Farewell to the “Shy-Drager Syndrome”

A recent consensus statement generated by the American Autonomic Society and the American Academy of Neurology [1] defines the various primary neurogenic causes of autonomic dysfunction. Implicit in this document is the need to bid good-bye to the use of the term “Shy-Drager syndrome” to identify a condition that was first described in 1962 [2].

Milton Shy and Glen Drager detailed the clinical features of two patients who had both orthostatic hypotension and central nervous system signs, and they carefully studied the neuropathologic changes in one of these patients [2]. Their conclusion that orthostatic hypotension and central nervous system dysfunction were probably related is obvious today but at the time seemed prescient. Although some previous reports had described these disparate phenomena in the same patients [3, 4], no one had claimed that the two were related. Shy and Drager described several aspects of autonomic dysfunction—in addition to orthostatic hypotension—and meticulously delineated various signs of degeneration of the cerebellar, extrapyramidal, and pyramidal systems. Since their seminal report, burgeoning interest in autonomic disorders has spawned studies outlining the clinical and neuropathologic findings in such patients [5, 6]. This research resulted in a far greater understanding of the interplay between structural defects and functional outcomes but inevitably added to a confusing array of terms. …