Cases reported "Shy-Drager Syndrome"

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1/4. vocal cord paralysis as a presenting sign in the shy-drager syndrome.

    A 70-year-old patient with bilateral vocal cord paralysis, accompanied by inspiratory stridor and increased snoring, is presented. Respiratory dysfunction, deteriorating over 18 months, necessitated permanent tracheostomy. Only two and a half years after the onset of symptoms the patient developed extrapyramidal signs combined with severe autonomic failure, suggesting a diagnosis of shy-drager syndrome. Vocal cord palsy preceding any other neurological or autonomic manifestations of that syndrome has been infrequently described. 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.
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ranking = 1
keywords = palsy
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2/4. Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and Gerhardt syndrome associated with shy-drager syndrome.

    This is the first report on a case of syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with Gerhardt syndrome (paralysis of bilateral vocal cords). 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. water load test showed alteration in diuresis which was corrected by phenytoin. arginine vasopressin secretion was not suppressed by plasma osmolality below 280 mOsm/kgH2O. 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.
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ranking = 0.5
keywords = palsy
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3/4. Abductor vocal fold palsy in the shy-drager syndrome presenting with snoring and sleep apnoea.

    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. This case emphasizes the need for thorough investigation of all patients with sleep-related breathing disorders with video and sound recordings prior to anaesthesia and surgery.
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ranking = 2.5
keywords = palsy
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4/4. sleep-related breathing disorders in patients with multiple system atrophy and vocal fold palsy.

    We performed sleep studies in eight patients with multiple system atrophy (MSA) and three patients with peripheral bilateral vocal fold palsy (PBVFP) and investigated stenosis of the upper airway tract during sleep in MSA patients with vocal fold palsy. Among the eight MSA patients in this study, five had definite glottic snoring and two others were suspected of having glottic snoring. Of the PBVFP patients, two had glottic snoring. Three of 11 patients died, and two of the three deaths occurred during sleep. Glottic snoring indicated a high degree of negative esophageal pressure. High negative esophageal pressure demonstrates severe narrowing of the upper airway tract. Therefore, glottic snoring should be considered a risk factor for sudden death in sleep. Repeated laryngoscopic examination is useful in evaluating the progressive process of vocal fold palsy while awake, but this examination performed only while awake is not enough to evaluate narrowing of the upper airway during sleep. sleep studies that include the measurement of esophageal pressure can be very useful in evaluating the severity of narrowing in the upper airway tract. It is suspected that sudden nocturnal death in MSA patients is caused not only by abnormal respiration resulting from impairment of the respiratory center, but also by glottic obstruction caused by sputum or by edema of the vocal folds. We recommend treatment of respiratory disorders when loud laryngeal snoring occurs in patients with MSA, even if they do not complain of dyspnea while awake.
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ranking = 3.5
keywords = palsy
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