Research

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 antideoxyribonuclease–B 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. 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, 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.

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].”

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.

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.

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.

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.

Letter to the Edito. Antistreptolysin-O Titers: Implications for Adult PANDAS. ANDREW J. CHURCH, B.SC., and RUSSELL C. DALE, M.B.CH.B., M.R.C.P., London, U.K. Published Online:1 Feb 2002. https://doi.org/10.1176/appi.ajp.159.2.320

To the Editor: A diagnosis of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is made when neuropsychiatric disease is precipitated by streptococcal infection (1). Antistreptolysin-O titers are an important tool for diagnosing recent streptococcal infection in patients with Sydenham’s chorea and PANDAS, since throat cultures are usually negative because of the latent onset of the neuropsychiatric disease. The upper limit for normal antistreptolysin-O titers is 200 IU/ml in children, but no normal upper limit exists for healthy adults. We propose that an antistreptolysin-O titer of 270 IU/ml is the significant upper limit for healthy adults. This finding will aid in the investigation and diagnosis of new cases of adult PANDAS.