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  • Title: [A case report of Shy-Drager disease].
    Author: Stepień A, Maksymiuk G.
    Journal: Neurol Neurochir Pol; 1997; 31(2):335-41. PubMed ID: 9380263.
    Abstract:
    Shy and Drager in 1960 first described a disease of adult onset characterized by the progressive development of autonomic deficits. The most frequent symptoms are related to orthostatic hypotension, impaired gastrointestinal motility, and urinary and sexual dysfunction. Recently the syndrome has been linked to other degenerative disorders like striatonigral degeneration and olivopontocerebellar atrophy. This combination of degenerative diseases has come to be known as multiple system atrophy (MSA). Shy-Drager disease is most often met in 5 and 6 decade in men and women equally. Apart from autonomic dysfunction symptoms we can find a combination of signs connected with lesions localized in different regions of brain and cord. However, hypertonic-akinetic syndrome is the most often met in patients as an additional symptom. According to this to make a differential diagnosis is often difficult and possible only after autopsy. We describe the case of 57 years old male patient who was admitted to our Clinic because of episodes of orthostatic hypotension, urinary incontinence, sexual impotence; and parkinsonism. In MRI there were changes described as degenerative localized in different parts of brain an cord. Treatment of hypertonic-akinetic syndrome with levodopa/carbidopa and agonists of dopamine receptors increased the frequency of hypotonic episodes. High level of sensitivity to antiparkinsonian drugs is well known feature found in patient with Shy-Drager disease.
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