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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

NSAIDs given prophylactically or within 30 days of flare onset may shorten neuropsychiatric symptom duration in patients with new-onset and relapsing/remitting PANS and PANDAS. A randomized placebo-control clinical trial of NSAIDs in PANS is warranted to formally assess treatment efficacy.

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,Frankovich, Jennifer
JCAP-July 2017

Improvement in neuropsychiatric symptoms was evident in roughly one-third of NSAID treatment trials. A randomized clinical trial will be necessary to confirm whether NSAIDs are successful in reducing neuropsychiatric symptoms in youth with PANS.

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-2017

Corticosteroids may be a helpful treatment intervention in patients with new-onset and relapsing/remitting PANS and PANDAS, hastening symptom improvement or resolution. When corticosteroids are given earlier in a disease flare, symptoms improve more quickly and patients achieve clinical remission sooner. Longer courses of corticosteroids may result in more durable remissions. A double-blind placebo-controlled clinical trial of corticosteroids in PANS is warranted to formally assess treatment efficacy.

 

Clinical Management of PANS: Part II—Use of Immunomodulatory Therapies

Frankovich Jennifer, Swedo Susan, Murphy Tanya, Dale Russell C., Agalliu Dritan, Williams Kyle, Daines Michael, Hornig Mady, Chugani Harry, Sanger Terence, Muscal Eyal, Pasternack Mark, Cooperstock Michael, Gans Hayley, Zhang Yujuan, Cunningham Madeleine, Bernstein Gail, Bromberg Reuven, Willett Theresa, Brown Kayla, Farhadian Bahare, Chang Kiki, Geller Daniel, Hernandez Joseph, Sherr Janell, Shaw Richard, Latimer Elizabeth, Leckman James, Thienemann Margo, and PANS/PANDAS Consortium
Journal of Child and Adolescent Psychopharmacology-2017
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These recommendations will help guide the use of anti-inflammatory and immunomodulatory therapy in the treatment of PANS.

Clinical Management of PANS: Part I–Psychiatric and Behavioral Interventions

Thienemann Margo, Murphy Tanya, Leckman James, Shaw Richard, Williams Kyle, Kapphahn Cynthia, Frankovich Jennifer, Geller Daniel, Bernstein Gail, Chang Kiki, Elia Josephine, and Swedo Susan
Journal of Child and Adolescent Psychopharmacology-July 2017
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While underlying infectious and inflammatory processes in PANS and PANDAS patients are treated, psychiatric and behavioral symptoms need simultaneous treatment to decrease suffering and improve adherence to therapeutic intervention. Psychological, behavioral, and psychopharmacologic interventions tailored to each child’s presentation can provide symptom improvement and improve functioning during both the acute and chronic stages of illness. In general, typical evidence-based interventions are appropriate for the varied symptoms of PANS and PANDAS. Individual differences in expected response to psychotropic medication may require marked reduction of initial treatment dose. Antimicrobials and immunomodulatory therapies may be indicated, as discussed in Parts 2 and 3 of this guideline series.

Overview of Treatment of Pediatric Acute-Onset Neuropsychiatric Syndrome

Swedo Susan E., Frankovich Jennifer, and Murphy Tanya K
Journal of Child and Adolescent Psychopharmacology-2017

Thus, treatment of PANS depends on three complementary modes of intervention:

  • Treating the symptoms with psychoactive medications, psychotherapies (particularly cognitive behavioral therapy), and supportive interventions.
  • Removing the source of the inflammation with antimicrobial interventions.
  • Treating disturbances of the immune system with immunomodulatory and/or anti-inflammatory therapies.

 

Hello from the Other Side: How Autoantibodies Circumvent the Blood–Brain Barrier in Autoimmune Encephalitis

Maryann P. Platt, Dritan Agalliu, Tyler Cutforth
Frontiers in Immunology-2017

Autoimmunity in the CNS remains confoundingly complex, in both etiology and treatment. While triggers for AE are defined in some cases, frequently, no clear infectious or cancerous cause can be found. BBB integrity clearly plays a role in disease development, and more research on differential barrier permeability over the course of disease would resolve many questions about autoantibody entry and pathogenesis.

Improvement of psychiatric symptoms in youth following resolution of sinusitis

Talia Mahony, Douglas Sidell, Hayley Gans, Kayla Brown, Bahare Farhadian, Melissa Gustafson, Janell Sherr, Margo Thienemann, Jennifer Frankovich
International Journal of Pediatric Otorhinolaryngology-2017

Improvement of psychiatric symptoms correlated with resolution of sinus disease in this retrospective study. Identification, treatment, and resolution of underlying infections, including sinusitis, may have the potential to change the trajectory of some neuropsychiatric illnesses.

Randomized, Controlled Trial of Intravenous Immunoglobulin for Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections

Kyle A. Williams, MD, PhD, Susan E. Swedo, MD, Cristan A. Farmer, PhD, Heidi Grantz, LCSW, Paul J. Grant, MD, Precilla D’Souza, CRNP, Rebecca Hommer, MD, Liliya Katsovich, MA, Robert A. King, MD, James F. Leckman, MD, Phd
Journal of the American Academy of Child & Adolescent Psychiatry–2016

IVIG was safe and well tolerated. Between-group differences were smaller than anticipated, and the double-blind comparison failed to demonstrate superiority of IVIG over placebo. The observed open-label improvements indicate that future trials would benefit from larger sample sizes designed in part to aid in the identification of biomarkers predictive of a positive response to immunotherapy. Future investigations focused on the natural history of PANDAS are also warranted.