Pediatric Acute-Onset Neuropsychiatric Syndrome: A Data Mining Approach to a Very Specific Constellation of Clinical Variables
- Antonella Gagliano
- , , MD, PhD
- Cecilia Galati
- , , MD
- Massimo Ingrassia
- , , BS
- Massimo Ciuffo
- , , BS
- Maria Ausilia Alquino
- , , BS
- Marcello G. Tanca
- , , BS
- Sara Carucci
- , , MD, PhD
- Alessandro Zuddas
- , , and MD
- Enzo Grossi
- , MD
Published Online: 7 Oct 2020Doi: https://doi.org/10.1089/cap.2019.0165
Abstract
Objectives: Pediatric acute onset neuropsychiatric syndrome (PANS) is a clinically heterogeneous disorder presenting with: unusually abrupt onset of obsessive compulsive disorder (OCD) or severe eating restrictions, with at least two concomitant cognitive, behavioral, or affective symptoms such as anxiety, obsessive-compulsive behavior, and irritability/depression. This study describes the clinical and laboratory variables of 39 children (13 female and 26 male) with a mean age at recruitment of 8.6 years (standard deviation 3.1).
Methods: Using a mathematical approach based on Artificial Neural Networks, the putative associations between PANS working criteria, as defined at the NIH in July 2010 (Swedo et al. 2012), were explored by the Auto Contractive Map (Auto-CM) system, a mapping method able to compute the multidimensional association of strength of each variable with all other variables in predefined dataset.
Results: The PANS symptoms were strictly linked to one another on the semantic connectivity map, shaping a central “diamond” encompassing anxiety, irritability/oppositional defiant disorder symptoms, obsessive-compulsive symptoms, behavioral regression, sensory motor abnormalities, school performance deterioration, sleep disturbances, and emotional lability/depression. The semantic connectivity map also showed the aggregation between PANS symptoms and laboratory and clinical variables. In particular, the emotional lability/depression resulted as a highly connected hub linked to autoimmune disease in pregnancy, allergic and atopic disorders, and low Natural Killer percentage. Also anxiety symptoms were shown to be strongly related with recurrent infectious disease remarking the possible role of infections as a risk factor for PANS.
Conclusion: Our data mining approach shows a very specific constellation of symptoms having strong links to laboratory and clinical variables consistent with PANS feature.
- Antonella Gagliano
- , , MD, PhD
- Cecilia Galati
- , , MD
- Massimo Ingrassia
- , , BS
- Massimo Ciuffo
- , , BS
- Maria Ausilia Alquino
- , , BS
- Marcello G. Tanca
- Sara Carucci
- , , MD, PhD
- Alessandro Zuddas
- , , and MD
- Enzo Grossi
- , MD
Abstract
Objectives: Pediatric acute onset neuropsychiatric syndrome (PANS) is a clinically heterogeneous disorder presenting with: unusually abrupt onset of obsessive compulsive disorder (OCD) or severe eating restrictions, with at least two concomitant cognitive, behavioral, or affective symptoms such as anxiety, obsessive-compulsive behavior, and irritability/depression. This study describes the clinical and laboratory variables of 39 children (13 female and 26 male) with a mean age at recruitment of 8.6 years (standard deviation 3.1).
Methods: Using a mathematical approach based on Artificial Neural Networks, the putative associations between PANS working criteria, as defined at the NIH in July 2010 (Swedo et al. 2012), were explored by the Auto Contractive Map (Auto-CM) system, a mapping method able to compute the multidimensional association of strength of each variable with all other variables in predefined dataset.
Results: The PANS symptoms were strictly linked to one another on the semantic connectivity map, shaping a central “diamond” encompassing anxiety, irritability/oppositional defiant disorder symptoms, obsessive-compulsive symptoms, behavioral regression, sensory motor abnormalities, school performance deterioration, sleep disturbances, and emotional lability/depression. The semantic connectivity map also showed the aggregation between PANS symptoms and laboratory and clinical variables. In particular, the emotional lability/depression resulted as a highly connected hub linked to autoimmune disease in pregnancy, allergic and atopic disorders, and low Natural Killer percentage. Also anxiety symptoms were shown to be strongly related with recurrent infectious disease remarking the possible role of infections as a risk factor for PANS.
Conclusion: Our data mining approach shows a very specific constellation of symptoms having strong links to laboratory and clinical variables consistent with PANS feature.
Revised Treatment Guidelines Released for Pediatric Acute Onset Neuropsychiatric Syndrome (PANS/PANDAS) -JCAP 2017
The special issue on PANS/PANDAS treatment guidelines was led by Guest Editors Jennifer Frankovich, MD, Stanford University, Susan Swedo, MD, NIH, and Tanya Murphy, MD, University of South Florida .
Treatment of PANS depends on three complementary modes of intervention:
1. Treating the symptoms with psychoactive medications, psychotherapies (particularly cognitive behavioral therapy), and supportive interventions.
2. Removing the source of the inflammation with antimicrobial interventions.
3. Treating disturbances of the immune system with immunomodulatory and/or anti-inflammatory therapies.
Clinical Management of Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS)
Part I—Psychiatric and Behavioral Interventions
Part II—Use of Immunomodulatory Therapies
Part III—Treatment and Prevention of Infections
Treatment of PANS depends on three complementary modes of intervention:
1. Treating the symptoms with psychoactive medications, psychotherapies (particularly cognitive behavioral therapy), and supportive interventions.
2. Removing the source of the inflammation with antimicrobial interventions.
3. Treating disturbances of the immune system with immunomodulatory and/or anti-inflammatory therapies.
Clinical Management of Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS)
Part I—Psychiatric and Behavioral Interventions
Part II—Use of Immunomodulatory Therapies
Part III—Treatment and Prevention of Infections
Full List of Articles
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Introduction
Overview of Treatment of Pediatric Acute-Onset Neuropsychiatric Syndrome Abstract Full Text Clinical Management of PANS Clinical Management of PANS: Part I–Psychiatric and Behavioral Interventions Part I—Psychiatric and Behavioral Interventions, Margo Thienemann, MD, Stanford University and coauthors present consensus guidelines for treating the psychiatric and behavioral symptoms of children with PANS/PANDAS. Symptom improvement is aimed at decreasing suffering, improving functioning, and making it easier for the children to adhere to therapeutic interventions. Abstract Full Text Clinical Management of PANS: Part II—Use of Immunomodulatory Therapies Part II—Use of Immunomodulatory Therapies, Jennifer Frankovich, MD, and coauthors provide recommendations to help guide the use of therapies targeting the neuroinflammation and post-infectious autoimmunity that are common in PANS-PANDAS. Abstract Full Text Clinical Management of PANS: Part III—Treatment and Prevention of Infections Part III—Treatment and Prevention of Infections, Michael Cooperstock, MD, MPH, University of Missouri School of Medicine (Columbia) and coauthors representing the PANS PANDAS Consortium, present a consensus guideline for managing the infection components of these neuropsychiatric conditions. Abstract Full Text |
Studies
Pediatric Acute-Onset Neuropsychiatric Syndrome Response to Oral Corticosteroid Bursts: An Observational Study of Patients in an Academic Community-Based PANS Clinic (Brown Kayla, Farmer Cristan, Farhadian Bahare, Hernandez Joseph, Thienemann Margo, and Frankovich Jennifer. JCAP. July 2017) Abstract Course of Neuropsychiatric Symptoms After Introduction and Removal of Nonsteroidal Anti-Inflammatory Drugs: A Pediatric Observational Study (Spartz Ellen J., Freeman G. Mark Jr., Brown Kayla, Farhadian Bahare, Thienemann Margo, and Frankovich Jennifer. JCAP. July 2017) Abstract Effect of Early and Prophylactic Nonsteroidal Anti-Inflammatory Drugs on Flare Duration in Pediatric Acute-Onset Neuropsychiatric Syndrome: An Observational Study of Patients Followed by an Academic Community-Based Pediatric Acute-Onset Neuropsychiatric Syndrome Clinic (Brown Kayla D., Farmer Cristan, Freeman G. Mark Jr., Spartz Ellen J., Farhadian Bahare, Thienemann Margo, and Frankovich Jennifer. JCAP. July 2017) Abstract |
Special Issue on PANS. Kiki Chang, MD, Guest Editor - JCAP 2015
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JCAP - Special Issue on Pediatric Acute-Onset Neuropsychiatric Syndrome -Kiki Chang, MD, Guest Editor
This is the first published collection of research papers concerning PANS and PANDAS by a Consortium of physicians and researchers. It represents the first group effort to methodically study PANS in order that improvements are made in diagnosis, evaluation, etiology and treatment. The Consortium is comprised of doctors and researchers who have diverse areas of expertise and are from highly respected hospitals and universities. This JCAP will help clinicians to better understand PANS as a whole, how to diagnose it and how to treat. The edition includes papers on use of Cefdinir, IVIG, Eating Disorders, IVIG, Plasma Apheresis, and more. These articles will help spread awareness on PANS itself as well as treatment options. The abstracts are available. Full articles are available for purchase or accessible at some medical libraries or via affiliations with some universities and hospitals. |
Full List of Articles
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Introduction
Special Issue on Pediatric Acute-Onset Neuropsychiatric Syndrome Kiki Chang, Harold S Koplewicz, Ron Steingard First Page View Consensus Statement Clinical Evaluation of Youth with Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS): Recommendations from the 2013 PANS Consensus Conference Kiki Chang, Jennifer Frankovich, Michael Cooperstock, Madeleine W. Cunningham, M. Elizabeth Latimer, Tanya K. Murphy, Mark Pasternack, Margo Thienemann, Kyle Williams, Jolan Walter, Susan E. Swedo, From the PANS Collaborative Consortium Full Consensus Statement Abstract Original Articles Characterization of the Pediatric Acute-Onset Neuropsychiatric Syndrome Phenotype Tanya K. Murphy, Priyal D. Patel, Joseph F. McGuire, Allison Kennel, P. Jane Mutch, E. Carla Parker-Athill, Camille E. Hanks, Adam B. Lewin, Eric A. Storch, Megan D. Toufexis, Gul H. Dadlani, Carina A. Rodriguez Abstract Clinical Presentation of Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections in Research and Community Settings Susan E. Swedo, Jakob Seidlitz, Miro Kovacevic, M. Elizabeth Latimer, Rebecca Hommer, Lorraine Lougee, Paul Grant Abstract Five Youth with Pediatric Acute-Onset Neuropsychiatric Syndrome of Differing Etiologies Jennifer Frankovich, Margo Thienemann, Sonal Rana, Kiki Chang Abstract Multidisciplinary Clinic Dedicated to Treating Youth with Pediatric Acute-Onset Neuropsychiatric Syndrome: Presenting Characteristics of the First 47 Consecutive Patients Jennifer Frankovich, Margo Thienemann, Jennifer Pearlstein, Amber Crable, Kayla Brown, Kiki Chang Abstract |
Disordered Eating and Food Restrictions in Children with PANDAS/PANS
Megan D. Toufexis, Rebecca Hommer, Diana M. Gerardi, Paul Grant, Leah Rothschild, Precilla D'Souza, Kyle Williams, James Leckman, Susan E. Swedo, Tanya K. Murphy Abstract Cefdinir for Recent-Onset Pediatric Neuropsychiatric Disorders: A Pilot Randomized Trial Tanya K. Murphy, E. Carla Parker-Athill, Adam B. Lewin, Eric A. Storch, P. Jane Mutch Abstract Use of Intravenous Immunoglobulin in the Treatment of Twelve Youths with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections Miro Kovacevic, Paul Grant, Susan E. Swedo Abstract Therapeutic Plasma Apheresis as a Treatment for 35 Severely Ill Children and Adolescents with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections M. Elizabeth Latimer, Nathan L'Etoile, Jakob Seidlitz, Susan E. Swedo Abstract Antineuronal Antibodies in a Heterogeneous Group of Youth and Young Adults with Tics and Obsessive-Compulsive Disorder Carol J. Cox, Amir J. Zuccolo, Erica V. Edwards, Adita Mascaro-Blanco, Kathy Alvarez, Julie Stoner, Kiki Chang, Madeleine W. Cunningham Abstract Cytokine Correlations in Youth with Tic Disorders E. Carla Parker-Athill, Jared Ehrhart, Jun Tan, Tanya K. Murphy Abstract Letter to the Editor PANDAS and Comorbid Kleine–Levin Syndrome Diana M. Gerardi, Joseph Casadonte, Priyal Patel, Tanya K. Murphy First Page |
