Shy-Drager Syndrome

A progressive neurodegenerative condition of the central and autonomic nervous systems characterized by atrophy of the preganglionic lateral horn neurons of the thoracic spinal cord. This disease is generally considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Affected individuals present in the fifth or sixth decade with ORTHOSTASIS and bladder dysfunction; and later develop FECAL INCONTINENCE; anhidrosis; ATAXIA; IMPOTENCE; and alterations of tone suggestive of basal ganglia dysfunction. (From Adams et al., Principles of Neurology, 6th ed, p536)

Also Known As:

Dysautonomia-Orthostatic Hypotension Syndrome; Syndrome, Dysautonomia-Orthostatic Hypotension; Autonomic Failures, Progressive; Dysautonomia Orthostatic Hypotension Syndrome; Dysautonomia-Orthostatic Hypotension Syndromes; Dysautonomic Orthostatic Hypotension; Dysautonomic Orthostatic Hypotensions; Failure, Progressive Autonomic; Failures, Progressive Autonomic; Hypotension Syndrome, Dysautonomia-Orthostatic; Hypotension Syndromes, Dysautonomia-Orthostatic; Hypotension, Dysautonomic Orthostatic; Hypotensions, Dysautonomic Orthostatic; Idiopathic Orthostatic Hypotension, Shy Drager Type; Orthostatic Hypotensions, Dysautonomic; Progressive Autonomic Failure; Progressive Autonomic Failures; Shy Drager Syndrome; Syndrome, Dysautonomia Orthostatic Hypotension; Syndrome, Shy-Drager; Syndromes, Dysautonomia-Orthostatic Hypotension; Autonomic Failure, Progressive; Idiopathic Orthostatic Hypotension, Shy-Drager Type; Orthostatic Hypotension, Dysautonomic

Networked: 133 relevant articles (5 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Orthostatic Hypotension (Postural Hypotension)
2.	Hypertension (High Blood Pressure)
3.	Diabetic Neuropathies (Diabetic Neuropathy)
4.	Multiple System Atrophy
5.	Parkinson Disease (Parkinson's Disease)

Experts

1.	Wenning, Gregor K: 3 articles (01/2013 - 08/2010)
2.	Goldstein, David S: 2 articles (06/2004 - 07/2002)
3.	Jordan, Jens: 2 articles (07/2002 - 03/2002)
4.	Biaggioni, Italo: 2 articles (05/2002 - 03/2002)
5.	Arias, Juan-Manuel: 1 article (09/2013)
6.	Arias-Montaño, José-Antonio: 1 article (09/2013)
7.	Escamilla-Sánchez, Juan: 1 article (09/2013)
8.	Flores-Clemente, Cecilia: 1 article (09/2013)
9.	Leurs, Rob: 1 article (09/2013)
10.	Osorio-Espinoza, Angélica: 1 article (09/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Shy-Drager Syndrome:

1.	Propranolol (Inderal)FDA LinkGeneric 11/01/1981 - "Effective treatment of orthostatic hypotension by propranolol in the Shy-Drager syndrome." 05/01/1982 - "Indomethacin and propranolol combined treatment for Shy-Drager syndrome." 01/01/1986 - "[Shy-Drager syndrome: description of a clinical case which failed to respond to propranolol, ergotamine and indomethacin]."
2.	Midodrine (Midon)FDA LinkGeneric 06/01/1993 - "Therefore, a recent multicenter study evaluated the safety and efficacy of midodrine therapy in 97 patients with neurogenic orthostatic hypotension due to various etiologies: Shy Drager syndrome (No. 18); Bradbury Eggleston syndrome (idiopathic orthostatic hypotension) (No. 20); diabetic autonomic neuropathy (No. 27); Parkinson's disease (No. 22); and miscellaneous (No. 10). " 07/01/1981 - "Five patients with idiopathic orthostatic hypotension, two of whom had central nervous system manifestations (Shy-Drager syndrome), were treated with midodrine, an investigational alpha-adrenergic agonist, in a dosage of 2.5 to 5 mg three times daily. " 11/01/1992 - "In order to increase CO and VR, we orally administered both denopamine 10 mg (a selective beta 1-adrenergic agonist) and midodrine HCl 4 mg (a selective alpha 1-adrenergic agonist) to six patients with progressive autonomic failure (AF). "
3.	EnzymesIBA 01/01/1985 - "Marked decrease in the enzyme activity in various brain regions was observed in striato-nigral degeneration, Shy-Drager syndrome, and progressive supranuclear palsy. " 08/01/1991 - "The effect of a balanced liquid meal on supine and postural blood pressure (BP) responses was investigated in three groups of patients with chronic autonomic failure; 10 with associated neurological impairment (multiple system atrophy (MSA), Shy-Drager syndrome) and seven without (of which five had pure autonomic failure (PAF); and two had a deficiency of the enzyme dopamine beta hydroxylase, DBH-deficiency). " 04/01/1995 - "The effect of supine exercise on blood pressure (BP), measured while recumbent and after head-up postural change, was investigated in three groups with marked postural hypotension due to chronic autonomic failure: 15 with associated neurological impairment (Shy-Drager syndrome, SDS, multiple system atrophy); 15 with pure autonomic failure (PAF) and two with a deficiency of the enzyme dopamine beta hydroxylase (DBH deficiency). "
4.	GlucocorticoidsIBA 12/01/1990 - "However, the normal pattern of circadian blood pressure rhythm is reversed in elderly people and in those with Cushing's syndrome, those undergoing glucocorticoid treatment, and those with hyperthyroidism, central and/or peripheral autonomic dysfunction (Shy-Drager syndrome, tetraplegia, diabetic or uremic neuropathy, etc), chronic renal failure, renal or cardiac transplantation, congestive heart failure, eclampsia, sleep apnea syndrome, malignant hypertension, systemic atherosclerosis and accelerated hypertensive organ damage. " 12/01/1990 - "However, in patients with Cushing's syndrome, under glucocorticoid treatment, or with hyperthyroidism, central and/or peripheral autonomic dysfunction (Shy-Drager syndrome, spinal cord injury, brainstem lesions, diabetic neuropathy, uremic neuropathy, etc), chronic renal failure, eclampsia, malignant hypertension, sleep apnea syndrome or systemic atherosclerosis, the normal circadian blood pressure rhythm appears to be eliminated or reversed, while in those with primary aldosteronism, renovascular hypertension, pheochromocytoma without paroxysmal hypertension, diabetes insipidus, acromegaly, hyperparathyroidism or hyperprolactinemia, the nocturnal blood pressure fall has been observed as in normal subjects. "
5.	Trimethaphan (Trimetaphan)IBA 05/01/2002 - "In patients with multiple system atrophy (Shy-Drager syndrome), supine hypertension is explained by residual sympathetic tone because it can be eliminated with the ganglionic blocker trimethaphan. "
6.	Norepinephrine (Noradrenaline)FDA LinkGeneric 01/01/1998 - "We conclude that ambulatory noradrenaline infusion is a new valuable tool for long-term treatment of advanced Shy-Drager syndrome." 01/01/1998 - "Ambulatory infusion of noradrenaline for long-term treatment of Shy-Drager syndrome." 03/01/1995 - "The Schellong test revealed deficient sympathetic orthostatic pressure response without an increase of plasma norepinephrine; therefore, a Shy-Drager syndrome was diagnosed. " 02/01/1995 - "At this point, after an extensive survey of the literature, the diagnosis of Shy-Drager syndrome was proposed and proved by monitoring ECG and serum levels of noradrenaline during postural changes. " 04/16/1985 - "[Ambulatory noradrenaline infusion in refractory Shy-Drager syndrome]."
7.	Dopamine (Intropin)FDA LinkGeneric 04/01/1976 - "Subsensitivity to dopamine was observed in patients with Parkinson's disease, but supersensitivity in those with the Shy-Drager syndrome." 10/02/1989 - "In both patients with Shy-Drager syndrome, basal plasma concentrations of epinephrine and dopamine were low under resting conditions and could not be stimulated in the upright position. " 04/01/1976 - "Blood pressure responses to noradrenaline and dopamine infusions in Parkinson's disease and the Shy-Drager syndrome." 04/01/1976 - "Studies of pulse rate and blood pressure responses to graded intravenous infusions of noradrenaline and dopamine were performed on five patients with Parkinson's disease, five with the Shy-Drager syndrome and seven healthy subject. " 06/01/1992 - "Autonomic neuropathies include those secondary to diabetes, peripheral autonomic impairment (the Bradbury-Eggleston syndrome), central autonomic impairment (the Shy-Drager syndrome), or newly described gene defects such as dopamine-beta-hydroxylase deficiency (which causes the absence of norepinephrine with an accumulation of dopamine). "
8.	Dopamine Agonists (Dopamine Agonist)IBA 04/01/1981 - "In a controlled trial lisuride, an ergolene derivative with dopamine receptor agonist properties was given maximum tolerated doses (2.4 mg/day) to seven patients with multiple system atrophy with autonomic failure (Shy-Drager syndrome). "
9.	XenonIBA 01/01/1979 - "The experimental study of the autoregulation of cerebral blood flow (CBF), of the cerebrovascular reactivity and of their interaction is performed in a confirmed case of Shy-Drager syndrome, by means of the xenon-clearance technique. "
10.	GABA-A Receptors (GABA(A) Receptor)IBA 08/01/1995 - "We used positron emission tomography with [11C]flumazenil to study gamma-aminobutyric acid type A/benzodiazepine receptor binding quantitatively in the cerebral hemispheres, basal ganglia, thalamus, cerebellum, and brainstem of 72 subjects, including 14 with multiple system atrophy of the ataxic (olivopontocerebellar atrophy) type, 5 with multiple system atrophy of the extrapyramidal/autonomic (Shy-Drager syndrome) type, 18 with sporadic olivopontocerebellar atrophy, 15 with dominantly inherited olivopontocerebellar atrophy, and 20 normal control subjects with similar age and sex distributions. "

Therapies and Procedures

1.	Therapeutics 07/01/1985 - "[Therapy of vesical dysfunction in the Shy-Drager syndrome]." 11/01/1992 - "[Effect of combined therapy with selective beta 1- and alpha 1-adrenergic agonists upon postprandial hypotension in patients with progressive autonomic failure]." 07/15/1995 - "[Shy-Drager syndrome--therapy experience with indomethacin]." 06/01/1973 - "Levodopa therapy for Parkinsonism in the Shy-Drager syndrome." 08/01/1976 - "While the limited and transient beneficial effect of levodopa on the bradykinesia in our case is possibly due to the progressive loss of striatal dopaminergic receptors seen in striatonigral degeneration, we propose that in Shy-Drager syndrome, levodopa therapy benefits orthostatic hypotension because of a suppression of the central depressor action of this drug. "
2.	Intravenous Infusions 03/01/1985 - "The arginine vasopressin response to an intravenous infusion of hypertonic saline was determined in six patients with progressive autonomic failure. " 08/01/1986 - "The blood pressure (BP) and heart rate (HR) responses to 5 min incremental intravenous infusions of noradrenaline (NA) and arginine vasopressin (AVP) were investigated both in patients with progressive autonomic failure (PAF) and in normal volunteers. " 04/01/1976 - "Studies of pulse rate and blood pressure responses to graded intravenous infusions of noradrenaline and dopamine were performed on five patients with Parkinson's disease, five with the Shy-Drager syndrome and seven healthy subject. "
3.	General Anesthesia 01/01/2001 - "A 53-year-old man with Shy-Drager syndrome underwent emergency surgery for the resection of the larynx under general anesthesia. " 06/01/1989 - "A 57-year-old man with Shy-Drager syndrome underwent plastic surgery for decubitus on the buttocks under general anesthesia. " 07/01/2005 - "A 61-year-old man with Shy-Drager Syndrome underwent partial pulmonary lobectomy under general anesthesia combined with thoracic epidural anesthesia. " 07/01/2005 - "[A patient with Shy-Drager syndrome managed under general anesthesia with thoracic epidural anesthesia]." 12/01/1994 - "Aggravated sleep apnea after general anesthesia in a patient with Shy-Drager syndrome."
4.	Anesthesia 01/01/2001 - "[Anesthesia for emergency surgery in a patient with Shy-Drager syndrome]." 10/01/1992 - "Anesthesia in Shy-Drager syndrome." 08/01/1979 - "[Shy-Drager syndrome and anesthesia]." 06/01/1989 - "[Anesthesia for Shy-Drager syndrome; effects of elastic bandage, phenylephrine, and IPPV]."
5.	Denervation 09/01/1988 - "In patients with progressive autonomic failure and multiple system atrophy, the bladder neck was incompetent in every case and the striated muscle of the urethra was affected by a process of denervation and re-innervation. " 04/01/1987 - "These findings do not permit the vocal cord paralysis seen in the Shy-Drager syndrome to be explained by motorneuron loss and denervation. " 01/01/1981 - "1. Denervation supersensitivity to adrenergic agonists occurs after degeneration of the sympathetic nervous system in the disease called multiple system atrophy (MSA) or the Shy Drager Syndrome.2. Supersensitivity to the chronotropic effect of i.v. isoprenaline on the heart was demonstrated in eight subjects with sympathetic nervous system degeneration and MSA.3. There was an increased number of beta-receptors present in MSA as measured by [H(3)]dihydroalprenolol ([H(3)]DHA) binding to beta-receptors on lymphocytes isolated from venous blood taken from the MSA subjects compared with [H(3)]DHA binding to lymphocytes from seven normal subjects. "