex test in normal and neuropathic subjects , 1983, Annals of neurology.

[110]  O. Hyndman,et al.  THE PILOCARPINE SWEATING TEST: I. A VALID INDICATOR IN DIFFERENTIATION OF PREGANGLIONIC AND POSTGANGLIONIC SYMPATHECTOMY , 1941 .

[111]  P. Cryer,et al.  Reduced plasma norepinephrine response to standing in autonomic dysfunction. , 1976, Archives of neurology.

[112]  R. Tuck,et al.  Autonomic dysfunction in Guillain-Barré syndrome. , 1981, Journal of neurology, neurosurgery, and psychiatry.

[113]  J M Detry,et al.  Importance of the splanchnic vascular bed in human blood pressure regulation. , 1972, Journal of applied physiology.

[114]  R. Hoeldtke,et al.  TREATMENT OF AUTONOMIC NEUROPATHY WITH A SOMATOSTATIN ANALOGUE SMS-201-995 , 1986, The Lancet.

[115]  S. Lightman,et al.  Blood pressure and vasopressin in progressive autonomic failure. Response to postural stimulation, L-dopa and naloxone. , 1983, Brain : a journal of neurology.

[116]  S. Lightman,et al.  Vasopressin secretion in progressive autonomic failure: evidence for defective afferent cardiovascular pathways. , 1985, Journal of neurology, neurosurgery, and psychiatry.

[117]  E Hultman,et al.  Blood pressure and heart rate response to voluntary and nonvoluntary static exercise in man. , 1982, Acta physiologica Scandinavica.

[118]  A. J. Man in 't Veld,et al.  Pindolol acts as beta-adrenoceptor agonist in orthostatic hypotension: therapeutic implications. , 1981, British medical journal.

[119]  K. Hagbarth,et al.  General characteristics of sympathetic activity in human skin nerves. , 1972, Acta physiologica Scandinavica.

[120]  A. Hollister,et al.  Clonidine raises blood pressure in severe idiopathic orthostatic hypotension. , 1983, The American journal of medicine.

[121]  P. Sönksen,et al.  Pupillary signs in diabetic autonomic neuropathy. , 1978, British medical journal.

[122]  D. Robertson,et al.  Postprandial alterations in cardiovascular hemodynamics in autonomic dysfunction states. , 1981, The American journal of cardiology.

[123]  C. L. Lim,et al.  Facial sweating in Horner's syndrome. , 1984, Brain : a journal of neurology.

[124]  G. Pickering,et al.  Reflex Regulation of Arterial Pressure during Sleep in Man: A Quantitative Method of Assessing Baroreflex Sensitivity , 1969, Circulation research.

[125]  H. Itskovitz,et al.  TREATMENT OF IDIOPATHIC ORTHOSTATIC HYPOTENSION (SHY-DRAGER SYNDROME) WITH INDOMETHACIN , 1978, The Lancet.