Presentation
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. [online-medical-dictionary.org]
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]
He presented to the emergency department with right lower extremity swelling, erythema and pain. [casereports.in]
Entire Body System
- 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]
The life expectancy with persons having Shy Drager Syndrome falls from the time and day she was diagnosed is from 7 up to 9 years. [ehealthwall.com]
- 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]
Chronic Fatigue Syndrome Support Group. Chronic Fatigue Syndrome (ME/CFS) describes a sense of exhaustion and post-exertion malaise, even when you have gotten enough rest and sleep. CFS and neuropathy. WhiteDolphin 11/01/2008. [ixmtgt.co]
Patients often have blurred vision and fatigue. Patients often see erectile syncope. Due to extensive degeneration of autonomic nerves, baroreflex arcs are blocked. [en.ybsite.org]
Some of the symptoms reported with MSA include: fatigue weakness blurred vision problems with bowel or bladder control incontinence erectile dysfunction constipation dry skin Eventually, most people with this condition progress to some degree of autonomic [healthline.com]
- Surgical Procedure
procedures and single-room maternity care. 2000 Brookside Drive Kingsport, TN 37660 (423) 857-7000 Johnson County Community Hospital Mountain City, TN Johnson County Community Hospital offers inpatient care and a 24-hour ER. [wellmont.org]
Respiratoric
- 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]
For patients with sleep irregularities, particularly if they involve interrupted breathing or snoring, physicians may recommend an evaluation in a sleep laboratory to determine if there is an underlying and treatable sleep disorder, such as sleep apnea [mc.vanderbilt.edu]
- Pneumonia
Patient developed aspiration pneumonia during oral feeding, despite of ICU management for the same cause patient died of respiratory arrest. [ncbi.nlm.nih.gov]
The most common causes of death are pneumonia (due to swallowing difficulties) and pulmonary embolus. Sources: www.ninds.nih.gov www.ahealthyme.com health.yahoo.com [everything2.com]
Pneumonia is the most common cause of death, although irregularities in heart beat or choking may be responsible for death in some patients. [multiplesystematrophy.org]
"People with MSA often develop pneumonia in the later stages of the disease and may suddenly die from cardiac or respiratory issues." [universityherald.com]
The autonomic abnormalities are seldom the direct cause of death A significant number of patients develop laryngeal stridor and difficulty swallowing, which can lead to pneumonia. [mc.vanderbilt.edu]
- Dyspnea
Hoarseness and dyspnea with stridor developed as a result of bilateral vocal cord paralysis in the median position and required an emergency tracheotomy. This combination of symptoms resulted in the diagnosis of Shy-Drager syndrome. [ncbi.nlm.nih.gov]
Keywords : Shy-Drager Syndrome, Parkinson Disease, Thrombosis, Embolism, Dyspnea, Chest Pain. [casereports.in]
Gastrointestinal
- Fecal Incontinence
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. [online-medical-dictionary.org]
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]
- Choking
Pneumonia is the most common cause of death, although irregularities in heart beat or choking may be responsible for death in some patients. [multiplesystematrophy.org]
“He wants so much to do that, and I can’t help him,” she said, choking up. “He don’t know that.” Jeffery receives support from a woman in California whose husband died from the disease and called tell her what to expect with her husband’s sickness. [mlive.com]
Cardiovascular
- Hypotension
Hypotension Dysautonomic Orthostatic Hypotensions Failure, Progressive Autonomic Failures, Progressive Autonomic Hypotension Syndrome, Dysautonomia-Orthostatic Hypotension Syndromes, Dysautonomia-Orthostatic Hypotension, Dysautonomic Orthostatic Hypotensions [online-medical-dictionary.org]
The disorder is characterized by postural (or orthostatic) hypotension (an excessive drop in blood pressure when the patient stands up), which causes dizziness or momentary blackouts. [alzheimer-europe.org]
S: System atrophy (Multiple system atrophy) H: Hypotension Y: Why is he shy? Urinary involvement (urgency/incontinence). [medicowesome.com]
Orthostatic Hypotensions — Hypotension, Dysautonomic Orthostatic — Hypotensions, Dysautonomic Orthostatic — Orthostatic Hypotensions, Dysautonomic — Dysautonomia-Orthostatic Hypotension Syndrome — Dysautonomia Orthostatic Hypotension Syndrome — Dysautonomia-Orthostatic [mesh.kib.ki.se]
- Orthostatic Hypotension
Hypotension Dysautonomic Orthostatic Hypotensions Failure, Progressive Autonomic Failures, Progressive Autonomic Hypotension Syndrome, Dysautonomia-Orthostatic Hypotension Syndromes, Dysautonomia-Orthostatic Hypotension, Dysautonomic Orthostatic Hypotensions [online-medical-dictionary.org]
The disorder is characterized by postural (or orthostatic) hypotension (an excessive drop in blood pressure when the patient stands up), which causes dizziness or momentary blackouts. [alzheimer-europe.org]
Orthostatic Hypotensions — Hypotension, Dysautonomic Orthostatic — Hypotensions, Dysautonomic Orthostatic — Orthostatic Hypotensions, Dysautonomic — Dysautonomia-Orthostatic Hypotension Syndrome — Dysautonomia Orthostatic Hypotension Syndrome — Dysautonomia-Orthostatic [mesh.kib.ki.se]
Code Applicable To Hypotension, postural Type 1 Excludes neurogenic orthostatic hypotension [Shy-Drager] ( G90.3 ) orthostatic hypotension due to drugs ( I95.2 ) neurogenic G90.3 neurogenic, orthostatic G90.3 Parkinsonism (idiopathic) (primary) G20 ICD [icd10data.com]
Musculoskeletal
- Neck Pain
Pain Comfort Cool Tennis Elbow Braces - Elbow Wraps, Sleeves & Bands Finger - Thumb Supports Foot and Ankle Braces - Supports Foot Pain & Injury Hernia Aids and Support Hip Surgery and Injury Knee Braces & Support Orthopedic Back Cushions Orthopedic [activeforever.com]
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]
- Muscle Twitch
twitches Cognitive impairment One study found a correlation between the deletion of genes in a specific genetic region and the development of MSA in a group of Japanese patients. [en.wikipedia.org]
twitches[12] Cognitive impairment[13] Genetics One study found a correlation between the deletion of genes in a specific genetic region and the development of MSA in a group of Japanese patients. [pspawareness.com]
Skin
- Decreased Sweating
Also remember autonomic symptoms like constipation, sexual impotence, vision disturbances, difficulty breathing and swallowing, sleep disturbances, and decreased sweating. [medicowesome.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]
- Dry Skin
On examination they had very low blood pressure when standing, and in addition to some Parkinsonian findings they also had wasting of muscles in hands and feet, dry skin due to loss of sweating, and loss of pigment in the iris of the eyes. [whonamedit.com]
Upon examination, they had very low blood pressure when standing, and in addition to some Parkinsonian findings, they also had wasting of muscles in their hands and feet, dry skin due to loss of sweating and loss of pigment in the iris of the eyes. [multiplesystematrophy.org]
Many patients also complain of dry mouth and dry skin, and because of abnormal sweating have problems with relation of body temperature. The hands and feet will be cold. [bionicinterface.com]
Some of the symptoms reported with MSA include: fatigue weakness blurred vision problems with bowel or bladder control incontinence erectile dysfunction constipation dry skin Eventually, most people with this condition progress to some degree of autonomic [healthline.com]
Neurologic
- Tremor
Parked: Symptoms of Parkinson's disease such as slow movement, stiff muscles, and tremor. Cerebellum: Cerebellar problems like coordination and speech. That's all! -IkaN [medicowesome.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]
Kinetic tremor is rhythmic, involuntary muscular contraction that occurs during purposeful movement, such as touching your finger to your nose. [healthcommunities.com]
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]
- 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]
Furthermore, 3 patients had cerebellar ataxia as an initial and principal symptom. [doi.org]
ataxia, impotence, visual troubles, tremor and disturbances of sweating, and muscle wasting leading to severe invalidism. [whonamedit.com]
- Bradykinesia
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]
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]
Hypotension Syndromes — Hypotension Syndrome, Dysautonomia-Orthostatic — Hypotension Syndromes, Dysautonomia-Orthostatic — Syndromes, Dysautonomia-Orthostatic Hypotension — Autonomic Failure, Progressive — Autonomic Failures, Progressive — Failure, Progressive [mesh.kib.ki.se]
Orthostatic Hypotension Syndrome Dysautonomia-Orthostatic Hypotension Syndrome Dysautonomia-Orthostatic Hypotension Syndromes Dysautonomic Orthostatic Hypotension Dysautonomic Orthostatic Hypotensions Failure, Progressive Autonomic Failures, Progressive [finto.fi]
Language: MeSH term: Accepted terms: English: Shy-Drager Syndrome - Autonomic Failure, Progressive - Idiopathic Orthostatic Hypotension, Shy-Drager Type - Orthostatic Hypotension, Dysautonomic - Dysautonomia-Orthostatic Hypotension Syndrome - Syndrome [hon.ch]
シソーラス検索結果 同義語(異表記): Dysautonomia-Orthostatic Hypotension Syndrome Dysautonomic Orthostatic Hypotension Progressive Autonomic Failure Shy-Drager Syndrome Shy-Drager Type Idiopathic Orthostatic Hypotension Shy-Drager症候群 シャイ・ドレーガー症候群 シャイ・ドレージャー症候群 シャイ・ドレイガー [lsd-project.jp]
- Slurred Speech
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]
slow or low-volume speech (dysarthria) Visual disturbances, such as blurred or double vision and difficulty focusing your eyes Difficulty swallowing (dysphagia) or chewing Changes in speech, such as slurred speech General signs and symptoms In addition [mayoclinic.org]
Cerebellar symptoms, which impact about 20% of people with MSA, include things like: uncoordinated voluntary movements progressive balance loss slurred speech jerkiness to eye movements Other symptoms that can appear in either type of MSA include: exaggerated [healthline.com]
[…] 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 Dry skin Impotence Impairment of the temperature regulatory system Visual problems Slurred [ehealthwall.com]
Urogenital
- Incontinence
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. [online-medical-dictionary.org]
Urinary involvement (urgency/incontinence). Also remember autonomic symptoms like constipation, sexual impotence, vision disturbances, difficulty breathing and swallowing, sleep disturbances, and decreased sweating. [medicowesome.com]
Urinary incontinence: Catheterisation can help to prevent incontinence If the urinary incontinence is due to detrusor, muscle instability anticholinergic agents can be helpful. Impotence: Treated with medications or injections. [myvmc.com]
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]
- Urinary Incontinence
Urinary involvement (urgency/incontinence). Also remember autonomic symptoms like constipation, sexual impotence, vision disturbances, difficulty breathing and swallowing, sleep disturbances, and decreased sweating. [medicowesome.com]
Urinary incontinence: Catheterisation can help to prevent incontinence If the urinary incontinence is due to detrusor, muscle instability anticholinergic agents can be helpful. Impotence: Treated with medications or injections. [myvmc.com]
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]
- Sexual Dysfunction
© 2002 Movement Disorder Society Citing Literature Number of times cited according to CrossRef: 28 Waleed Altaweel and Raouf Seyam, Sexual Dysfunction and Fertility in Neurogenic Lower Urinary Tract Dysfunction, Neurourology, 10.1007/978-94-017-7509- [doi.org]
The most frequent symptoms are related to orthostatic hypotension, impaired gastrointestinal motility, and urinary and sexual dysfunction. [pubmed.ncbi.nlm.nih.gov]
Sexual dysfunction, urinary incontinence, fecal incontinence, dysphagia, unequal pupils, atrophy of the iris, external ophthalmoplegia, and wasting of distal limb muscles all reflect the degenerative changes within the central nervous system and autonomic [accessanesthesiology.mhmedical.com]
[…] disorders Agitated sleep due to "acting out" dreams Abnormal breathing at night or a harsh breathing sound (stridor) Sexual dysfunction Inability to achieve or maintain an erection (impotence) Loss of libido Cardiovascular problems Color changes in hands [mayoclinic.org]
dysfunction irregular heartbeats trouble with emotional regulation. [news-medical.net]
Workup
(See Workup.) Table 4. [emedicine.com]
Clinical Testing and Workup Specialized tests that can aid in the diagnosis of MSA include: Magnetic resonance imaging (MRI), which may demonstrate changes in certain brain areas. [rarediseases.org]
Treatment
Care and treatment Orthostatic hypotension in Shy-Drager syndrome is treatable, but there is not known effective treatment for the progression central nervous system degeneration. The general treatment course is aimed at controlling symptoms. [alzheimer-europe.org]
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]
Indomethacin and propranolol combined treatment for Shy-Drager syndrome [14]. [wikigenes.org]
Prognosis
The prognosis of Shy-Drager syndrome is very poor; patients are markedly disabled and have shorter survival. [ncbi.nlm.nih.gov]
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]
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]
Etiology Etiology of MSA is unknown but presence of cytoplasmic aggregates of α-synuclein, primarily in oligodendroglia, in combination with neurodegeneration in striatonigral and olivopontocerebellar structures are the pathological hallmark features. [orpha.net]
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]
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]
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]
This measure helps prevent the bladder from stretching and urinary tract infections from developing. Washing the hands, cleansing the area around the urethra, and using a sterile or clean catheter also help prevent infections. [msdmanuals.com]