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Intravenous immunoglobulin treatment improves multiple neuropsychiatric outcomes in patients with pediatric acute-onset neuropsychiatric syndrome
Eremija J, Patel S, Rice S, Daines M. Intravenous immunoglobulin treatment improves multiple neuropsychiatric outcomes in patients with pediatric acute-onset neuropsychiatric syndrome. Front Pediatr. 2023 Oct 16;11:1229150. doi: 10.3389/fped.2023.1229150. PMID: 37908968; PMCID: PMC10613689.
  • A 5-year retrospective study was undertaken in Children’s Postinfectious Autoimmune Encephalopathy Center at University of Arizona.
  • 12 children diagnosed with PANS and treated with immunomodulatory IVIG dose who completed neuropsychological testing before and after treatment
  • Participants received 1–7 IVIG courses.
  • Improvement occurred in 11/12 patients, in one or multiple domains/subdomains, independently of time between disease onset and IVIG initiation (0–7 years)
  • Improvement was primarily seen in memory (58%), sensory-motor (37%) and visual-motor integration (30%).
  • 5/12 had hypogammaglobulinemia requiring ongoing IVIG replacement. Emphasizes the presumed role of immune dysfunction in disease pathogenesis.
  • 1/12 had isolated low IgA. 1/12 discontinued IVIG therapy due to severe adverse effects
Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections Manifestations in a Teenager

Sivakanthan A, Gedeon J, Sadaf S, et al. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections manifestations in a teenager. Prim Care Companion CNS Disord. 2023;25(5):23cr03505. October 5, 2023. https://doi.org/10.4088/PCC.23cr03505

This is a case of an 18-year-old male patient with PANDAS resistant to most treatments recommended in the literature. The patient lives with his parents, was recently home schooled, and has a history of autoimmune encephalitis, OCD, and Tourette’s disorder. He was admitted to an inpatient psychiatric unit for daily physical aggression toward his parents.

Estimate of the incidence of PANDAS and PANS in 3 primary care populations
Wald ER, Eickhoff J, Flood GE, Heinz MV, Liu D, Agrawal A, Morse RP, Raney VM, Veerapandiyan A, Madan JC. Estimate of the incidence of PANDAS and PANS in 3 primary care populations. Front Pediatr. 2023 Sep 21;11:1170379. doi: 10.3389/fped.2023.1170379. PMID: 37808558; PMCID: PMC10551157.

Results: The combined study population consisted of 95,498 individuals. The majority were non-Hispanic Caucasian (85%), 48% were female and the mean age was 7.1 (SD 3.1) years. Of 357 potential cases, there were 13 actual cases [mean age was 6.0 (SD 1.8) years, 46% female and 100% non-Hispanic Caucasian]. The estimated annual incidence of PANDAS/PANS was 1/11,765 for children between 3 and 12 years with some variation between different geographic areas.

Conclusion: Our results indicate that PANDAS/PANS is a rare disorder with substantial heterogeneity across geography and time. A prospective investigation of the same question is warranted.

Post-infectious inflammation, autoimmunity, and OCD: Sydenham Chorea, Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infection (PANDAS), and Pediatric Acute-onset Neuropsychiatric Disorder (PANS)

A Vreeland; D Calaprice; N Or-Geva; RE. Frye; D Agalliu; HM. Lachman; C Pittenger; S Pallanti; K Williams; M Ma; M Thienemann; A Gagliano; E Mellins; J Frankovich https://doi.org/10.1159/000534261

In this review article, we outline the accumulating evidence supporting the role neuroinflammation plays in these disorders. We describe work with animal models including patient-derived anti-neuronal autoantibodies, and we outline imaging studies that show alterations in the basal ganglia. In addition, we present research on metabolites, which are helpful in deciphering functional phenotypes, and on the implication of sleep in these disorders. Finally, we encourage future researchers to collaborate across medical specialties (e.g., pediatrics, psychiatry, rheumatology, immunology, and infectious disease)in order to further research on clinical syndromes presenting with neuropsychiatric manifestations.

Living with Paediatric acute-onset neuropsychiatric syndrome (PANS) – a qualitative study of Children’s experiences in Sweden

N Ringer, C Benjaminson, H Bejnö, Living with Paediatric acute-onset neuropsychiatric syndrome (PANS) – a qualitative study of Children’s experiences in Sweden. Cogent Psychology (2023), 10: 2259745 DOI:10.1080/23311908.2023.2259745

The study aimed to understand the experience of living with PANS from the perspective of children with the diagnosis.

  • PANS causes suffering due to the aversive emotional, cognitive, and physical experiences and the negative consequences the symptoms cause. The dramatic and abrupt onset plus the nature of the symptoms cause intense fear.
  • PANS makes them feel that they have no control or power over their lives. It is complicated due to the difficulty in predicting and managing symptoms. Also, difficult to describe their needs. It is difficult to work with providers who do not listen or really try to understand their needs and experiences.
  • Meaningful support consists of having someone who does not give up and will continue trying treatments. Also, caregivers who are stable, empowering, and supportive are important.
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.