Evaluation and Management of Autonomic Nervous System Disorders

ABSTRACT

Autonomic nervous system dysfunction may manifest with a variety of symptoms, with orthostatic intolerance (including orthostatic hypotension or tachycardia) and sweating abnormalities (increased or decreased sweating) being common problems requiring medical evaluation and treatment. Determination of the underlying diagnosis for these symptoms is critical in terms of classification of the disorder and its prognosis. Recent advances in evaluation of patients with these conditions and treatment modalities have enabled physicians to improve overall management of patients with these disorders. These advances include testing for ganglionic acetylcholine receptor antibody in patients with suspected autoimmune autonomic neuropathy and use of pyridostigmine for treatment of patients with orthostatic hypotension or tachycardia.

KEYWORDS

Dysautonomia - orthostatic hypotension - postural tachycardia syndrome

REFERENCES

• 1 Vernino S, Low P A, Fealey R D, Stewart J D, Farrugia G, Lennon V A. Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies.  N Engl J Med. 2000;  343 847-855
• 2 Klein C M, Vernino S, Lennon V A et al.. The spectrum of autoimmune autonomic neuropathies.  Ann Neurol. 2003;  53 752-758
• 3 Sandroni P, Vernino S, Klein C M et al.. Idiopathic autonomic neuropathy: comparison of cases seropositive and seronegative for ganglionic acetylcholine receptor antibody.  Arch Neurol. 2004;  61 44-48
• 4 Vernino S, Lennon V A. Neuronal ganglionic acetylcholine receptor autoimmunity.  Ann N Y Acad Sci. 2003;  998 211-214
• 5 Vernino S, Low P A, Lennon V A. Experimental autoimmune autonomic neuropathy.  J Neurophysiol. 2003;  90 2053-2059
• 6 Wang Z, Low P A, Jordan J et al.. Autoimmune autonomic ganglionopathy: IgG effects on ganglionic acetylcholine receptor current.  Neurology. 2007;  68 1917-1921
• 7 Goldstein D S. Dysautonomias: clinical disorders of the autonomic nervous system.  Ann Intern Med. 2002;  137 753-763
• 8 Raj S R. The postural tachycardia syndrome (POTS): pathophysiology, diagnosis and management.  Indian Pacing Electrophysiol J. 2006;  6 84-99
• 9 Grubb B P. Neurocardiogenic syncope and related disorders of orthostatic intolerance.  Circulation. 2005;  111 2997-3006
• 10 Medow M S, Stewart J M. The postural tachycardia syndrome.  Cardiol Rev. 2007;  15 67-75
• 11 Thieben M J, Sandroni P, Sletten D M et al.. Postural orthostatic tachycardia syndrome: the Mayo Clinic experience.  Mayo Clin Proc. 2007;  82 308-313
• 12 Low P A, Vernino S, Suarez G. Autonomic dysfunction in peripheral nerve disease.  Muscle Nerve. 2003;  27 646-661
• 13 Winker R, Barth A, Valic E et al.. Functional adrenergic receptor polymorphisms and idiopathic orthostatic intolerance.  Int Arch Occup Environ Health. 2005;  78 171-177
• 14 Naschitz J E, Slobodin G, Elias N, Rosner I. The patient with supine hypertension and orthostatic hypotension: a clinical dilemma.  Postgrad Med J. 2006;  82 246-253
• 15 Goldstein D S, Pechnik S, Holmes C, Eldadah B, Sharabi Y. Association between supine hypertension and orthostatic hypotension in autonomic failure.  Hypertension. 2003;  42 136-142
• 16 Low P A. Autonomic neuropathies.  Curr Opin Neurol. 2002;  15 605-609
• 17 Mori K, Iijima M, Koike H et al.. The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy.  Brain. 2005;  128 2518-2534
• 18 Dyck P J. The clinical heterogeneity of immune sensory and autonomic neuropathies with (or without) sicca.  Brain. 2005;  128 2480-2482
• 19 O'Suilleabhain P, Low P A, Lennon V A. Autonomic dysfunction in the Lambert-Eaton myasthenic syndrome: serologic and clinical correlates.  Neurology. 1998;  50 88-93
• 20 Goldstein D S, Holmes C, Sharabi Y, Brentzel S, Eisenhofer G. Plasma levels of catechols and metanephrines in neurogenic orthostatic hypotension.  Neurology. 2003;  60 1327-1332
• 21 Tipre D N, Goldstein D S. Cardiac and extracardiac sympathetic denervation in Parkinson's disease with orthostatic hypotension and in pure autonomic failure.  J Nucl Med. 2005;  46 1775-1781
• 22 Sharabi Y, Eldadah B, Li S T et al.. Neuropharmacologic distinction of neurogenic hypotension syndromes.  Clin Neuropharmacol. 2006;  29 97-105
• 23 Mabuchi N, Hirayama M, Koike Y et al.. Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy.  J Neurol Neurosurg Psychiatry. 2005;  76 947-952
• 24 Colman N, Nahm K, Ganzeboom K S et al.. Epidemiology of reflex syncope.  Clin Auton Res. 2004;  14(Suppl 1) 9-17
• 25 Assessment: Clinical autonomic testing report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology.  Neurology. 1996;  46 873-880
• 26 Gehrking J A, Hines S M, Benrud-Larson L M, Opher-Gehrking T L, Low P A. What is the minimum duration of head-up tilt necessary to detect orthostatic hypotension?.  Clin Auton Res. 2005;  15 71-75
• 27 Gibbons C H, Freeman R. Delayed orthostatic hypotension: a frequent cause of orthostatic intolerance.  Neurology. 2006;  67 28-32
• 28 Colosimo C, Tiple D, Wenning G K. Management of multiple system atrophy: state of the art.  J Neural Transm. 2005;  112 1695-1704
• 29 Claydon V E, Hainsworth R. Salt supplementation improves orthostatic cerebral and peripheral vascular control in patients with syncope.  Hypertension. 2004;  43 809-813
• 30 Mathias C J, Young T M. Water drinking in the management of orthostatic intolerance due to orthostatic hypotension, vasovagal syncope and the postural tachycardia syndrome.  Eur J Neurol. 2004;  11 613-619
• 31 Young T M, Mathias C J. The effects of water ingestion on orthostatic hypotension in two groups of chronic autonomic failure: multiple system atrophy and pure autonomic failure.  J Neurol Neurosurg Psychiatry. 2004;  75 1737-1741
• 32 Deguchi K, Ikeda K, Sasaki I et al.. Effects of daily water drinking on orthostatic and postprandial hypotension in patients with multiple system atrophy.  J Neurol. 2007;  254 735-740
• 33 Oldenburg O, Kribben A, Baumgart D, Philipp T, Erbel R, Cohen M V. Treatment of orthostatic hypotension.  Curr Opin Pharmacol. 2002;  2 740-747
• 34 Podoleanu C, Maggi R, Brignole M et al.. Lower limb and abdominal compression bandages prevent progressive orthostatic hypotension in elderly patients.  J Am Coll Cardiol. 2006;  48 1425-1432
• 35 Low P A, Gilden J L, Freeman R, Sheng K N, McElligott M A. Efficacy of midodrine vs. placebo in neurogenic orthostatic hypotension. A randomized, double-blind multicenter study. Midodrine Study Group.  JAMA. 1997;  277 1046-1051
• 36 Singer W, Opfer-Gehrking T L, Nickander K K, Hines S M, Low P A. Acetylcholinesterase inhibition in patients with orthostatic intolerance.  J Clin Neurophysiol. 2006;  23 476-481
• 37 Singer W, Opfer-Gehrking T L, McPhee B R, Hilz M J, Bharucha A E, Low P A. Acetylcholinesterase inhibition: a novel approach in the treatment of neurogenic orthostatic hypotension.  J Neurol Neurosurg Psychiatry. 2003;  74 1294-1298
• 38 Singer W, Sandroni P, Opfer-Gehrking T et al.. Pyridostigmine treatment trial in neurogenic orthostatic hypotension.  Arch Neurol. 2006;  63 513-518
• 39 Sandroni P, Opfer-Gehrking T L, Singer W, Low P A. Pyridostigmine for treatment of neurogenic orthostatic hypotension: a follow-up survey study.  Clin Auton Res. 2005;  15 51-53
• 40 Yamamoto T, Sakakibara R, Yamanaka Y et al.. Pyridostigmine in autonomic failure: can we treat postural hypotension and bladder dysfunction with one drug?.  Clin Auton Res. 2006;  16 296-298
• 41 Mathias C J, Fosbraey P, da Costa D F, Thornley A, Bannister R. The effect of desmopressin on nocturnal polyuria, overnight weight loss and morning postural hypotension in patients with autonomic failure.  Br Med J (Clin Res Ed). 1986;  293 353-354
• 42 Kawakami K, Abe H, Harayama N, Nakashima Y. Successful treatment of severe orthostatic hypotension with erythropoietin.  Pacing Clin Electrophysiol. 2003;  26 105-107
• 43 Shibao C, Gamboa A, Abraham R et al.. Clonidine for the treatment of supine hypertension and pressure natriuresis in autonomic failure.  Hypertension. 2006;  47 522-526
• 44 Shibao C, Gamboa A, Diedrich A, Biaggioni I. Management of hypertension in the setting of autonomic dysfunction.  Curr Treat Options Cardiovasc Med. 2006;  8 105-109
• 45 Raj S R, Black B K, Biaggioni I, Harris P A, Robertson D. Acetylcholinesterase inhibition improves tachycardia in postural tachycardia syndrome.  Circulation. 2005;  111 2734-2740
• 46 Schroeder C, Vernino S, Birkenfeld A L et al.. Plasma exchange for primary autonomic failure.  N Engl J Med. 2005;  353 1585-1590
• 47 Ishitobi M, Haginoya K, Kitamura T, Munakata M, Yokoyama H, Iinuma K. Acute dysautonomia: complete recovery after two courses of IVIg.  Brain Dev. 2004;  26 542-544
• 48 Heafield M T, Gammage M D, Nightingale S, Williams A C. Idiopathic dysautonomia treated with intravenous gammaglobulin.  Lancet. 1996;  347 28-29
• 49 Modoni A, Mirabella M, Madia F et al.. Chronic autoimmune autonomic neuropathy responsive to immunosuppressive therapy.  Neurology. 2007;  68 161-162
• 50 Mericle R A, Triggs W J. Treatment of acute pandysautonomia with intravenous immunoglobulin.  J Neurol Neurosurg Psychiatry. 1997;  62 529-531
• 51 Venkataraman S, Alexander M, Gnanamuthu C. Postinfectious pandysautonomia with complete recovery after intravenous immunoglobulin therapy.  Neurology. 1998;  51 1764-1765
• 52 Palm F, Löser C, Gronau W, Voigtländer V, Grau A J. Successful treatment of acquired idiopathic generalized anhidrosis.  Neurology. 2007;  68 532-533