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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.

Palatal Petechiae in the Absence of Group A Streptococcus in Pediatric Patients with Acute-Onset Neuropsychiatric Deterioration: A Cohort Study

Mahony Talia, Sidell Douglas, Gans Hayley, Cooperstock Michael, Brown Kayla, Cheung Joanne M, Farhadian Bahare, Gustafson Melissa, Thienemann Margo, and Frankovich Jennifer
JCAP-2017

Despite the established relationship between palatal petechiae and GAS, no patient with palatal petechiae in our clinic tested positive for GAS and only 32% had evidence of recent GAS. Most did not have an identifiable cause for the palatal lesions. This finding suggests the potential for alternative causes of palatal petechiae or undetectable GAS in our patient population. The high prevalence of palatal petechiae without GAS infection suggests that the pathogenesis of PANS is multifactorial and may involve disruption or inflammation of the microvasculature.

Prospective Longitudinal Analysis of Immune Responses in Pediatric Subjects After Pharyngeal Acquisition of Group A Streptococci

Nicholas D. Hysmith, Edward L. Kaplan, P. Patrick Cleary, Dwight R. Johnson, Thomas A. Penfound, James B. Dale
Journal of Pediatric Infectious Disease Society-2017
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The prospective study examining the immunological response to infection by group A streptococcus (GAS) was performed in serum samples obtained from pediatric subjects. Study examined the immune response to 13 shared GAS antigens and 18 type-specific M peptides, but found no novel pattern of immune responses. Importantly, the study found that 65% of new streptococcus infections did not result in symptoms despite a detectable immunological response. This finding is supported by previous research and suggests that the majority of GAS infections remain undetected and contribute to the burden of the disease when left untreated.

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.