Shy-Drager syndrome: recognition and management.
نویسنده
چکیده
Shy-Drager syndrome is an uncommon disorder in which the patient experiences progressive autonomic failure. It is a multisystem degenerative disease that involves the central autonomic, cerebellar, basal ganglia, pyramidal, or spinal motor neurons. The mean age of onset is 55 years. A male predominance is noted, with a 2 or 3:1 ratio. About 11 percent of patients with orthostatic hypotension have Shy-Drager syndrome. The disease is progressive, and patients die 7 to 20 years after the onset of neurologic symptoms. The syndrome was first noted in 1925, when Bradbury and Eggleston 1 published a paper on 3 patients with postural hypotension and unchanging pulse rate. Shy and Drager2 provided the first comprehensive clinical and pathologic details of central nervous system involvement in this disorder. We report 1 patient with cholinergic dysfunction and orthostatic hypotension. The diagnosis of this rare neurological disease in our patient was made through a work-up in a geriatric assessment unit.
منابع مشابه
A case of Shy, Drager Syndrome
SUMMARY Shy, Drager syndrome is multi system degeneration of the central nervous system along with pandysautonomia. It begins after age 50 and affects men twice as women. The cardinal manifestation are due to basal ganglia and orthostatic hypotension. In spite of extrapyramidal involvement the response is poor to conventional anti-Parkinsonian medications and if this is the case in a middle ag...
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A 46-year-old woman with Shy-Drager syndrome is presented. She has impaired vocal fold abduction during sleep, but has no laryngeal dysfunction while she is awake. In order to reduce laryngeal obstruction during sleep, she initially underwent laterofixation of 1 vocal fold (Ejnell's method) with little lasting success because of accidental slipping of the ligature. Later, she successively under...
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عنوان ژورنال:
- The Journal of the American Board of Family Practice
دوره 8 4 شماره
صفحات -
تاریخ انتشار 1995