Research

Novel Drug Significantly Reduces Tics in Tourette’s ― Without Side Effects

An investigational drug that blocks the dopamine-1 (D1) receptor reduces tics and is safe and well tolerated in children with Tourette syndrome (TS), a new study shows.

Importantly, unlike current medications for the disorder, ecocipam does not lead to weight gain, anxiety, depression, or tardive dyskinesia compared to placebo ― a factor that may lead to better adherence.

A case of Obsessive-compusive and related disorders against the background of Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)
Madaan A, Das A, Das SK, Das RC. A case of Obsessive-compusive and related disorders against the background of Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS). Indian J Psychiatry. 2022 Mar;64(Suppl 3):S659. doi: 10.4103/0019-5545.341932. Epub 2022 Mar 24. PMCID: PMC9129622.
Conclusion: In all school-age children presenting with obsessive compulsive and related disorder with other symptoms or not, a possible link to PANDAS should be evaluated and ruled out.
Case Report – Pedijatrijsko autoimuno neuropsihijatrijsko oboljenje udruženo sa streptokokom

Pedijatrijsko autoimuno neuropsihijatrijsko oboljenje udruženo sa streptokokom

Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection


Jasmina Škorić1, Bojan Pavković1.
1 Dom zdravlja Dr Simo Milošević’, Beograd, Srbija

http://prevped.preventivnapedijatrija.rs/index.php/casopis/article/download/41/24

Conclusion: The patient showed signs of improvement after the intravenous administration of immunoglobulin which confirmed the underlying immunological aspect of the disease. Additionally, serologic test results that showed elevated antistreptolysin O titer and antideoxyribonucleaseB titer indicated streptococcal etiology. Precisely, these misdirected antibodies set off an inflammatory response that led to the basal ganglia damage resulting in behavioral changes, motor, cognitive and emotional disorders.

PANDAS in an Adult?: A Case Report

PANDAS in an Adult?: A Case Report. Deshmukh RP, Mane AB, Singh S.Ind J Priv Psychiatry 2022; 16 (1):44-45. DOI: 10.5005/jp-journals-10067-0104

“We report a case in order to suggest that we should be alert while assessing OCD in young patients, as it might be secondary to streptococcal infection. This has therapeutic implications. Trials of immunomodulator therapy can be given as suggested in a few kinds of literature. Although still experimental, it may have potential for the future, especially in those not responding to conventional treatment… This report might suggest that PANDAS-like syndrome may occur in adults also. We should be cautious of this syndrome during treatment.”

Neuroinflammation in a Rat Model of Tourette Syndrome

Neuroinflammation in a Rat Model of Tourette Syndrome, Zhongling Ke, Yanhui Chen, Guofeng Chen, Yanyan Liu, Neuroinflammation in a Rat Model of Tourette Syndrome, Frontiers in Behavioral Neuroscience, Vol 16, Front. Behav. Neurosci., 10 March 2022, DOI: 10.3389/fnbeh.2022.710116   

Conclusion: The IDPN-induced TS rats had significant neuroinflammation in the brain, and the interaction between dopamine (DA) dysregulation and immune dysfunction may play a vital role in the pathogenic mechanisms of TS.

Therapeutic Apheresis and Immunosuppression in Immunologic Diseases: A Review and Own Observations

Bambauer R, Schiel R. Therapeutic Apheresis and Immunosuppression in Immunologic Diseases: A Review and OwnObservations. Clin Immunol Res. 2021; 5(2): 1-36. ISSN 2639-8486

TA should be reserved for treatment of children and adolescents

who are severely affected by PANDAS. In such patients, it


appears to be safe, well-tolerated, and beneficial treatment


option [130]. Bien et al reported in 2020, besides the first-line


interventions of steroids, IVIG, and TA as second-line treatments


cyclophosphamide or rituximab [131].”

Abnormal mTOR Activity in Pediatric Autoimmune Neuropsychiatric and MIA-Associated Autism Spectrum Disorders

Trifonova, E.A.; Mustafin, Z.S.; Lashin, S.A.; Kochetov, A.V. Abnormal mTOR Activity in Pediatric Autoimmune Neuropsychiatric and MIA-Associated Autism Spectrum Disorders. Int. J. Mol. Sci. 2022, 23, 967. https://doi.org/10.3390/ijms23020967

Abstract: Autism spectrum disorder (ASD) is a neurodevelopmental condition characterized by

the early onset of communication and behavioral problems. ASD is highly heritable; however,

environmental factors also play a considerable role in this disorder. A significant part of both

syndromic and idiopathic autism cases could be attributed to disorders caused by mammalian

target of rapamycin (mTOR)-dependent translation deregulation. This narrative review analyzes both bioinformatic and experimental evidence that connects mTOR signaling to the maternal autoantibody-related (MAR) autism spectrum and autoimmune neuropsychiatric disorders simultaneously. In addition, we reconstruct a network presenting the interactions between the mTOR signaling and eight MAR ASD genes coding for ASD-specific maternal autoantibody target proteins. The research discussed in this review demonstrates novel perspectives and validates the need for a subtyping of ASD on the grounds of pathogenic mechanisms. The utter necessity of designing ELISA-based test panels to identify all antibodies related to autism-like behavior is also considered.

Discovering prescription patterns in pediatric acute-onset neuropsychiatric syndrome patients
Lopez Pineda A, Pourshafeie A, Ioannidis A, Leibold CM, Chan AL, Bustamante CD, Frankovich J, Wojcik GL. Discovering prescription patterns in pediatric acute-onset neuropsychiatric syndrome patients. J Biomed Inform. 2021 Jan;113:103664. doi: 10.1016/j.jbi.2020.103664. Epub 2020 Dec 28. PMID: 33359113.
  • PANS has an inflammatory or autoimmune etiology associated with an infection.
  • Longitudinal clustering can subset patients with polypharmacy treatments.
  • Included 43 consecutive new-onset pre-pubertal patients who had at least 3 clinic visits. “We used a cut-off age of 12 years to include only pre-pubertal children, as hormones might play a role in psychiatric symptoms and behaviors.”
  • Algorithm identified six clusters with distinct medication usage history which may represent clinician’s practice of treating PANS of different severities and etiologies i.e.,
    • two most severe groups requiring high dose intravenous steroids
    • two arthritic or inflammatory groups requiring prolonged nonsteroidal anti-inflammatory drug (NSAID)
    • two mild relapsing/remitting group treated with a short course of NSAID.
  • The psychometric scores as outcomes in each cluster generally improved within the first two years.
    Diagnostics, Differential Diagnostics And The Treatment Of Pandas Syndrome – Case Description

    Diagnostics, Differential Diagnostics And The Treatment Of Pandas Syndrome – Case Description Mukhtorjonova Khusnigul Nodirbekovna, Tukhtabayeva Kamila Abrarovna, Tashkent Padiatric Medical Institute. World Bulletin of Public Health (WBPH). Volume-6, January 2022. ISSN: 2749-3644

    This article gives a case of PANDAS syndrome in an 11-year-old boy. The
    course and clinic of the disease in the form of prolonged sneezing attacks
    made it difficult to diagnose this syndrome. Conducting biochemical blood
    tests, daily video electroencephalographic monitoring (EEG monitoring) and
    magnetic resonance imaging of the brain (MRI) allowed to determine the
    condition. The appointment of antibiotic therapy and intravenous
    administration of immunoglobulin led to remission of the disease. It is
    necessary to conduct further studies to determine the effectiveness of the
    above drugs for this syndrome.

    Read case study.

    Profiling Behavioral and Psychological Symptoms in Children undergoing treatment for Spondyloarthritis and Polyarthritis

    McHugh A, Chan A, Herrera C, Park JM, Balboni I, Gerstbacher D, Hsu JJ, Lee T, Thienemann M, Frankovich J. Profiling Behavioral and Psychological Symptoms in Children undergoing treatment for Spondyloarthritis and Polyarthritis. J Rheumatol. 2022 Feb 1:jrheum.210489. doi: 10.3899/jrheum.210489. Epub ahead of print. PMID: 35105715.

    Results: There were 111 patients and 1753 healthy controls (HCs). Compared to HCs, patients with SpA or PolyA had worse total competence and internalizing scores. Higher cJADAS10 scores were associated with worse total competence, worse internalizing, and higher total problems scores. Most of these differences reached statistical significance (P < 0.01). Self-harm/suicidality was almost 4-fold higher in patients with PolyA than HCs (OR 3.6, 95% CI 1.3-9.6, P = 0.011).

    Conclusion: Our study shows that patients with SpA and PolyA with more active disease have worse psychological functioning in activities, school, and social arenas, and more internalized emotional disturbances, suggesting the need for regular mental health screening by rheumatologists.

    Status Of Vitamin D In Children With Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS)
    McNally JD, Menon K, Chakraborty P, Fisher L, Williams KA, Al-Dirbashi OY, Doherty DR; Canadian Critical Care Trials Group. The association of vitamin D status with pediatric critical illness. Pediatrics. 2012 Sep;130(3):429-36. doi: 10.1542/peds.2011-3059. Epub 2012 Aug 6. PMID: 22869837.
    “Numerous studies have indicated an association between vitamin D deficiency and the immune dysregulation and the pathogenesis of autoimmunity. An increased prevalence of vitamin D deficiency has been demonstrated in several pediatric autoimmune diseases, including autoimmune thyroid disease and Type-1 diabetes mellitus. This study was undertaken to determine the prevalence of vitamin D deficiency in children with PANS, an autoimmune disorder characterized by abrupt-onset neuropsychiatric symptoms.”
    Neuropsychiatric syndrome with myoclonus after SARS-CoV-2 infection in a paediatric patient

    M. Della Corte, C. Delehaye, E. Savastano, M.F. De Leva, P. Bernardo, A. Varone, Neuropsychiatric syndrome with myoclonus after SARS-CoV-2 infection in a paediatric patient, Clinical Neurology and Neurosurgery, Volume 213, 2022. DOI: 10.1016/j.clineuro.2022.107121

    • 12 Year old subacutely developed a positive and negative myoclonus of limbs and face, drowsiness and memory deficits after getting infected by SARS-CoV-2.
    • Neuropsychological examination showed an impairment in memory, visual-motor coordination, inductive reasoning skills, attention, and concentration
    • First treated with clonazepam and then with intravenous methylprednisolone for five days, with poor response.
      • Then received a cycle of IVIG, thus reaching a gradual and complete recovery.
    • To date, this is the first case of a COVID-19 associated myoclonus affecting a paediatric patient.