The neuromuscular evaluation of postural orthostatic tachycardia syndrome

Postural orthostatic tachycardia syndrome (POTS) is a diagnosis with a wide spectrum of symptomatology and a variety of clinicopathological associations, including hereditary, autoimmune, and infectious associations. There is little consensus regarding the appropriate diagnostic approach to the condition. The aim of this work was to provide guidance on the initial diagnosis and when and how to expand diagnostic testing. We define the "typical" POTS patient as younger, female, and with a normal examination apart from joint hyperextensibility. Red flags for "atypical" POTS would be older age at onset, male, prominent syncope, review of systems suggestive of specific alternative diagnoses, examination abnormalities other than joint hyperextensibility, or disease refractory to nonpharmacological and other first-line treatments. Although a limited evaluation is appropriate in POTS with typical features, we recommend an expanded and individualized workup in atypical cases, including additional cardiac testing, autonomic testing, neuropathy workup, and/or autoimmune workup (including consideration of Guillain-Barré syndrome), depending on clinical presentation. We emphasize the importance of shared decision-making in this condition for which treatment remains primarily symptomatic regardless of etiology.