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Intravenous immunoglobulin for the treatment of autoimmune encephalopathy in children with autism

Kathleen Connery, Marie Tippett, Leanna M. Delhey, Shannon Rose, John C. Slattery, Stephen G. Kahler, Juergen Hahn, Uwe Kruger, Madeleine W. Cunningham, Craig Shimasaki, Richard E. Frye
Transl Psychiatry-2018

In an open-labeled IVIG study in children with comorbid ASD and PANS/ PANDAS, anti-tubulin and anti-D2R (as measured by the Cunningham panel) were associated with responsiveness to IVIG treatment, suggesting that these autoantibodies could be biomarkers to select for positive IVIG treatment outcomes. Research continues to explore serum biomarkers and genetic risk factors that can provide a diagnostic tool and/or complement diagnosis in children with PANS/PANDAS.

Family Impacts Reported By Parents Raising Children With Pediatric Acute-onset Neuropsychiatric Syndrome (PANS)

Marcey Mettica, B.S., M.S
A Dissertation Submitted in Partial Fulfillment of the Requirements for The Degree of Doctor of Family Studies in The Graduate School of The Texas Woman’s University
PDF of Dissertation

This study explored variables that were thought to possiblyinfluence family impact and explored relationships between demographic factors, illness factors, and symptom severity with impact on the family

Borrelia burgdorferi adhere to blood vessels in the dura mater and are associated with increased meningeal T cells during murine disseminated borreliosis

li Divan, Timothy Casselli, S. Anand Narayanan, Sanjib Mukherjee, David C. Zawieja, John A. Watt, Catherine A. Brissette, M. Karen Newell-Rogers, Published: May 3, 2018. https://doi.org/10.1371/journal.pone.0196893

Traditionally, mice haven’t shown neuroborreliosis, mirroring human symptoms, but this study achieved colonization of the central nervous system in mice. Using two strains of B. burgdorferi, researchers infected mice through the skin, consistently detecting spirochetes in the brain membranes 75 days post-infection. Live spirochetes were present, adhering to blood vessels, indicating long-term infection. The presence of CD3+ T cells suggested an immune response. These findings provide new avenues for studying neuroborreliosis and may offer insights into potential treatments for patients.

Gut Microbiota Profiling and Gut-Brain Crosstalk in Children Affected by Pediatric Acute-Onset Neuropsychiatric Syndrome and Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections

Quagliariello A, Del Chierico F, Russo A, Reddel S, Conte G, Lopetuso LR, Ianiro G, Dallapiccola B, Cardona F, Gasbarrini A, Putignani L.
Front Microbiology-2018
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Excerpt: The results obtained from this study suggest that streptococcal infections can alter gut microbial communities leading to a pro-inflammatory state in the gut by selecting for specific bacterial strains that are normally associated with gut inflammation and activation of the immune response. This condition is likely maintained in patients, even after the infection itself has resolved.

PANDAS and anorexia nervosa-a spotters’ guide: Suggestions for medical assessment
Vincenzi B, O’Toole J, Lask B. PANDAS and anorexia nervosa–a spotters’ guide: suggestions for medical assessment. Eur Eat Disord Rev. 2010 Mar;18(2):116-23. doi: 10.1002/erv.977. PMID: 20148396.
Conclusion: Given the current state of knowledge, diagnosingPANDAS is extraordinarily difficult. However, extrapolating from knowledge aboutSydenham’s choreaandPANDAS–OCD and PANDAS–tics/TS, a heightened index of suspicion for the existence of an auto-immune reaction in the brain response to environmental infectious agents culminating in ‘anorexia nervosa’ should be maintained.
Principles and approaches to the treatment of immune-mediated movement disorders

Mohammad SS, Dale RC
Eur J Paediatric Neurol-2018

Excerpt: Immune mediated movement disorders include movement disorders in the context of autoimmune encephalitis such as anti-NMDAR encephalitis, post-infectious autoimmune movement disorders such as Sydenham chorea, paraneoplastic autoimmune movement disorders such as opsoclonus myoclonus ataxia syndrome, and infection triggered conditions such as paediatric acute neuropsychiatric syndrome. This review focuses on the approach to treatment of immune mediated movement disorders, which requires an understanding of the immunopathogenesis, whether the disease is destructive or ‘altering’, and the natural history of disease. 

Paediatric Autoimmune Neuropsychiatric Disorder Associated with Group A Beta-Haemolytic Streptococcal Infection: An Indication for Tonsillectomy? A Review of the Literature

Rajgor AD, Hakim NA, Ali S, Darr A.
Int J Otolaryngology-2018
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PANDAS is a significantly debilitating condition, with detrimental effects on a child’s psychophysical development. This review provides an insight into the current literature and the potential for surgery to treat PANDAS. However due to the current limitations of the literature drawing definitive conclusions is difficult. Thus, the next required steps would involve conducting a prospective collaborative study.

Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection (PANDAS): Clinical Manifestations, IVIG Treatment Outcomes, Results from a Cohort of Italian Patients

Piero Pavone, Raffaele Falsaperia, Francesco Nicita, Andreana Zecchini, Chiari Battaglia, Alberto Spalice, Lucia Iozzi, Enrico Parano, Giovanna Vitaliti, Alberto Verrotti, Vincenzo Belcastro, Sung Yoon Cho, Dong-Kyu Jin, Salvatore Savasta 
Neuropsychiatry-2018
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In this cohort, 29 out of 34 of children treated with IVIG showed a reduction or disappearance of the symptomatology, while in 5 cases (15%), despite 3 IVIG courses, there was only temporary improvement of clinical symptoms, with their reappearance within 1 to 6 months. According to the results of literature and ours no definitive conclusions may be drawn from IVIG treatment for PANDAS. Limitations of the present study include the absence of casecontrols and long-term follow up. However, in our opinion, treatment with IVIG has been shown to be effective in PANDAS children with a serious-severe type and to be well tolerated. As it has been reported by Frankovic et al.  most of the PANS Research Consortium (PRC) members prefer use of IVIG in treatment of patients with this disorder in moderate to severe forms.