Characteristics and outcomes of youth with functional seizures attending intensive interdisciplinary pain treatment.

Functional seizures (FS) can be debilitating and negatively impact quality of life. Yet intervention research for FS is limited, especially for youth. This study examined clinical characteristics and outcomes of youth with FS (13-23 years) presenting to a pediatric intensive interdisciplinary pain treatment (IIPT) program in the midwestern United States. Sixty youth (mean age = 16.5 years; 83.3 % female) met inclusion criteria. At intake, comorbid chronic pain, somatic symptoms, autonomic dysfunction, eating and weight disturbances, and mental health concerns were common. Despite this high symptom burden, youth with FS reported significant improvements in functioning measured with the Functional Disability Inventory, t(53) = 9.80, p <.001, d = 1.32; depression measured with the Center for Epidemiological Studies - Depression Scale for Children, t(53) = 6.76, p <.001, d = 0.91; anxiety measured with the Spence Children's Anxiety Scale, t(53) = 3.97, p < .001, d = 0.53; and catastrophizing measured with the Pain Catastrophizing Scale for Children, t(53) = 6.44, p <.001, d = 0.86, following completion of the program, suggesting that IIPT may be an effective treatment option for highly disabled and emotionally distressed youth with FS. Future research is needed to continue to refine best practices for youth with FS to reduce suffering and improve outcomes.

Bridging the gap in BASCULE syndrome: A retrospective case series of a recently described clinical entity.

BACKGROUND: Bier anemic spots, cyanosis with urticaria-like eruption (BASCULE) syndrome is a recently described entity with episodic urticarial lesions and white anemic halos on a background of erythrocyanosis, commonly affecting the lower extremities. Possible association with autonomic dysfunction remains poorly understood. Existing publications are limited, but the condition is suggested as highly underrecognized. OBJECTIVE: To further characterize clinical and epidemiologic data for BASCULE syndrome. METHODS: We performed an IRB-approved retrospective chart review on patients with BASCULE syndrome evaluated at Mayo Clinic from April 2021 to November 2022. RESULTS: A total of 17 patients were identified (13 female, 4 male). Median age of onset was 12 years (range 9-17). Lower extremities were involved in all patients (17). Most patients were symptomatic with pruritus (8) or burning pain (8); three were asymptomatic. Triggers were standing (11), hot showers or hot environments (7), or no clear trigger (4). Autonomic dysfunction was present in 10 patients. Treatment responses were observed from propranolol (3) and high-dose cetirizine (1). CONCLUSION: Novel epidemiologic data from 17 pediatric and young adult patients with BASCULE syndrome further supports an association with autonomic dysfunction and suggests a higher prevalence than previously acknowledged.

Assessment of comorbid symptoms in pediatric autonomic dysfunction.

PURPOSE: Pediatric patients with autonomic dysfunction and orthostatic intolerance (OI) often present with co-existing symptoms and signs that might or might not directly relate to the autonomic nervous system. Our objective was to identify validated screening instruments to characterize these comorbidities and their impact on youth functioning. METHODS: The Pediatric Assembly of the American Autonomic Society reviewed the current state of practice for identifying symptom comorbidities in youth with OI. The assembly includes physicians, physician-scientists, scientists, advanced practice providers, psychologists, and a statistician with expertise in pediatric disorders of OI. A total of 26 representatives from the various specialties engaged in iterative meetings to: (1) identify and then develop consensus on the symptoms to be assessed, (2) establish committees to review the literature for screening measures by member expertise, and (3) delineate the specific criteria for systematically evaluating the measures and for making measure recommendations by symptom domains. RESULTS: We review the measures evaluated and recommend one measure per system/concern so that assessment results from unrelated clinical centers are comparable. We have created a repository to apprise investigators of validated, vetted assessment tools to enhance comparisons across cohorts of youth with autonomic dysfunction and OI. CONCLUSION: This effort can facilitate collaboration among clinical settings to advance the science and clinical treatment of these youth. This effort is essential to improving management of these vulnerable patients as well as to comparing research findings from different centers.

Creating a data dictionary for pediatric autonomic disorders.

PURPOSE: Whether evaluating patients clinically, documenting care in the electronic health record, performing research, or communicating with administrative agencies, the use of a common set of terms and definitions is vital to ensure appropriate use of language. At a 2017 meeting of the Pediatric Section of the American Autonomic Society, it was determined that an autonomic data dictionary comprising aspects of evaluation and management of pediatric patients with autonomic disorders would be an important resource for multiple stakeholders. METHODS: Our group created the list of terms for the dictionary. Definitions were prioritized to be obtained from established sources with which to harmonize. Some definitions needed mild modification from original sources. The next tier of sources included published consensus statements, followed by Internet sources. In the absence of appropriate sources, we created a definition. RESULTS: A total of 589 terms were listed and defined in the dictionary. Terms were organized by Signs/Symptoms, Triggers, Co-morbid Disorders, Family History, Medications, Medical Devices, Physical Examination Findings, Testing, and Diagnoses. CONCLUSION: Creation of this data dictionary becomes the foundation of future clinical care and investigative research in pediatric autonomic disorders, and can be used as a building block for a subsequent adult autonomic data dictionary.

Excessive Postural Tachycardia and Postural Orthostatic Tachycardia Syndrome in Youth: Associations With Distress, Impairment, Health Behaviors, and Medication Recommendations.

Among adolescents with fatigue and postural dizziness, it is unclear how health behaviors and emotional distress relate to the presence of excessive postural tachycardia. We prospectively evaluated adolescents aged 13-22 years presenting with symptoms suggestive of autonomic dysfunction between September 2017 and December 2018. Patients underwent standard 10-minute, 70-degree head-up tilt testing. Clinician diagnoses and recommendations were recorded from the medical record. Patients completed validated self-report measures of lifestyle factors, autonomic symptoms, depression, anxiety, and functional disability. Of 179 patients, 58 were diagnosed with postural orthostatic tachycardia syndrome and 59 had excessive postural tachycardia, with 90.5% concordance between the 2 groups. Presence of excessive postural tachycardia was associated with greater baseline fluid intake and likelihood of medication prescription in their treatment plan. Medication findings were replicated for postural orthostatic tachycardia syndrome diagnosis. Presence of excessive postural tachycardia or postural orthostatic tachycardia syndrome did not differentiate patients on perceived symptom severity, emotional distress, disability, or health behaviors but did appear to determine treatment recommendations.

Treatment of Postural Orthostatic Tachycardia Syndrome With Medication: A Systematic Review.

PURPOSE: Postural orthostatic tachycardia syndrome has been recognized for decades, but treatment is largely based on anecdotal experience and expert opinion. Pharmacologic treatment is inconsistent and unstandardized. We did a systematic review to identify controlled studies from which informed treatment decisions can be made. METHOD: Through a standard systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we identified all English-language studies of a medication treatment for postural orthostatic tachycardia syndrome that included a comparison or control group and followed outcomes for at least 1 week of treatment. RESULTS: A total of 626 studies were identified by the search criteria, and 8, involving a total of 499 patients, met the criteria. No studies were adequately similar to allow for meta-analysis. Of the identified 8 studies, 2 were randomized controlled trials and 4 had been subjected to peer review. In individual studies, there was some favorable effect with fludrocortisone, beta blockers, midodrine, and selective serotonin reuptake inhibitors. CONCLUSION: There is a paucity of high-quality data about effectiveness of medication in the treatment of postural orthostatic tachycardia syndrome. Nonetheless, 2 randomized trials and 6 other reports show some favorable effects of medication.

When patients are harmed, but are not wronged: ethics, law, and history.

Iatrogenic injury-injury caused unintentionally by medical treatment-breaks the oldest and most famous rule of medical ethics: primum non nocere, or above all, do no harm. Medical malpractice law, however, focuses on whether an injury was caused by negligence, not on whether an injury was iatrogenic. Iatrogenic injury inflicted without negligence is a common pattern in medical malpractice lawsuits; it is likely the pattern of Jacobs v Cross (Minnesota, 1872), in which Dr W. W. Mayo testified as an expert witness. As a matter of law, the doctor defendants should win all those lawsuits, for iatrogenic injury inflicted without negligence is not a legal wrong in the United States and has not been considered a legal wrong for hundreds of years. However, the medical ethics applicable to doctors' duties to report incompetence in colleagues, including those who inflict excessive iatrogenic injury, have developed dramatically over time. In 1872, the ethical codes in the United States exhorted doctors not to criticize another doctor, even if incompetent. Today, doctors in the United States are ethically required to report an incompetent colleague.