Pure autonomic failure

In this issue of Neurology , Kaufmann et al.1 describe two patients who presented with isolated autonomic failure; years later, one developed features of Parkinson disease and the other features of dementia with Lewy bodies. Pure autonomic failure is now the preferred name for the disorder characterized by isolated peripheral autonomic failure.2 This disorder was previously called progressive autonomic failure, idiopathic orthostatic hypotension, and the Bradbury-Eggleston syndrome. Pure autonomic failure is characterized by gradually progressive autonomic dysfunction with prominent orthostatic hypotension in the absence of cognitive, motor, or sensory impairment.3 Bradbury and Eggleston comprehensively described three patients with an “extensive and peculiar disturbance in the functional activity of the vegetative nervous system.”4 These patients all had incapacitating postural hypotension with cardiovascular, gastrointestinal, urogenital, thermoregulatory, sudomotor, and pupillomotor dysfunction with “signs of slight and indefinite changes in the nervous system.” Their insightful description remains the paradigm for the disorder, characterized by isolated autonomic dysfunction without signs of central or peripheral nervous system disease, now known as pure autonomic failure. But cases such as …