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Pure autonomic failure is a progressive, adult-onset, degenerative disorder of the autonomic nervous system characterized clinically by orthostatic hypotension, and bladder and sexual dysfunction with no other associated neurologic deficits1. Pure autonomic failure is one of three disorders classified as primary autonomic failure, the other two being PD and multiple system atrophy.1 Alpha-synuclein, a 140-amino-acid protein, accumulates in cytoplasmic inclusions in affected brain areas of patients with PD2 and with multiple system atrophy.3 Here, we show that alpha-synuclein accumulates in cytoplasmic inclusions in brainstem nuclei and in pre- and postganglionic autonomic neurons of a patient with pure autonomic failure.
Case history.
The patient was a 63-year-old man with a 15-year history of severe orthostatic hypotension with recurrent syncope, urinary incontinence, erectile failure, and decreased sweating with heat intolerance. No other neurologic deficits were present. Blood pressure dropped markedly on standing, without increase in heart rate. Sympathetic failure was substantiated by low levels of …
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