Posts by:
Gabriella True

Clinicopathologic Characteristics of PANDAS in a Young Adult: A Case Report
Kulumani Mahadevan LS, Murphy M, Selenica M, Latimer E, Harris BT. Clinicopathologic Characteristics of PANDAS in a Young Adult: A Case Report. Dev Neurosci. 2023;45(6):335-341. doi: 10.1159/000534061. Epub 2023 Sep 12. PMID: 37699369; PMCID: PMC10753865.

Although this disease is primarily thought to be a disease of childhood, it is reported to occur also in adults. PANDAS is a well-defined clinical entity, but the neuropathology of this condition has not been established yet. We describe the clinical course of a 26-year-old female diagnosed with PANDAS. She committed suicide and her brain was biobanked for further studies. We examined the banked tissue and performed special stains, immunohistochemical, and immunofluorescence analyses to characterize the neuropathology of this condition. Histology of the temporal lobes, hippocampus, and basal ganglia shows mild gliosis and Alzheimer’s type II astrocytes. Acute hypoxic ischemic changes were noted in hippocampus CA1 and CA2 areas. Immunostaining shows increased parenchymal/perivascular GFAP staining and many vessels with mild increases in CD3-, CD4-, and CD25-stained lymphocytes in the basal ganglia. The findings suggest that CD4- and CD25-positive T cells might have an important role in understanding the neuroinflammation and pathogenesis of this condition. The case represents the first neuropathological evaluation report for PANDAS.

A Sydenham’s Chorea Story
A Sydenham’s Chorea Story

  Sydenhams Chorea is the prototype disorder for PANDAS. If you or your child has chorea movements, it is important to get a correct diagnosis. One may have an easier...

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An Exploratory Study of Obsessive-Compulsive Behavior and School Problems Associated with Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS)
Maura A. Miglioretti, Ara J. Schmitt, Kara E. McGoey & M. Tina Benno (2023) An Exploratory Study of Obsessive-Compulsive Behavior and School Problems Associated with Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS), Journal of Applied School Psychology, DOI: 10.1080/15377903.2023.2263391
  • Parents (n = 208) of children with PANS participated in this study by completing a survey that included the Children’s Yale-Brown Obsessive-Compulsive Scale Parent Report (CY-BOCS-PR) and additional questions regarding child school performance and participation in formal special services at school.
  • Anxiety and inattention, among a variety of other school-based problems, are reported at school in this population.
  • Approximately 60% of the children received some form of school special services across their educational career.
  • A variety of mild to severe obsessive-compulsive behaviors were reported in the children, though the severity of those behaviors did not predict the existence of a school special services plan.
  • School psychologists must be prepared to evaluate children with PANS and recommend evidence-based interventions.
Symptom flares after COVID-19 infection versus vaccination among youth with PANS/PANDAS
LaRusso MD, Abadia CE. Symptom flares after COVID-19 infection versus vaccination among youth with PANS/PANDAS. Allergy Asthma Proc. 2023 Sep 1;44(5):361-367. doi: 10.2500/aap.2023.44.230049. PMID: 37641227; PMCID: PMC10476495.

These results support COVID-19 vaccination for most individuals with PANS/PANDAS given that the frequencies of symptom flares and setbacks after COVID-19 infection were significantly higher than after the COVID-19 vaccine. Nonetheless, further studies are needed to fully assess the risk-benefit balance and to provide a more individualized approach to disease prevention in people with immune vulnerabilities.

The PANDAS/PANS disorders. Is it time for more allergist-immunologists to get involved?
Bellanti JA. The PANDAS/PANS disorders. Is it time for more allergist-immunologists to get involved? Allergy Asthma Proc. 2023 Sep 1;44(5):296-305. doi: 10.2500/aap.2023.44.230029. PMID: 37641225.
Conclusion: The PANDAS/PANS disorders are a continuing burden for growing numbers of patients, health-care providers, and the global health-care systems, and are a particular challenge for the allergist/immunologist who is increasingly being called upon for their management. Because of the importance of immunologic factors in the pathogenesis and treatment of these conditions with anti-inflammatory and immune-modulating treatments, the allergist/immunologist is well poised to offer consultative care.
Efficacy of Antidopaminergic Pharmacotherapy in Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS): A Case Report
Hefelfinger D, Kaufman H, Gilman A, Gebhart R. Efficacy of Antidopaminergic Pharmacotherapy in Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS): A Case Report. Cureus. 2023 Aug 26;15(8):e44164. doi: 10.7759/cureus.44164. PMID: 37753029; PMCID: PMC10519259.

 

The use of antidopaminergic pharmacotherapy to target the induction of D1 and D2 dopamine receptors was efficacious in this patient and warranted additional research. Further studies must determine definitive treatment guidelines and look at biomarkers to aid the diagnosis. This will help in ensuring a timely diagnosis and treatment for patients and will help minimize the likelihood of neuropsychiatric symptoms persisting into adulthood.

The Dysfunctional Mechanisms Throwing Tics: Structural and Functional Changes in Tourette Syndrome
Lamanna J, Ferro M, Spadini S, Racchetti G, Malgaroli A. The Dysfunctional Mechanisms Throwing Tics: Structural and Functional Changes in Tourette Syndrome. Behav Sci (Basel). 2023 Aug 10;13(8):668. doi: 10.3390/bs13080668. PMID: 37622808; PMCID: PMC10451670.
“TS etiology is very complex, with strong genetic influences, repeated streptococcal infections, and also pre and perinatal phenomena [13]. There is accumulating evidence that immune dysregulation contributes to the pathophysiology of OCD, TS, and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS). TS patients may, in fact, have a predisposition to autoimmune responses or impaired general immunity; recently, beta-hemolytic streptococcal infections and/or an increase in anti-basal ganglia antibodies have been found in patients with TS. “
Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections and pediatric acute-onset neuropsychiatric syndrome.
Hoppin, K. M., & Doran, P. R. (2023). Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections and pediatric acute-onset neuropsychiatric syndrome. In M. M. Perfect, C. A. Riccio, & M. A. Bray (Eds.), Health-related disorders in children and adolescents: A guidebook for educators and service providers (pp. 185–193). American Psychological Association. https://doi.org/10.1037/0000349-022

In the mid-1990s, researchers at the National Institute of Mental Health identified a link between recent streptococcal infection and the development of neuropsychiatric symptoms such as separation anxiety, tics, behavioral regression, rage, restricted eating, and obsessive–compulsive disorder. Research has found that in pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) and pediatric acute-onset neuropsychiatric syndrome (PANS), exposure to an infection or other agent that activates the immune system creates an immune response that goes awry, causing autoantibodies (or immune cells) to attack brain cells instead. PANDAS and PANS are complex neurological disorders, and their medical impact is significant. Educators should be aware that PANDAS and PANS may have a significant impact on a student’s attendance. This chapter discusses etiology, prevalence and incidence, symptoms, and outcomes of PANDAS and PANS. It presents the psychoeducational implications and school-based interventions organized by medical management, cognitive/academic functioning, and social–emotional and behavioral functioning. (PsycInfo Database Record (c) 2023 APA, all rights reserved)

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Pediatric de novo movement disorders and ataxia in the context of SARS-CoV-2

Wilpert, NM., de Almeida Marcelino, A.L., Knierim, E. et al. Pediatric de novo movement disorders and ataxia in the context of SARS-CoV-2. J Neurol 270, 4593–4607 (2023). https://doi.org/10.1007/s00415-023-11853-5

Our data suggest that children with de novo movement disorders, and cerebellar inflammation can benefit from immune-modulating therapy, especially methylprednisolone, despite negative results in clinical investigations.