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Advanced Neurology





                                        CASE SERIES
                                        Autonomic storms and autonomic movement

                                        disorder associated with postural orthostatic
                                        tachycardia syndrome misdiagnosed as

                                        functional neurological disorder



                                        Alyssa Khoo * , Jacob Brik 1  , and Anna D. Hohler 1,2
                                                  1
                                        1 Department of Neurology, St. Elizabeth’s Medical Center, Brighton, Massachusetts, United States
                                        of America
                                        2 Department  of  Neurology,  Faculty  of  Neurology  Education,  Boston  University  Chobanian  and
                                        Avedisian School of Medicine, Boston, Massachusetts, United States of America




                                        Abstract
                                        Patients with postural orthostatic tachycardia syndrome (POTS) typically present with
                                        a constellation of symptoms, some of which may mimic other disorders and may lead
                                        to misdiagnosis, including autonomic storms and autonomic movement disorders.
                                        We discuss two cases in which patients with POTS were misdiagnosed as functional
                                        neurological disorder. In both cases, the patients were initially misdiagnosed  on
                                        hospital admission and then referred to neurologists, who correctly diagnosed them
                                        with POTS. We also review video evidence of these patients’ storms to showcase
            *Corresponding author:
            Alyssa Khoo                 the visible presentation of POTS-related myoclonic episodes. Our study highlights
            (ayk9839@nyu.edu)           the challenges of distinguishing POTS from other conditions due to overlapping,
            Citation: Khoo A, Brik J, Hohler AD.   non-specific symptoms. In addition, this case series emphasizes the importance
            Autonomic storms and autonomic   of a thorough clinical assessment, including an orthostatic assessment and tilt
            movement disorder associated with   table testing, to ensure an accurate diagnosis. Misdiagnosis can delay appropriate
            postural orthostatic tachycardia
            syndrome misdiagnosed as    management of POTS, highlighting the need for heightened awareness among
            functional neurological disorder.   clinicians when evaluating autonomic symptoms.
            Adv Neurol. 2025;4(2):123-127.
            doi: 10.36922/an.7653
                                        Keywords: Postural orthostatic tachycardia syndrome; Functional neurological disorder;
            Received: December 16, 2024
                                        Autonomic storms; Myoclonus; Misdiagnosis; Clinical video
            Revised: January 11, 2025
            Accepted: February 11, 2025
            Published online: March 7, 2025  1. Introduction
            Copyright: © 2025 Author(s).
            This is an Open-Access article   Patients diagnosed with postural orthostatic tachycardia syndrome (POTS) typically
            distributed under the terms of the   have  complex  medical  histories.  POTS,  a  syndrome  that  presents  with  rapid  heart
            Creative Commons Attribution   rate on standing, can be spurred by several factors, including, but not limited to,
            License, permitting distribution,
                                                                                           1,2
            and reproduction in any medium,   stress, viral infection, trauma, pregnancy, and immunization.  Autonomic storms
            provided the original work is   associated with POTS typically present with a constellation of symptoms including,
            properly cited.             but not limited to, alterations in levels of consciousness, increased posturing, dystonia,
            Publisher’s Note: AccScience   hypertension, hyperthermia, tachycardia, tachypnea, diaphoresis, agitation, tremor, and
            Publishing remains neutral with   myoclonus. More commonly seen after traumatic brain injuries, these storms may be
            regard to jurisdictional claims in                                          3,4
            published maps and institutional   difficult to diagnose, especially in ambulatory care settings.  In the US, POTS affects
                                                                                                             5
            affiliations.               up to 3,000,000 people, which represent approximately 1% of the nation’s population.

            Volume 4 Issue 2 (2025)                        123                               doi: 10.36922/an.7653
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