Horacio Kaufmann, MD
Kaufmann H. Primary Autonomic Failure: Three Clinical Presentations of One Disease?. Ann Intern Med. 2000;133:382-384. doi: 10.7326/0003-4819-133-5-200009050-00014
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Published: Ann Intern Med. 2000;133(5):382-384.
Three neurodegenerative diseases of unknown cause involve primary autonomic failure. These diseases are pure autonomic failure, in which autonomic impairment (that is, orthostatic hypotension and bladder and sexual dysfunction) occurs alone; Parkinson disease, in which autonomic failure is combined with an extrapyramidal movement disorder; and multiple-system atrophy (also called Shy–Drager syndrome), in which autonomic failure is combined with an extrapyramidal or cerebellar movement disorder or both (1).
During the early stages of multiple-system atrophy, autonomic deficits may be the sole clinical manifestation; therefore, the disease may resemble pure autonomic failure. However, after a variable period that can be as long as several years, extrapyramidal or cerebellar deficits or both invariably develop. In Parkinson disease, extrapyramidal motor problems are the presenting feature; later in the disease process, patients may develop severe autonomic failure, making it difficult to distinguish between Parkinson disease and multiple-system atrophy. To further complicate the distinction, some patients with multiple-system atrophy display motor deficits similar to those seen in Parkinson disease before autonomic failure is apparent.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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