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Shy Drager Syndrome


Presentation

  • The case of an elderly male with Shy-Drager syndrome is presented. His presentation to the Sleep Clinic for assessment of snoring illustrates bilateral abductor vocal fold palsy as a rare presentation of the syndrome.[ncbi.nlm.nih.gov]
  • The most common otolaryngologic presentation of this syndrome is with stridor attributable to abductor paralysis of the vocal cords. The association with endolymphatic hydrops has not been previously described.[ncbi.nlm.nih.gov]
  • We present the case along with literature review of bilateral vocal cord paralysis with the Shy-Drager syndrome.[ncbi.nlm.nih.gov]
  • Nursing Care of the patient and family coping with Shy Drager Syndrome and the challenges it presents to the Movement Disorder Nurse are discussed. A coordinate, multidisciplinary team approach is suggested.[ncbi.nlm.nih.gov]
  • A case of Shy-Drager syndrome (proven at autopsy) initially presented as a depressive disorder. Shy-Drager syndrome should be added to the list of subcortical neurologic disorders which may initially present with an affective disturbance.[ncbi.nlm.nih.gov]
Falling
  • The abnormal NA response to exercise was consistent with the BP fall being due to inadequate compensatory sympathetic activity. In SDS, the small NA increase, in the presence of supersensitivity, may have reduced their BP fall as compared to PAF.[ncbi.nlm.nih.gov]
  • Fludrocortisone reduced the fall of the mean blood pressure significantly. DOPS reduced the fall of both the diastolic blood pressure and rCBF significantly.[ncbi.nlm.nih.gov]
  • In both SDS groups there were abnormal responses to exercise: blood pressure fell in SDS-C, but did not fall or rise in SDS-P.[ncbi.nlm.nih.gov]
  • After the patient received DL-threo-DOPS, the fall in his mean arterial blood pressure on head-up tilting was reduced and he had no syncope when standing.[ncbi.nlm.nih.gov]
  • The main haemodynamic defect was an impressive postural fall in stroke volume and cardiac output pointing to denervation of the capacitance vessels.[ncbi.nlm.nih.gov]
Fatigue
  • Stress and fatigue can intensify tremors, according to the NIH. Shy-Drager syndrome is a very difficult disease. Poor Johnny Cash.[uaddit.com]
  • I'm scared due to reading that many of my symptoms, which also include diarhea and fatigue, are often linked with the beginning stages of Shy-Drager Syndrome.[healthboards.com]
  • Characteristics Persons who have this syndrome may experience the following symptoms, signs and characteristics: Weakness of the muscles Ache in the neck Constipation Diarrhea Loss of libido Urinary incontinence or Inable to control bladder or bowel Dizziness Fatigue[ehealthwall.com]
  • […] as defined by the American Autonomic Society. [22] Symptoms associated with orthostatic hypotension include the following: Light-headedness Dizziness Dimming of vision Head, neck, or shoulder pain Altered mentation Weakness - Especially of the legs Fatigue[emedicine.medscape.com]
  • Commonly associated symptoms include light-headedness, dizziness, weakness of legs, fatigue and syncope. Postprandial hypotension may be a major feature.[patient.info]
Sleep Apnea
  • A patient with autonomic insufficiency and extrapyramidal signs (Shy-Drager syndrome) and sleep apnea syndrome (SAS) underwent hemodynamic studies.[ncbi.nlm.nih.gov]
  • This diagnosis should be considered in cases of vocal cord palsy of undetermined etiology, especially when associated with increased snoring or episodes of sleep apnea.[ncbi.nlm.nih.gov]
  • A 67-year-old Japanese man suffering from progressive autonomic failure was diagnosed as having Shy-Drager syndrome (SDS) with hyponatremia due to SIADH and severe sleep apnea caused by a bilateral recurrent nerve palsy.[ncbi.nlm.nih.gov]
  • A patient with obstructive sleep apnea due to Shy-Drager syndrome, who has been successfully managed by cord lateralization, is presented.[ncbi.nlm.nih.gov]
  • Patients complain of impotence, change in writing style, slurred speech, sleep apnea, difficulty with urination, frequent urinary tract infections, a hoarse voice, passing out, headache, neck pain, dimming of vision and yawning.[mc.vanderbilt.edu]
Fecal Incontinence
  • Syncope, urinary incontinence, impotence, constipation, fecal incontinence, cardiac arrythmias as well as other symptoms occur as a result of widespread pathological changes in multiple areas of the central and autonomic nervous system.[ncbi.nlm.nih.gov]
  • Therapy with an anal pacemaker and the beta 1 agonist, prenalterol, cured both bladder and fecal incontinence.[ncbi.nlm.nih.gov]
  • 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.[selfdecode.com]
Hypotension
  • Abstract The effect of xamoterol on the orthostatic hypotension associated with Shy-Drager syndrome was investigated in three patients.[ncbi.nlm.nih.gov]
  • Loss of neurons from the intermediolateral columns of the thoracic spinal cord, thought to be the cause of the postural hypotension, was present in all cases.[ncbi.nlm.nih.gov]
  • Their conclusion that orthostatic hypotension and central nervous system dysfunction were probably related is obvious today but at the time seemed prescient.[doi.org]
  • These results suggest that impaired sympathetic neural activity is a key factor in exercise-induced hypotension.[ncbi.nlm.nih.gov]
  • Xamoterol, a cardioselective beta 1-adrenoceptor partial agonist, has been reported to be effective on postural hypotension.[ncbi.nlm.nih.gov]
Orthostatic Hypotension
  • Abstract The effect of xamoterol on the orthostatic hypotension associated with Shy-Drager syndrome was investigated in three patients.[ncbi.nlm.nih.gov]
  • Their conclusion that orthostatic hypotension and central nervous system dysfunction were probably related is obvious today but at the time seemed prescient.[doi.org]
  • Orthostatic hypotension in a patient with Shy-Drager syndrome was treated for 6 months with oral DL-threo-3,4-dihydroxyphenylserine (DL-threo-DOPS).[ncbi.nlm.nih.gov]
  • Treatment of idiopathic orthostatic hypotension is often unsatisfactory. A patient with the Shy-Drager syndrome, in which the most important symptom is orthostatic hypotension, was treated with prenalterol, initially 30 mg six times daily.[ncbi.nlm.nih.gov]
  • We present a case of a 72-year-old man who had a 2-year history of orthostatic hypotension, neurogenic bladder, impotence, anhydrosis, and extremity weakness and paresthesias.[ncbi.nlm.nih.gov]
Decreased Sweating
  • Also remember autonomic symptoms like constipation, sexual impotence, vision disturbances, difficulty breathing and swallowing, sleep disturbances, and decreased sweating.[medicowesome.blogspot.com]
  • Other symptoms include generalized weakness, double vision or other vision disturbances, difficulty breathing and swallowing, sleep disturbances, and decreased sweating. Because the disease resembles others, a correct diagnosis may take years.[my.clevelandclinic.org]
Urinary Incontinence
  • Later on she developed features of autonomic dysfunction including postural hypotension, hyperhydrosis and urinary incontinence so was diagnosed as Shy-Drager Syndrome.[ncbi.nlm.nih.gov]
  • Syncope, urinary incontinence, impotence, constipation, fecal incontinence, cardiac arrythmias as well as other symptoms occur as a result of widespread pathological changes in multiple areas of the central and autonomic nervous system.[ncbi.nlm.nih.gov]
  • Symptoms of autonomic nervous system failure, such as constipation, impotence in men, and urinary incontinence, usually predominate early in the course of the disease.[medicinenet.com]
  • Symptoms of autonomic nervous system failure, such as constipation , impotence in men, and urinary incontinence , usually predominate early in the course of the disease.[medicinenet.com]
  • Imagine a guy who has urinary incontinence (That's why he is shy!). He puts (or parks) his dagger knife (drager) into his brain (Cerebellum, to be precise). So the mnemonic goes: SHy Parked his Drager into his Cerebellum.[medicowesome.blogspot.com]
Tremor
  • The neurological manifestations consisted of a wide-based ataxic gait, bilateral dysmetria with intentional tremor, staccato speech, rigidity, bradykinesia, and an iris-thinning. There was reproducible orthostatic hypotension.[ncbi.nlm.nih.gov]
  • Tremors *Tremors are one of the symptoms of Parkinsonian Shy-Drager syndrome. Stress and fatigue can intensify tremors, according to the NIH. Shy-Drager syndrome is a very difficult disease. Poor Johnny Cash.[uaddit.com]
  • Shy-Drager syndrome leads to dizziness or fainting when standing up, urinary incontinence, impotence, and muscle tremors.[medical-dictionary.thefreedictionary.com]
  • They later developed constipation, urinary and rectal incontinence, slowness of movement, unsteady gait, slurring of speech, mild tremor and other Parkinsonian symptoms.[whonamedit.com]
  • There are three types of Multiple System Atrophy: the cerebellar form, which affects balance, coordination and speech; the parkinsonian form (striatonigral degeneration) which causes stiff muscles, slow movement, and mild tremors; and mixed cerebellar[everything2.com]
Cerebellar Ataxia
  • Probable MSA requires a sporadic, progressive adult-onset disorder including rigorously defined autonomic failure and poorly levodopa-responsive parkinsonism or cerebellar ataxia.[ncbi.nlm.nih.gov]
  • We describe the clinical features of the disease, which include four domains: autonomic failure/urinary dysfunction, parkinsonism and cerebellar ataxia, and corticospinal dysfunction.[ncbi.nlm.nih.gov]
  • ataxia, impotence, visual troubles, tremor and disturbances of sweating, and muscle wasting leading to severe invalidism.[whonamedit.com]
  • “Probable MSA” requires autonomic failure/urinary dysfunction plus poorly responsive parkinsonism or cerebellar ataxia. Pathologic confirmation is required for a definitive diagnosis (3) .[journals.lww.com]
  • “Probable MSA” requires autonomic failure/urinary dysfunction plus poorly responsive parkinsonism or cerebellar ataxia. Pathologic confirmation is required for a definitive diagnosis (3).[journals.lww.com]
Bradykinesia
  • A previously healthy 60 years old female patient presented with bradykinesia, postural instability and increased rigidity of both upper and lower limbs for 2 years and was diagnosed as Parkinsonism for last years.[ncbi.nlm.nih.gov]
  • The neurological manifestations consisted of a wide-based ataxic gait, bilateral dysmetria with intentional tremor, staccato speech, rigidity, bradykinesia, and an iris-thinning. There was reproducible orthostatic hypotension.[ncbi.nlm.nih.gov]
  • 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[ncbi.nlm.nih.gov]
  • For patients afflicted with this condition, rigidity and bradykinesia are the primary extrapyramidal symptoms which are present. The "plus" refers to autonomic nervous system dysfunction which leads to much of the disability seen in this disorder.[ncbi.nlm.nih.gov]
  • Specifically, Parkinson’s features include tremor, bradykinesia and rigid muscles, which lead to impaired posture and balance, as well as speech changes.[casereports.in]
Dysautonomia
  • Shy-Drager Syndrome Synonyms Autonomic Failure, Progressive Autonomic Failures, Progressive Dysautonomia Orthostatic Hypotension Syndrome Dysautonomia-Orthostatic Hypotension Syndrome Dysautonomia-Orthostatic Hypotension Syndromes Dysautonomic Orthostatic[online-medical-dictionary.org]
  • From our own data, cardiovascular dysautonomia emerged as an independent predictor of cognitive impairment.[doi.org]
  • Tjalf Ziemssen and Heinz Reichmann, Review: Treatment of dysautonomia in extrapyramidal disorders, Therapeutic Advances in Neurological Disorders, 10.1177/1756285609348902, 3, 1, (53-67), (2009).[doi.org]
  • Acquired postganglionic cholinergic dysautonomia. Case report and review of the literature. Pediatrics 1982; 70 : 976–978. PubMed Google Scholar 44. Loiudice TA, Buhac I. Diabetic visceral neuropathy: treatment with bethanechol chloride.[link.springer.com]
Extrapyramidal Symptoms
  • For patients afflicted with this condition, rigidity and bradykinesia are the primary extrapyramidal symptoms which are present. The "plus" refers to autonomic nervous system dysfunction which leads to much of the disability seen in this disorder.[ncbi.nlm.nih.gov]
  • In some patients, chronic orthostatic hypotension may be a presenting symptom, but in other cases, extrapyramidal symptoms or cerebellar symptoms may predominate in the early stages.[mc.vanderbilt.edu]

Workup

Iron Increased
  • MR imaging disclosed a prominent putamina hypodensity in T2-weighted images at high field strength due to iron increased depositing in this basal ganglia.[ncbi.nlm.nih.gov]
Hyponatremia
  • A 67-year-old Japanese man suffering from progressive autonomic failure was diagnosed as having Shy-Drager syndrome (SDS) with hyponatremia due to SIADH and severe sleep apnea caused by a bilateral recurrent nerve palsy.[ncbi.nlm.nih.gov]

Treatment

  • Fludrocortisone 0.5 mg a day was added to treatment with further improvement. This clinical effect was maintained throughout 12 months of follow up, during which the treatment was continued unchanged.[ncbi.nlm.nih.gov]
  • After implantation of a port-a-cath system, intravenous noradrenaline treatment could be continued on an outpatient basis.[ncbi.nlm.nih.gov]
  • Average untreated 24-h intra-arterial blood pressure was 132/78 mmHg; during treatment with xamoterol it rose to 138/90 mmHg.[ncbi.nlm.nih.gov]
  • A review of the literature showed that bilateral vocal cord paralysis is a recognized abnormality in this condition for which permanent speaking tracheostomy is the treatment of choice.[ncbi.nlm.nih.gov]
  • Daily administration of this drug resulted in symptomatic improvement of the autonomic functions, and relapse followed discontinuation of treatment.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis of Multiple System Atrophy (Shy Drager Syndrome) Prognosis is poor and on average patients will survive about 6 years after the inital presentation of MSA.[myvmc.com]
  • Prognosis The prognosis is more guarded in the multiple system atrophy patient than in pure autonomic failure. It is rare for a patient to survive 10 years.[mc.vanderbilt.edu]
  • Prognosis For Multiple System Atrophy (MSA) or Shy Drager Syndrome The overall prognosis outlook for Multiple System Atrophy or Shy Drager Syndrome is poor as the disease is progressive impairing mental and physical functions gradually.[epainassist.com]
  • I understand that this is a devastating diagnosis to receive and I am sorry I cannot give you a more optimistic prognosis. Symptoms, however, can often be controlled or made somewhat better.[netdoctor.co.uk]

Etiology

  • She developed signs of progressive autonomic failure suggesting a diagnosis of Shy-Drager syndrome, a rare degenerative neurologic condition of unknown etiology.[ncbi.nlm.nih.gov]
  • This diagnosis should be considered in cases of vocal cord palsy of undetermined etiology, especially when associated with increased snoring or episodes of sleep apnea.[ncbi.nlm.nih.gov]
  • Impairment of the afferent pathways of baroreceptors, or impairment of the osmoreceptors could be speculated as the etiological factor of the SIADH observed in this case.[ncbi.nlm.nih.gov]
  • Its pathogenesis may involve multiple genetic and nongenetic factors, but its etiology remains largely unknown.[ncbi.nlm.nih.gov]
  • Etiology is unknown A rare neuroaxonal dystrophy, histologically characterized by axonal spheroids, iron deposition, lewy body (lb)-like intraneuronal inclusions and neurofibrillary tangles Spastic weakness of the muscles innervated by the cranial nerves[icd9data.com]

Epidemiology

  • For a discussion of epidemiology and pathology, please refer to multiple systemic atrophy (MSA). Clinical presentation The principle clinical finding in Shy-Drager syndrome is arterial orthostatic hypotension.[radiopaedia.org]
  • However, analytical epidemiological studies are hampered because the condition is relatively rare.[ncbi.nlm.nih.gov]
  • Seiten 1-9 Epidemiology Krismer, Florian, MD (et al.) Seiten 11-15 Neuropathology Jellinger, Kurt, MD Seiten 17-56 Aetiopathogenesis Jellinger, Kurt, MD (et al.) Seiten 57-81 Animal Models Stefanova, Nadia, MD, PhD, PD Seiten 83-96[springer.com]
  • Summary Epidemiology Prevalence ranges from 1/50,000-1/20,000. MSA-parkinsonian type (MSA-p) predominates in the Western Hemisphere and MSA-cerebellar type (MSA-c) predominates in the Eastern Hemisphere. Genders are equally distributed.[orpha.net]
Sex distribution
Age distribution

Pathophysiology

  • These findings suggest important pathophysiological roles of cerebellin and CRH in these cerebellar diseases. Such significant decreases were not found in neuropeptide Y and somatostatin.[ncbi.nlm.nih.gov]
  • These findings support the current view that parasympathetic, pudendal and sympathetic involvement is part of the pathophysiology of the Shy-Drager syndrome.[ncbi.nlm.nih.gov]
  • The goal of this paper is to provide an overview of the pathophysiology, signs and symptoms of and treatment for SDS.[ncbi.nlm.nih.gov]
  • Pooling SDS subgroups, therefore, may obscure pathophysiological differences to certain stimuli. Clinically when postural hypotension is being assessed, separation of the subgroups may not be essential, as they responded similarly.[ncbi.nlm.nih.gov]
  • In this review, neuropathological features of MSA are described in detail, along with recent advances in the pathophysiology and genetics of the disease.[ncbi.nlm.nih.gov]

Prevention

  • Postural hypotension: Mechanical maneuvers such as leg-crossing, squatting and bending forward can prevent episodes of postural hypotension as can waering external support garments. Sleeping with the head of the bed elevated.[myvmc.com]
  • Since the cause of why Shy Drager Syndrome exists is still unknown, there are no known ways to prevent the occurrences of this kind of syndrome.[ehealthwall.com]
  • There is no cure for this condition, and there is no known way to prevent the disease from getting worse. The goal of treatment is to control symptoms. Source: Genetic and Rare Diseases Information Center (GARD), supported by ORDR-NCATS and NHGRI.[diseaseinfosearch.org]

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