shy drager syndrome

Summary

Summary: 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, which differentiates this condition from other forms of idiopathic orthostatic hypotension (HYPOTENSION, ORTHOSTATIC). 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)

Top Publications

  1. ncbi Shy-Drager syndrome: multisystem atrophy with comorbid depression
    Jean Hemingway
    Department of Psychiatry and Psychology, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    Psychosomatics 46:73-6. 2005
  2. pmc The effects of water ingestion on orthostatic hypotension in two groups of chronic autonomic failure: multiple system atrophy and pure autonomic failure
    T M Young
    Neurovascular Medicine Unit, Faculty of Medicine, Imperial College London at St Mary s Hospital, London, UK
    J Neurol Neurosurg Psychiatry 75:1737-41. 2004
  3. ncbi New findings on the neuropathology of multiple system atrophy
    Eduardo E Benarroch
    Neurophysiological Laboratory, Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Auton Neurosci 96:59-62. 2002
  4. ncbi Deep brain stimulation improves orthostatic regulation of patients with Parkinson disease
    B Stemper
    Department of Neurology, University of Erlangen Nuernberg, Germany
    Neurology 67:1781-5. 2006
  5. ncbi Plasma levels of catechols and metanephrines in neurogenic orthostatic hypotension
    D S Goldstein
    Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD 20892 1620, USA
    Neurology 60:1327-32. 2003
  6. ncbi Assessment of autonomic dysfunction of multiple system atrophy with laryngeal abductor paralysis as an early manifestation
    Kazushi Deguchi
    Department of Gastroenterology and Neurology, Kagawa University School of Medicine, 1750 1 Ikenobe, Miki cho, Kita gun, Kagawa 761 0793, Japan
    Clin Neurol Neurosurg 109:892-5. 2007
  7. ncbi [Blood pressure and heart rate regulation in diabetics]
    P Valensi
    , , , avenue du 14-Juillet, 93143 Bondy
    Arch Mal Coeur Vaiss 93:51-8. 2000
  8. ncbi Recognizing and treating diabetic autonomic neuropathy
    A I Vinik
    the Strelitz Diabetes Research Institutes, Department of Internal Medicine and Anatomy/Neurobiology, Eastern Virginia Medical School, Norfolk, USA
    Cleve Clin J Med 68:928-30, 932, 934-44. 2001
  9. ncbi [Dopamine-beta-hydroxylase]
    Kazumasa Isobe
    Graduate School of Comprehensive Human Science, Tsukuba University
    Nihon Rinsho 63:391-4. 2005
  10. ncbi [Shy-Drager syndrome]
    Yukio Nakamura
    Department of Cardiology, National Hospital Organization, Kanazawa Medical Center
    Nippon Rinsho . 2007

Detail Information

Publications114 found, 100 shown here

  1. ncbi Shy-Drager syndrome: multisystem atrophy with comorbid depression
    Jean Hemingway
    Department of Psychiatry and Psychology, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    Psychosomatics 46:73-6. 2005
  2. pmc The effects of water ingestion on orthostatic hypotension in two groups of chronic autonomic failure: multiple system atrophy and pure autonomic failure
    T M Young
    Neurovascular Medicine Unit, Faculty of Medicine, Imperial College London at St Mary s Hospital, London, UK
    J Neurol Neurosurg Psychiatry 75:1737-41. 2004
    ....
  3. ncbi New findings on the neuropathology of multiple system atrophy
    Eduardo E Benarroch
    Neurophysiological Laboratory, Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Auton Neurosci 96:59-62. 2002
    ....
  4. ncbi Deep brain stimulation improves orthostatic regulation of patients with Parkinson disease
    B Stemper
    Department of Neurology, University of Erlangen Nuernberg, Germany
    Neurology 67:1781-5. 2006
    ..To evaluate whether subthalamic nucleus (STN) stimulation has an effect on the orthostatic regulation of patients with Parkinson disease (PD), we studied cardiovascular regulation during on and off phases of STN stimulation...
  5. ncbi Plasma levels of catechols and metanephrines in neurogenic orthostatic hypotension
    D S Goldstein
    Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD 20892 1620, USA
    Neurology 60:1327-32. 2003
    ..From the concept of a unitary sympathoadrenomedullary system, one might predict parallel sympathoneural and adrenomedullary abnormalities in NOH...
  6. ncbi Assessment of autonomic dysfunction of multiple system atrophy with laryngeal abductor paralysis as an early manifestation
    Kazushi Deguchi
    Department of Gastroenterology and Neurology, Kagawa University School of Medicine, 1750 1 Ikenobe, Miki cho, Kita gun, Kagawa 761 0793, Japan
    Clin Neurol Neurosurg 109:892-5. 2007
    ..Diagnosis of atypical MSA with LAP in the early stages might be accelerated by a detailed investigation focused on urinary symptoms and neuroendocrine approaches...
  7. ncbi [Blood pressure and heart rate regulation in diabetics]
    P Valensi
    , , , avenue du 14-Juillet, 93143 Bondy
    Arch Mal Coeur Vaiss 93:51-8. 2000
    ....
  8. ncbi Recognizing and treating diabetic autonomic neuropathy
    A I Vinik
    the Strelitz Diabetes Research Institutes, Department of Internal Medicine and Anatomy/Neurobiology, Eastern Virginia Medical School, Norfolk, USA
    Cleve Clin J Med 68:928-30, 932, 934-44. 2001
    ..Strict glycemic control can slow the onset of diabetic autonomic neuropathy and sometimes reverse it. Pharmacologic and nonpharmacologic therapies are available to treat symptoms...
  9. ncbi [Dopamine-beta-hydroxylase]
    Kazumasa Isobe
    Graduate School of Comprehensive Human Science, Tsukuba University
    Nihon Rinsho 63:391-4. 2005
  10. ncbi [Shy-Drager syndrome]
    Yukio Nakamura
    Department of Cardiology, National Hospital Organization, Kanazawa Medical Center
    Nippon Rinsho . 2007
  11. ncbi Catechol-o-methyltransferase and blood pressure in humans
    Jens Jordan
    Franz Volhard Clinical Research Center and Helios Klinikum Berlin, Medical Faculty of the Charite, Humboldt University, Berlin, Germany
    Circulation 106:460-5. 2002
    ..Whether catechol-O-methyltransferase (COMT), the enzyme that metabolizes extraneuronal norepinephrine, contributes to blood pressure regulation in humans is unknown...
  12. ncbi Modulation of QT interval during autonomic nervous system blockade in humans
    Andre Diedrich
    Autonomic Dysfunction Center, Vanderbilt University, Nashville, Tenn 37232 2195, USA
    Circulation 106:2238-43. 2002
    ..We used the alternative approach of interrupting neurotransmission at the level of autonomic ganglia to determine its effect on the QT interval...
  13. ncbi Shy-Drager syndrome and severe unexplained intraoperative hypotension responsive to vasopressin
    Ricardo Vallejo
    Anesthesiology Department, Jackson Memorial Hospital University of Miami, Florida, USA
    Anesth Analg 95:50-2, table of contents. 2002
    ..We suggest that vasopressin may be the drug of choice in patients with Shy-Drager syndrome with refractory hypotension...
  14. ncbi Spatial patterns of alpha-synuclein positive glial cytoplasmic inclusions in multiple system atrophy
    Richard A Armstrong
    Vision Sciences, Aston University, Birmingham, United Kingdom
    Mov Disord 19:109-12. 2004
    ..These spatial patterns contrast with those reported for filamentous neuronal inclusions in the tauopathies and alpha-synucleinopathies...
  15. ncbi Blood pressure recovery from Valsalva maneuver in patients with autonomic failure
    Elizabeth R Vogel
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 65:1533-7. 2005
    ..Blood pressure (BP) changes in response to the Valsalva maneuver (VM) reflect the integrity of the baroreflex that regulates BP, and the phases of VM are widely used indices of adrenergic evaluation...
  16. ncbi Pyridostigmine treatment trial in neurogenic orthostatic hypotension
    Wolfgang Singer
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Arch Neurol 63:513-8. 2006
    ..By enhancing ganglionic transmission, pyridostigmine bromide can potentially ameliorate OH without worsening supine hypertension...
  17. ncbi Acute paraneoplastic dysautonomia
    A Al-Shekhlee
    Department of Neurology, University Hospitals of Cleveland, Case Western Reserve University, Cleveland, Ohio 44106, USA
    Eur Neurol 49:64-5. 2003
  18. pmc Excessive nitric oxide function and blood pressure regulation in patients with autonomic failure
    Alfredo Gamboa
    Department of Medicine, Vanderbilt University, Nashville, Tenn 37212, USA
    Hypertension 51:1531-6. 2008
    ..Potentiation of NO could be used in the treatment of supine hypertension, and its inhibition offers a novel approach to improve orthostatic hypotension...
  19. ncbi Treating blood pressure in familial dysautonomia
    Irwin J Schatz
    Clin Auton Res 15:249-50. 2005
  20. pmc From orthostatic hypotension to Shy-Drager syndrome
    J M S Pearce
    J Neurol Neurosurg Psychiatry 75:1666. 2004
  21. ncbi Octreotide for orthostatic hypotension
    M Lamarre-Cliche
    Clinical Pharmacology, Centre Hospitalier de l Universite de Montreal CHUM, Canada
    Can J Clin Pharmacol 6:213-5. 1999
    ..It is a useful adjunct for the short term treatment of orthostatic hypotension associated with dysautonomia. Its long term efficacy and safety need to be studied...
  22. ncbi Chronic hypotension. In the shadow of hypertension
    D Robertson
    Department of Medicine, Vanderbilt University, Nashville, TN 37232-2195
    Am J Hypertens 5:200S-205S. 1992
    ..The differentiation of these and other causes of symptomatic hypotension may in many cases lead to beneficial therapy...
  23. ncbi [Central nervous system disorders]
    Ryuji Sakakibara
    Department of Neurology, Chiba University School of Medicine
    Nihon Rinsho 60:315-21. 2002
  24. ncbi Autonomic control after blockade of the norepinephrine transporter: a model of orthostatic intolerance
    Robert P Carson
    Autonomic Dysfunction Center, Vanderbilt University, Nashville, Tennessee 37232 2195, USA
    J Appl Physiol 93:2192-8. 2002
    ..Thus attenuated baroreflex function and reduced sympathetic outflow may contribute to the orthostatic intolerance of severe NET deficiency...
  25. ncbi Cardiac and extracardiac sympathetic denervation in Parkinson's disease with orthostatic hypotension and in pure autonomic failure
    Dnyanesh N Tipre
    Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Nucl Med 46:1775-81. 2005
    ..In this study, we examined whether 6-(18)F-fluorodopamine can visualize sympathetic innervation in extracardiac organs and, if so, whether patients with PD+OH or PAF have neuroimaging evidence of extracardiac noradrenergic denervation...
  26. ncbi [Morphological magnetic resonance imaging: its value for the diagnosis of neurodegenerative diseases]
    U Hahn
    Praxis für Radiologische Diagnostik und Nuklearmedizin, Stuttgart Bad Cannstatt
    Dtsch Med Wochenschr 133:S15-8. 2008
    ..MRI is of minor importance for differentiating Alzheimer's disease from frontotemporal dementia or dementia with Lewy bodies. However, specific patterns are found in cerebral amyloid angiopathy and prion diseases....
  27. ncbi Cardiac response rate variability in physically abused women of childbearing age
    Michael J Rice
    Washington State University, Intercollegiate College of Nursing, 2917 W Ft Wright Drive, Spokane, WA 99224, USA
    Biol Res Nurs 7:204-13. 2006
    ..These findings support further testing with larger samples to identify vasovagal changes in abused pregnant women...
  28. ncbi [Autonomic dysfunction and orthostatic intolerance. Cardiologic diagnosis and treatment options]
    Thomas Klingenheben
    Praxis für Kardiologie, Bonn
    Med Klin (Munich) 101:76-9. 2006
    ..definition of these disorders has changed significantly. This review summarizes the current knowledge of diagnosis and treatment of the syndromes of orthostatic intolerance...
  29. ncbi Changes in cerebral oxygenation and haemodynamics during postural blood pressure changes in patients with autonomic failure
    Katharine Hunt
    Department of Neuroanaesthesia and Neurocritical Care, The National Hospital for Neurology and Neurosurgery and Institute of Neurology, Queen Square, London WC1N 3BG, UK Centre for Anaesthesia, University College London, UK
    Physiol Meas 27:777-85. 2006
    ..Postural hypotension in patients with autonomic failure is associated with a substantial decrease in absolute cerebral oxygenation measured by NIRS and this might reflect a critical reduction in cerebral oxygen delivery...
  30. ncbi Functional neuroimaging of sympathetic innervation of the heart
    David S Goldstein
    Building 10, Room 6N252, NINDS, NIH, 10 Center Drive, MSC 1620, Bethesda, MD 20892 1620, USA
    Ann N Y Acad Sci 1018:231-43. 2004
    ..Analogous abnormalities might help explain increased cardiovascular risk in psychiatric disorders such as melancholic depression...
  31. ncbi Drug treatment of orthostatic hypotension because of autonomic failure or neurocardiogenic syncope
    Maxime Lamarre-Cliche
    Hôpital Européen Georges Pompidou Centre d Investigations Cliniques, Paris, France
    Am J Cardiovasc Drugs 2:23-35. 2002
    ..Little is known about the effectiveness and tolerability of specific combinations of pressor drugs. Consequently, sound clinical judgment and close follow-up of patients should always guide combination therapy...
  32. ncbi Orthostatic hypotension and attention in Parkinson's disease with and without dementia
    C Peralta
    Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, Austria
    J Neural Transm 114:585-8. 2007
    ..828, P < 0.05; DeltaDBP and TEA-2, r = 0.828, P < 0.05). We conclude that OH is frequent in PDD and should be addressed therapeutically since it may exacerbate attentional dysfunction...
  33. pmc Pupil findings in a consecutive series of 150 patients with generalised autonomic neuropathy
    F Bremner
    Department of Neuro Ophthalmology, internal box 142, National Hospital for Neurology and Neurosurgery, Queen Square, PO Box 142, London WC1N 3BG, UK
    J Neurol Neurosurg Psychiatry 77:1163-8. 2006
    ..To detect and characterise the pattern and extent of pupil abnormalities in patients with generalised autonomic failure...
  34. ncbi Clinical effects of elastic bandage on neurogenic orthostatic hypotension
    Y Hasegawa
    Dept of Neurology, Nagoya City Higashi General Hospital, Nagoya, Japan
    J Gravit Physiol 7:P159-60. 2000
    ..We investigated short-term clinical effects of commercially available EB on OH, and estimated the mechanism of its effectiveness by measuring some blood pressure-related humoral variables in neurodegenerative patients with OH...
  35. ncbi Ambulatory blood pressure variations relative to sitting or standing position in renal transplant patients
    Jean Phillippe Baguet
    Department of Cardiology and Hypertension bDepartment of Nephrology, Grenoble University Hospital, Grenoble, France
    Blood Press Monit 10:93-6. 2005
    ..The main objective of this study was to analyse the variations in blood pressure and heart rate recorded by ambulatory measurement during changes in position in renal transplant patients...
  36. ncbi The nature of the autonomic dysfunction in multiple system atrophy
    Samir M Parikh
    Autonomic Dysfunction Center, Department of Medicine, Vanderbilt University, AA 3228 MCN, Nashville, TN 37232 2195, USA
    J Neurol Sci 200:1-10. 2002
    ..We will review recent evidence bearing on autonomic tone in MSA and discuss their therapeutic implications, particularly in terms of the possible development of a bionic baroreflex for better control of blood pressure...
  37. ncbi Multiple system atrophy: alpha-synuclein and neuronal degeneration
    Mari Yoshida
    Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Aichi, Japan
    Neuropathology 27:484-93. 2007
    ..The oligo-myelin-axon-neuron complex mechanism, along with the direct involvement of neurons themselves, may synergistically accelerate the degenerative process of MSA...
  38. ncbi Familial mydriasis, cardiac arrhythmia, respiratory failure, muscular weakness and hypohidrosis
    O Sjaastad
    Department of Neurology and the Laboratory for the study of autonomic disorders, Trondheim University Hospital, Norway
    Acta Neurol Scand Suppl 174:3-31. 2000
    ..The main features were: mydriasis, cardiac arrhythmia, cardiomegaly, hypohidrosis, respiratory failure, and muscular weakness...
  39. ncbi [Syndromes of autonomic insufficiency associated with orthostatic intolerance: classification, diagnostic and therapeutic approach]
    B P Grubb
    Medical College of Ohio, Dept of Medicine, Div of Cardiology, Richard D Ruppert Health Center, Toledo 43614 5809, USA
    Z Kardiol 88:541-9. 1999
    ..This review summarizes the current knowledge of autonomic disorders, their classification and diagnostic and therapy strategies...
  40. ncbi [General anesthesia in a patient with idiopathic orthostatic hypotension]
    I Ionescu
    Zentrum für Anästhesiologie und Intensivmedizin, Klinik fur Anasthesiologie, Universitatsklinikum Hamburg Eppendorf, Deutschland
    Anaesthesist 59:140-3. 2010
    ..In the case presented perioperative hemodynamic stability was achieved by aggressive volume therapy guided by global end-diastolic volume measurement and low-dose catecholamine therapy...
  41. ncbi Intrathecal baclofen for autonomic instability due to spinal cord injury
    Markus Kofler
    Department of Neurology, Hospital Hochzirl, Austria
    Auton Neurosci 146:106-10. 2009
    ..This case demonstrates that ITB administration may help to stabilize autonomic dysreflexia and orthostatic hypotension in patients with spinal cord lesions...
  42. ncbi Apolipoprotein E e4 allele frequency in patients with multiple system atrophy
    N J Cairns
    Brain Bank, Department of Neuropathology, Institute of Psychiatry, London, UK
    Neurosci Lett 221:161-4. 1997
    ..The APOE e4 allele frequency of the MSA cases (0.11) was not significantly different from that of the control subjects (0.12). These data indicate that the APOE e4 allele is not a risk factor for multiple system atrophy...
  43. ncbi [Historical overview of REM sleep behavior disorder in relation to its pathophysiology]
    Naoko Tachibana
    Department of Neurology and Center for Sleep related Disorders, Kansai Electric Power Hospital, 2 1 7 Fukushima, Fukushima ku, Osaka 553 0003, Japan
    Brain Nerve 61:558-68. 2009
    ..RBD is not a homogeneous clinical entity, and further refinement of its diagnostic classification is warranted to avoid diagnostic confusion...
  44. ncbi [Development of L-threo-DOPS, a norepinephrine precursor amino acid]
    J Katsube
    Sumitomo Pharmaceuticals Co, Ltd, Osaka, Japan
    Yakugaku Zasshi 114:823-46. 1994
    ..The R & D of L-DOPS described in this paper includes studies on industrial production, efficacy pharmacology (mode of action), metabolism and clinical trial of this agent...
  45. ncbi Determination of D,L-threo-3,4-dihydroxyphenylserine and of the D- and L-enantiomers in human plasma and urine
    F Boomsma
    Department of Internal Medicine I, University Hospital Dijkzigt, Erasmus University, Rotterdam, The Netherlands
    J Chromatogr 427:219-27. 1988
    ..28; r = 0.997; n = 52). Excellent agreement was found between the sum of the D- and L-DOPS concentrations and the measured total D,L-DOPS concentration (regression line DOPS (D + L) = 0.955 DOPS (total, LE) + 116.65; r = 0.992; n = 100)...
  46. ncbi Orthostatic intolerance and the postural tachycardia syndrome: genetic and environment pathophysiologies. Neurolab Autonomic Team
    D Robertson
    Center for Space Physiology and Medicine, Clinical Research Center, Vanderbilt University, Nashville, TN 37232 2195, USA
    Pflugers Arch 441:R48-51. 2000
    ..This leads to poor clearance of norepinephrine from synapses, with consequent raised heart rate. Clinical features of these syndromes are presented...
  47. ncbi Orthostatic intolerance in pediatrics
    Julian M Stewart
    Department of Pediatrics and Physiology, The Center for Pediatric Hypotension, New York Medical College, Valhalla 10595, USA
    J Pediatr 140:404-11. 2002
  48. ncbi [Anesthesia for emergency surgery in a patient with Shy-Drager syndrome]
    H Hashimoto
    Department of Anesthesiology, University of Tokyo, Faculty of Medicine, Tokyo 113-8655
    Masui 50:40-1. 2001
    ..He was extubated on the first postoperative day without any complications. The effects of vecuronium, norepinephrine, and dopamine might be changed in the patient with Shy-Drager syndrome...
  49. ncbi Dysautonomia and cognitive dysfunction in Parkinson's disease
    Werner Poewe
    Department of Neurology, Medical University Innsbruck, Innsbruck, Austria
    Mov Disord 22:S374-8. 2007
    ..This raises the possibility that spread of synuclein pathology to involve neocortical areas producing cognitive decline could be somehow linked to involvement of the autonomic nervous system in PD...
  50. pmc New squatting test indices are useful for assessing baroreflex sensitivity in diabetes mellitus
    M Nakagawa
    Department of Cardiovascular Science, Division of Laboratory Medicine, Oita University School of Medicine, Oita, Japan
    Diabet Med 25:1309-15. 2008
    ..Our aim was to develop new simple squatting test indices and to analyse their applicability to the assessment of baroreflex sensitivity (BRS) in patients with diabetes...
  51. ncbi Characteristic MRI findings in multiple system atrophy: comparison of the three subtypes
    H Naka
    Third Department of Internal Medicine, Hiroshima University School of Medicine, Japan
    Neuroradiology 44:204-9. 2002
    ..Our findings suggest that, although MSA can be divided clinically into three subtypes, most of the features on MRI are common and overlap in the subtypes, independently of the clinical presentation...
  52. ncbi [A case of pure autonomic failure (PAF) with sleep apnea syndrome (SAS) and successful treatment of dysautonomia with nasal continuous positive airway pressure (CPAP)]
    Eri Hamada
    Department of Neurology, Ehime Prefectural Central Hospital
    Nihon Ronen Igakkai Zasshi 41:233-6. 2004
    ..This case raised the possibility that nasal CPAP may be useful for orthostatic hypotension as well as SAS in neurodegenerative diseases...
  53. ncbi [MSA update]
    Shoji Tsuji
    Department of Neurology, Graduate School of Medicine, University of Tokyo
    Rinsho Shinkeigaku 45:821-3. 2005
    ..Integrated analyses of non-parametric linkage analyses on the familial MSA cases and association studies on sporadic MSA cases are expected to accelerate the studies on identification of genes involved in the pathogenesis of MSA...
  54. ncbi Autonomic nervous system function in chronic dizziness
    Jeffrey P Staab
    Department of Psychiatry, University of Pennsylvania Health System, Philadelphia, Pennsylvania 19104, USA
    Otol Neurotol 28:854-9. 2007
    ....
  55. ncbi [Mechanism of neuronal degeneration of multiple system atrophy]
    Mari Yoshida
    Institute for Medical Science of Aging, Aichi Medical University, 21 Karimata, Yazako, Nagakute cho, Aichi Gun, Aichi 480 1195
    Brain Nerve 61:1051-60. 2009
    ..Therefore, both the direct involvement of neurons themselves and the oligodendroglia-myelin-axon mechanism may synergistically accelerate the degenerative process of MSA...
  56. ncbi Excessive daytime sleepiness and unintended sleep in Parkinson's disease
    David B Rye
    Department of Neurology, Emory University School of Medicine, 101 Woodruff Circle, WMRB Suite 6000, PO Drawer V, Atlanta, GA 30322, USA
    Curr Neurol Neurosci Rep 6:169-76. 2006
    ..Sleepiness in parkinsonism, especially a narcolepsy-like phenotype, may necessitate treatment with wake-promoting agents such as bupropion, modafinil, or traditional psychostimulants...
  57. pmc Are the orthostatic fluid shifts to the calves augmented in autonomic failure?
    Roland D Thijs
    Department of Neurology and Clinical Neurophysiology, Leiden University Medical Centre, Postal Zone J3 R, PO Box 9600, 2300 RC, Leiden, The Netherlands
    Clin Auton Res 20:19-25. 2010
    ..In autonomic failure (AF), blood pressure (BP) falls upon standing which is commonly ascribed to defective vasoconstriction and excessive pooling. Observations on the amount of pooling in AF are contradictory...
  58. ncbi Chiari drop attacks: surgical decompression and the role of tilt table testing
    David Straus
    Pritzker School of Medicine, Chicago, IL, USA
    Pediatr Neurosurg 45:384-9. 2009
    ..It has been postulated that Chiari drop attack is secondary to dysautonomia caused by hindbrain compression. There has been recent debate regarding the association between CM1, dysautonomia and Chiari drop attack...
  59. ncbi Cerebral vasomotor reactivity in Parkinson's disease, multiple system atrophy and pure autonomic failure
    Tanya Gurevich
    Neurology Department, Tel Aviv Medical Center, Israel Sieratzki Chair of Neurology, Tel Aviv University, Israel
    J Neurol Sci 243:57-60. 2006
    ..We conclude that BFV is normal in PD, MSA and PAF and that the VMR, as investigated by TCD and the Diamox test, did not disclose differences in cerebral vasomotor responses between these conditions...
  60. ncbi Dopamine agonist withdrawal syndrome in Parkinson disease
    Christina A Rabinak
    Department of Neurology and Neuroscience, Weill Cornell Medical College, New York, New York 10021, USA
    Arch Neurol 67:58-63. 2010
    ..To report and characterize a dopamine agonist (DA) withdrawal syndrome (DAWS) in Parkinson disease...
  61. ncbi Red flags for multiple system atrophy
    Martin Köllensperger
    Section for Clinical Neurobiology, Department of Neurology, Innsbruck Medical University, Austria
    Mov Disord 23:1093-9. 2008
    ..9 (+/-7.0) months earlier than with the Consensus criteria alone. We propose a combination of two out of six red flag categories as additional diagnostic criteria for probable MSA-P...
  62. ncbi [Orthostatic hypotension]
    Yukio Nakamura
    Department of Cardiology, National Hospital Organization, Kanazawa Medical Center
    Nippon Rinsho . 2007
  63. ncbi Neuropharmacologic distinction of neurogenic orthostatic hypotension syndromes
    Yehonatan Sharabi
    Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 1620, USA
    Clin Neuropharmacol 29:97-105. 2006
    ....
  64. ncbi Recent developments in multiple system atrophy
    Gregor K Wenning
    Section of Clinical Neurobiology, Department of Neurology, Innsbruck Medical University, Anichstrasse 35, 6020 Innsbruck, Austria
    J Neurol 256:1791-808. 2009
    ..An outline of the rationale for managing symptomatic deterioration in MSA is provided together with a summary of novel experimental therapeutic approaches to decrease disease progression...
  65. ncbi Effects of daily water drinking on orthostatic and postprandial hypotension in patients with multiple system atrophy
    Kazushi Deguchi
    Dept of Gastroenterology and Neurology, Kagawa University Faculty of Medicine, 1750 1 Ikenobe, Kita gun, Kagawa, 761 0793, Japan
    J Neurol 254:735-40. 2007
    ..The aim of this study was to evaluate the practical effect of daily water drinking on OH and PPH in the morning when patients with AF are usually most affected...
  66. pmc Other autonomic neuropathies associated with ganglionic antibody
    Paola Sandroni
    Mayo Clinic, Department of Neurology, 200 First Street SW, Rochester, MN 55905, USA
    Auton Neurosci 146:13-7. 2009
    ..Other antigens and antibodies yet to be identified are more likely to be responsible in these disorders...
  67. ncbi Is cerebral autoregulation impaired in Parkinson's disease? A transcranial Doppler study
    N Vokatch
    Department of Neurology, Geneva University Hospitals and Medical School, Switzerland
    J Neurol Sci 254:49-53. 2007
    ..These results provide evidence of an impaired cerebral autoregulation in PD patients which appears independent of dopaminergic treatment...
  68. ncbi Normal cardiovascular reflex testing in patients with parkin disease
    Francesca Del Sorbo
    Istituto Neurologico Carlo Besta, Milano, Italy
    Mov Disord 22:528-32. 2007
    ..Urinary dysautonomia warrants further investigation in patients with parkin disease...
  69. ncbi Autoimmune autonomic ganglionopathy with Sjögren's syndrome: significance of ganglionic acetylcholine receptor antibody and therapeutic approach
    Takayuki Kondo
    Department of Neurology, Kyoto University Hospital, Kyoto, Japan
    Auton Neurosci 146:33-5. 2009
    ..These two cases indicate that chronic AAG may be treatable by immunomodulatory therapy, and have relevance to SS...
  70. pmc Clinical laboratory evaluation of autoimmune autonomic ganglionopathy: Preliminary observations
    David S Goldstein
    Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, Bethesda, MD 20892 1620, USA
    Auton Neurosci 146:18-21. 2009
    ..PAF entails cardiac sympathetic denervation, whereas cardiac sympathetic neuroimaging by thoracic 6-[(18)F]fluorodopamine scanning indicates intact myocardial sympathetic innervation in AAG...
  71. doi Postural tachycardia syndrome
    Blair P Grubb
    Department of Medicine, University of Toledo, 3000 Arlington Ave, Toledo, Ohio 43614, USA
    Circulation 117:2814-7. 2008
  72. ncbi RNA sequestration to pathological lesions of neurodegenerative diseases
    S D Ginsberg
    Center for Neurodegenerative Disease Research and Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104 4283, USA
    Acta Neuropathol 96:487-94. 1998
    ..These observations demonstrate the selective localization of RNA species to distinct pathological lesions of neurodegenerative disease brains...
  73. ncbi Evaluation and management of autonomic nervous system disorders
    Caroline M Klein
    Department of Neurology, The University of North Carolina School of Medicine, 3114 Bioinformatics, Campus Box 7025, Chapel Hill, NC 27599 7025, USA
    Semin Neurol 28:195-204. 2008
    ..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...
  74. ncbi Quantitative autonomic testing in the management of botulism
    Raffi Topakian
    Department of Neurology, Academic Teaching Hospital Wagner Jauregg, Wagner Jauregg Weg 15, Linz 4020, Austria
    J Neurol 256:803-9. 2009
    ..QAT identified autonomic involvement in botulism patients with otherwise benign neurological presentation. Comprehensive evaluation of autonomic failure may provide useful information for the management of botulism...
  75. ncbi Aging of complex heart rate dynamics
    Zbigniew R Struzik
    Educational Physiology Laboratory, Graduate School of Education, The University of Tokyo, Japan
    IEEE Trans Biomed Eng 53:89-94. 2006
    ..Based upon this, we propose a novel picture of "autonomic aging," characterized by an insufficiency of the SNS function to cope dynamically with various environmental stimuli...
  76. ncbi Case report: Marathon runner with severe autonomic failure
    Emily M Garland
    Autonomic Dysfunction Center, Vanderbilt University, Nashville, TN 37232, USA
    Lancet 366:S13. 2005
  77. ncbi Unusual causes of severe orthostatic hypotension
    M Abu Nasar
    Hosp Med 63:240-1. 2002
  78. ncbi Diagnosis and treatment of supine hypertension in autonomic failure patients with orthostatic hypotension
    Jens Jordan
    Hypotension and Syncope Unit, Clinical Research Center, Franz Volhard Clinic, Medical Faculty of the Charite, Humboldt University, Berlin, Germany
    J Clin Hypertens (Greenwich) 4:139-45. 2002
    ..During the day, avoiding the supine position is often all that is required. Short-acting vasodilators (e.g., transdermal nitroglycerin) can be used during the night...
  79. ncbi [A case of "pure" progressive autonomic failure in an elderly male]
    T Kuwahara
    Second Department of Internal Medicine, Ehime University School of Medicine
    Nihon Ronen Igakkai Zasshi 29:790-3. 1992
    ..It shows symptoms of autonomic failure without other neurological disturbances which manifest as Shy-Drager syndrome. Treatment with L-DOPS increased his blood pressure level and attenuated his symptoms due to orthostatic hypotension...
  80. ncbi Neurodegeneration, parkinsonian syndromes and autonomic failure
    Christopher J Mathias
    University Department of Clinical Neurology, National Hospital for Neurology, University College London, UK
    Auton Neurosci 96:50-8. 2002
  81. ncbi [Dysautonomic features in patients with Cuban type 2 spinocerebellar ataxia]
    G Sánchez-Cruz
    Clínica Cubana para la Investigación y Rehabilitación de las Ataxias Hereditarias CIRAH, Holguin, Cuba
    Rev Neurol 33:428-34. 2001
    ..Dysautonomic features are the clinical signs and symptoms derived from anomalous functioning of the sympathetic or parasympathetic nervous systems in either the peripheral or central parts...
  82. ncbi [Autonomic dysfunction with nocturnal dyspnea (Gerhardt-syndrome) in a patient with multiple system atrophy]
    M Eissler
    Allgemeinmedizin,
    Med Klin (Munich) 96:626-31. 2001
    ..TREATMENT AND COURSE: The patient needed a tracheotomy and treatment of the autonomic symptoms. He could be discharged in better psychic condition, without syncopes or respiratory problems...
  83. ncbi Autonomic failure after stroke--is it indicative for pathophysiology of complex regional pain syndrome?
    B Riedl
    Neurologische Klink, Friedrich Alexander Universitat, Erlangen, Germany
    Acta Neurol Scand 103:27-34. 2001
    ..In conclusion, our investigation suggests that many clinical symptoms and the main features of sympathetic dysfunction in CRPS could be explained by a CNS pathophysiology...
  84. ncbi Overexpression of the alpha1B-adrenergic receptor causes apoptotic neurodegeneration: multiple system atrophy
    M J Zuscik
    Department of Molecular Cardiology NB50, The Lerner Research Institute, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44195, USA
    Nat Med 6:1388-94. 2000
    ..Our results indicate that overstimulation of alpha1BAR leads to apoptotic neurodegeneration with a corresponding multiple system atrophy indicative of Shy-Drager syndrome, a disease whose etiology is unknown...
  85. ncbi Diurnal blood pressure variation in progressive autonomic failure
    M J Carvalho
    Centro de Estudos de Função Autonomica, Hospital S Joao, Oporto Medical School, Oporto, Portugal
    Hypertension 35:892-7. 2000
    ..For the maintenance of a normal circadian BP pattern, not only an intact sympathetic but also an intact afferent parasympathetic ANS is a prerequisite...
  86. ncbi Visceral orthostatic hypotension in patients with severe autonomic dysfunction
    H Terazaki
    First Department of Internal Medicine, Kumamoto University School of Medicine, Honjo, Japan
    J Auton Nerv Syst 68:101-8. 1998
    ..These results suggest that FAP is a disease for which this kind of ultrasonographic examination should be applied, and that Doppler ultrasonography may be a helpful tool to evaluate visceral OH...
  87. ncbi Autoimmune autonomic failure in a patient with myeloma-associated Shy-Drager syndrome
    D S Goldstein
    Clinical Neuroscience Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD 20892, USA
    Clin Auton Res 6:17-21. 1996
    ..Since in this case the monoclonal antibody produced by the myeloma bound specifically to LC cells, the results are consistent with the hypothesis that in this patient the Shy-Drager syndrome may have had an immune-mediated basis...
  88. ncbi Electro-oculogram in multiple system and late onset cerebellar atrophies
    J Arpa
    Servicio de Neurologia, Hospital La Paz, Madrid
    Rev Neurol 23:969-74. 1995
    ..05) and SND subjects (p < 0.01). SDS also showed abnormalities of the oculomotor system...
  89. ncbi High beta-adrenoceptor density on peripheral blood mononuclear cells in progressive multiple sclerosis: a manifestation of autonomic dysfunction?
    Y Zoukos
    Multiple Sclerosis Laboratory, National Hospital for Neurology and Neurosurgery, London, England
    Acta Neurol Scand 90:382-7. 1994
    ....
  90. ncbi Effect of L-threo-3,4-dihydroxyphenylserine on muscle sympathetic nerve activities in Shy-Drager syndrome
    T Kachi
    Department of Neurology, National Chubu Hospital, Obu, Japan
    Neurology 38:1091-4. 1988
    ..We suggest that the OH improved mainly because of the increase in MSA due to L-threo-DOPS, and that the drug may activate sympathetic outflow at a site proximal to the sympathetic ganglion...
  91. ncbi Beta-adrenergic blood pressure regulation in Shy-Drager syndrome and pheochromocytoma
    M Middeke
    Medizinische Poliklinik, Ludwig Maximilians Universitt, Munchen
    Klin Wochenschr 67:1004-9. 1989
    ..The findings in pheochromocytoma add support to the theory that an acute catecholamine stimulation gives rise to an acute beta-adrenergic sensitization leading to blood pressure elevation.(ABSTRACT TRUNCATED AT 250 WORDS)..
  92. pmc Shy-Drager syndrome. Effect of fludrocortisone and L-threo-3,4-dihydroxyphenylserine on the blood pressure and regional cerebral blood flow
    S Matsubara
    Department of Neurology, Kanazawa University School of Medicine, Japan
    J Neurol Neurosurg Psychiatry 53:994-7. 1990
    ..Fludrocortisone reduced the fall of the mean blood pressure significantly. DOPS reduced the fall of both the diastolic blood pressure and rCBF significantly...
  93. ncbi Xamoterol in the treatment of orthostatic hypotension associated with multiple system atrophy (Shy-Drager syndrome)
    J N West
    University of Birmingham Department of Cardiovascular Medicine, East Birmingham Hospital
    Q J Med 74:209-13. 1990
    ..Intravenous test doses of xamoterol did not predict either the attenuation of orthostatic hypotension or the development of supine hypertension in all patients...
  94. ncbi Postural variation in intraocular pressure in primary chronic autonomic failure
    Martin J Dumskyj
    Department of Oncology, Gastroenterology, Endocrinology and Metabolism, St George s Hospital Medical School, Cranmer Terrace, London, UK, SW17 ORE
    J Neurol 249:712-8. 2002
    ..These data show that patients with autonomic failure are subject to large posture-related changes in IOP. These appear to be related to the large posture-induced changes in systemic blood pressure which occur in these patients...
  95. ncbi Hypertension in orthostatic hypotension and autonomic dysfunction
    Italo Biaggioni
    Departments of Medicine and Pharmacology, Division of Clinical Pharmacology, 1500 21st Avenue South, Suite 3500, Vanderbilt University, Nashville, TN 37212, USA
    Cardiol Clin 20:291-301, vii. 2002
    ..Supine hypertension complicates the treatment of these patients but can be managed by overnight administration of antihypertensive medications...
  96. ncbi Helicobacter pylori infection and gastric function in primary autonomic neuropathy
    Simona Maule
    U O A D U Medicina 4, Ospedale San Vito, Torino, Italy
    Clin Auton Res 12:193-6. 2002
    ..Hp detection might be important for the gastrointestinal and extradigestive complications of such infection...
  97. ncbi Duplication of distal part of the basilar artery
    Masanori Tsutsumi
    Department of Neurosurgery, Fukuoka University Chikushi Hospital, Chikushino City, Japan
    Radiat Med 20:265-7. 2002
    ..We report a case of duplication or fenestration of the distal part of the basilar artery with Shy-Drager syndrome. Duplication and fenestration of the basilar artery are discussed, and a review of embryologic development is presented...
  98. ncbi [Dopamine]
    Ryoji Ozono
    Department of Clinical Laboratory Medicine, Hiroshima University Graduate School of Biomedical Sciences
    Nihon Rinsho 63:388-90. 2005
  99. ncbi The Lewis family revisited: no evidence for autosomal dominant multiple system atrophy
    Jose Berciano
    Department of Neurology, University Hospital Marqués de Valdecilla UC, Santander, Spain
    Parkinsonism Relat Disord 11:363-5. 2005
    ..There remains no conclusive evidence to support the notion of hereditary MSA...
  100. ncbi [A patient with Shy-Drager syndrome managed under general anesthesia with thoracic epidural anesthesia]
    Kenji Shigematsu
    Department of Anesthesiology, Fukuoka University School of Medicine, Fukuoka
    Masui 54:788-90. 2005
    ..It did not increase as expected with intravenous ephedrine. Intravenous etilefrine promptly raised the blood pressure. There was no postoperative cardiorespiratory complications...
  101. ncbi The PINK1 phenotype can be indistinguishable from idiopathic Parkinson disease
    A Albanese
    National Neurologic Institute Carlo Besta, Universita Cattolica del Sacro Cuore, Milan, Italy
    Neurology 64:1958-60. 2005
    ....

Research Grants4

  1. Bionic Baroreflex System for Blood Pressure Control
    Andre Diedrich; Fiscal Year: 2002
    ..A Phase II study would involve studies in animals and in patients with MSA to determine the long-term efficacy of the bionic baroreflex system in improving blood pressure control...
  2. BRAINSTEM SUBSTRATES OF DOPAMINE MODULATED MOVEMENTS
    David Rye; Fiscal Year: 2002
    ..g., OCD, ADD, Tourette's and schizophrenia, the results will also bear relevance to a wider spectrum of disease. ..
  3. SLEEP/DOPAMINE PHENOTYPES IN GENETICALLY DISTINCT MICE
    David Rye; Fiscal Year: 2002
    ..abstract_text> ..
  4. Circuitry of Midbrain Dopamine in Sleep & Wake
    David Rye; Fiscal Year: 2005
    ..g., schizophrenia) and hypodopaminergic (e.g., PD and restless legs/periodic leg movements of sleep). ..