Blog

The neuropsychological profile of children with basal ganglia encephalitis: a case series

Pawela C, Brunsdon RK, Williams TA, Porter M, Dale RC, Mohammad SS
Dev Med Child Neurol-2016
PDF

Although based on a small, cross‐sectional case series, our results highlight the potential importance of early identification and IVIg treatment of BGE. Given the cognitive and behavioural deficits described in this report, our clinical recommendation would be to try to treat patients rapidly with immune therapies, when clinical suspicion of BGE is high, to minimize the inflammatory component of the disease.

Expression of C-Reactive Protein and Serum Amyloid A in Early to Late Manifestations of Lyme Disease
Uhde M, Ajamian M, Li X, Wormser GP, Marques A, Alaedini A. Expression of C-Reactive Protein and Serum Amyloid A in Early to Late Manifestations of Lyme Disease. Clin Infect Dis. 2016 Dec 1;63(11):1399-1404. doi: 10.1093/cid/ciw599. Epub 2016 Sep 1. PMID: 27585799; PMCID: PMC5106611.

The immune response to invasive microbes involves two main components: the innate and adaptive response. C-reactive protein (CRP) and serum amyloid A (SAA) are proteins elevated during inflammation. In Lyme disease, CRP is elevated in early stages with erythema migrans but normal in neurological symptoms and arthritis. However, post-treatment Lyme disease syndrome (PTLDS) shows higher CRP, indicating persistent inflammation. SAA is elevated only in patients with erythema migrans. These findings suggest that lingering symptoms post-antibiotic therapy in Lyme disease are associated with elevated CRP, warranting further study on long-term inflammatory processes.

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.

Microglial Dysregulation in OCD, Tourette Syndrome, and PANDAS
Luciana Frick, Christopher Pittenger, “Microglial Dysregulation in OCD, Tourette Syndrome, and PANDAS”, Journal of Immunology Research, vol. 2016, Article ID 8606057, 8 pages, 2016. https://doi.org/10.1155/2016/8606057

Mechanisms by which microglial abnormalities contribute to disease are likely to be shared across distinct etiologies and traditional diagnoses. For instance, abnormal synaptic pruning was observed both in animals inoculated with GAS (which may capture key elements of the pathophysiology of PANDAS) and in mice that develop excessive grooming after inactivation of the progranulin gene. In both cases, increased synaptic pruning cooccurs with microglia activation.

Pediatric Autoimmune Disorders Associated with Streptococcal Infections and Tourette’s Syndrome in Preclinical Studies

Chiara Spinello, Giovanni Laviola and Simone Macrì
Frontiers in Neuroscience – 2016
PDF

In the present review we discuss the available evidence in preclinical models in support of the link between TS and pediatric autoimmune neuropsychiatric disorders associated with streptococcus infections (PANDAS), and the existing gaps that future research shall bridge. Specifically, we report recent preclinical evidence indicating that the immune responses to repeated streptococcal immunizations relate to the occurrence of behavioral and neurological phenotypes reminiscent of TS.

Pandas: A Rare Case Report

Y. Kulkarni, R. Warkari, “Pandas: A Rare Case Report”, International Journal of Science and Research (IJSR), Volume 5 Issue 2, February 2016, pp. 148-149, https://www.ijsr.net/get_abstract.php?paper_id=NOV161061

Abstract: PANDAS, also labelled as PANS, is a pediatric autoimmune neuropsychiatric disorder due to streptococcal infection. Although it affects 1 in 1000 children, cases reported in Indian context are relatively few because of lack of awareness about the syndrome amongst clinicians. We report here a case of a 13 years old male from a rural area presenting to us with an acute onset of repetitive behavior following a febrile illness 2 months prior. Investigations confirmed rheumatic valvular heart disease. A diagnosis of PANDAS was made and treatment was started. The patient responded to treatment and gained symptomatic relief. Timely intervention
and high index of suspicion towards a pediatric febrile illness can help to reduce the morbidity associated with this neuropsychiatric
disorder.

Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS)

Graziella Orefici, PhD, Francesco Cardona, MD, Carol J. Cox, PhD, and Madeleine W. Cunningham, PhD.
From the Book: Streptococcus pyogenes Basic Biology to Clinical Manifestations – University of Oklahoma Health Sciences Center-2016
PDF

The inclusion of a chapter on pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (or PANDAS) is essential to provide a history of the disease and provide current information about its association with Streptococcus pyogenes (group A streptococci), tics, obsessive compulsive disorder (OCD) and its relationship to Sydenham chorea (SC), which is the neurologic manifestation of acute rheumatic fever.

N-Acetylcysteine in the Treatment of Excoriation Disorder – A Randomized Clinical Trial

Jon E. Grant, JD, MD, MPH; Samuel R. Chamberlain, MD, PhD; Sarah A. Redden, BA; Eric W. Leppink, BA; Brian L. Odlaug, PhD; Suck Won Kim, MD
JAMA Psychiatry-2016

This investigation suggests that N-acetylcysteine appears to be effective and well tolerated in the acute treatment of SPD. As effective treatments for skin picking emerge, it becomes increasingly important that physicians and other mental health care professionals screen for the disorder to provide timely treatment.