Diagnostic Approach to Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS): A Narrative Review of Literature Data

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) are clinical conditions characterized by the sudden onset of obsessive–compulsive disorder and/or tics, often accompanied by other behavioral symptoms in a group of children with streptococcal infection. PANDAS-related disorders, including pediatric acute-onset neuropsychiatric syndrome (PANS), childhood acute neuropsychiatric symptoms (CANS), and pediatric infection triggered autoimmune neuropsychiatric disorders (PITANDs), have also been described. Since first defined in 1998, PANDAS has been considered a controversial diagnosis. A comprehensive review of the literature was performed on PubMed and Scopus databases, searching for diagnostic criteria and diagnostic procedures of PANDAS and related disorders. We propose a test panel to support clinicians in the workout of PANDAS/PANS patients establishing an appropriate treatment. However, further studies are needed to improve our knowledge on these acute-onset neuropsychiatric conditions.

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Plasmapheresis, Rituximab, and Ceftriaxone Provided Lasting Improvement for a 27-Year-Old Adult Male with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS)

Case Reports in Psychiatry ◽

10.1155/2021/8697902 ◽

2021 ◽

Vol 2021 ◽

pp. 1-4

Author(s):

Adam Krouse ◽

Huihua Li ◽

Joseph A. Krenzer ◽

William Nicholas Rose

Keyword(s):

Clinical Symptoms ◽

Neuropsychiatric Symptoms ◽

Neuropsychiatric Disorders ◽

Sudden Onset ◽

Autoimmune Response ◽

Urine Drug Screen ◽

Streptococcal Infections ◽

Obsessive Compulsive ◽

Compulsive Disorder ◽

Old Adult

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is a specific autoimmune response to group-A streptococcal (GAS) infections in children and adolescents with a sudden onset of neuropsychiatric disorders including obsessive-compulsive disorder (OCD) or tic-like symptoms. We present a case report of a 27-year-old male patient who had lasting improvement with plasmapheresis, rituximab, and ceftriaxone. Our patient first developed sudden psychosis and confusion after GAS infections at age 17. He had elevated anti-streptolysin O (ASO) titers, negative urine drug screen, no ETOH in blood, normal CBC, normal TSH, normal salicylate, normal acetaminophen, and a normal head CT. The tentative diagnosis of PANDAS was made, and the patient was thereafter treated with antipsychotics, antibiotics, tonsillectomy, and IVIG which resulted in remissions and relapses of his neuropsychiatric symptoms. Once he reached age 27, he received a trial of therapeutic plasma exchange (TPE), rituximab, and ceftriaxone. This eventually resulted in sustained benefit and minimal fluctuations of his clinical symptoms. Our report is noteworthy in three ways.One, he is a 27-year-old adult with PANDAS.Two, he improved after TPE, rituximab, and ceftriaxone. Our literature search yielded minimal data on the use of plasmapheresis for nonteenage adults with PANDAS. Three, he had unusual symptoms of PANDAS, as the typical OCD and/or tic-like symptoms were not observed.

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Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections

Journal of Medical - Clinical Research & Reviews ◽

10.33425/2639-944x.1229 ◽

2021 ◽

Vol 5 (8) ◽

Author(s):

Mohammad O AL fattani ◽

Asmaa M Al Refaie ◽

Shuruq Hassan Alsulami ◽

Najia Al Hojaili

Keyword(s):

Febrile Illness ◽

Neuropsychiatric Disorders ◽

Age Group ◽

Pediatric Age ◽

Streptococcal Infections ◽

Obsessive Compulsive ◽

Tic Disorder ◽

Compulsive Disorder ◽

Pediatric Age Group ◽

Suicidal Attempts

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is a rear disease in pediatric age group which constitute a unique manifestation associated with obsessive-compulsive disorder (OCD) and tic disorder in children. However, this diagnosis has carried a lot of controversies, related mainly to its management. Here we are presenting one case of PANDAS syndrome who was treated successfully with full coarse of antibiotic for 10 days, where all his symptoms disappeared completely in subsequent days including suicidal attempts. PANDAS should be considered in children with neuropsychiatric disorders (tics, obsessive behavior etc.) especially if symptoms associated within a period of infection such as febrile illness or sore throats.

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No Neurochemical Evidence of Neuronal Injury or Glial Activation in Children with Paediatric Acute-onset Neuropsychiatric Syndrome

10.21203/rs.3.rs-66815/v1 ◽

2020 ◽

Author(s):

Mats Johnson ◽

Elisabeth Fernell ◽

Christopher Gillberg ◽

Anders Fasth ◽

Kaj Blennow ◽

...

Keyword(s):

Diagnostic Criteria ◽

Neuropsychiatric Symptoms ◽

Neuronal Injury ◽

Acute Onset ◽

University Hospital ◽

Serum Concentrations ◽

Obsessive Compulsive ◽

Neurofilament Light ◽

Compulsive Disorder ◽

Autoimmune Aetiology

Abstract Background Paediatric Acute-onset Neuropsychiatric Syndrome (PANS) is characterized by an acute onset of obsessive compulsive disorder, combined with at least two other neuropsychiatric symptoms with similarly acute onset. Diagnostic criteria also require that no specific medical aetiology is identified. Although there are no verified aetiological biomarkers, PANS is assumed to be a neuroinflammatory disorder with a possible autoimmune aetiology. Neurochemical markers such as neurofilament light (NfL, a neuronal injury marker) and glial fibrillary acidic protein (GFAP, an astrocytic activation marker) have not been published for this patient group.Methods Blood samples from 17 children meeting diagnostic criteria for PANS, after assessment at the Child Neuropsychiatry Centre, Sahlgrenska University Hospital, Gothenburg, Sweden, were analysed for serum concentrations of NfL and GFAP. Ten age-matched children without any neurological or psychiatric disorder served as a comparison group. Results No difference was found in mean NfL and mean GFAP serum concentrations between children with PANS and controls. NfL and GFAP concentrations did not correlate with disease duration between PANS onset and time of blood tests. Conclusion Neuronal injury and astrocyte activation do not seem to be a major event in PANS. The study group was small, and even if findings may be reassuring for parents and patients, they should be interpreted with caution and verified in larger cohort and possibly with other markers in both serum and CSF.

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The PANDAS Subgroup: Recognition and Treatment

CNS Spectrums ◽

10.1017/s1092852900021799 ◽

2001 ◽

Vol 6 (5) ◽

pp. 419-426 ◽

Cited By ~ 22

Author(s):

Susan E. Swedo ◽

Marjorie Garvey ◽

Lisa Snider ◽

Charlotte Hamilton ◽

Henrietta L. Leonard

Keyword(s):

Controlled Trial ◽

Neuropsychiatric Symptoms ◽

Sudden Onset ◽

Tic Disorders ◽

Temporal Association ◽

Obsessive Compulsive ◽

Compulsive Disorder ◽

Treatment And Prevention ◽

Group A ◽

Immunomodulatory Therapies

AbstractA subgroup of patients with childhood-onset obsessive-compulsive disorder (OCD) has been identified who share a common clinical course characterized by dramatic symptom exacerbations following Group A beta-hemolytic streptococcal (GABHS) infections. The term PANDAS has been applied to the subgroup, to indicate the postulated etiology of their symptoms: Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections. Five clinical characteristics define the PANDAS subgroup: presence of OCD and/or tic disorder, prepubertal symptom onset, sudden onset or abrupt exacerbations (sawtooth course), association with neurological abnormalities (presence of adventitious movements or motoric hyperactivity during exacerbations), and temporal association between symptom exacerbations and GABHS infections. Post-streptococcal symptom exacerbations are typically quite dramatic, with patients reporting that their symptoms “…came on overnight” or “…appeared all of a sudden a few days after I had a sore throat.” The post-streptococcal inflammatory nature of the neuropsychiatric symptoms provides novel opportunities for treatment and prevention, including immunomodulatory therapies such as therapeutic plasma exchange (TPE) and intravenous immunoglobulin (IVIG). A recently completed placebo-controlled trial revealed that both IVIG and TPE were effective in reducing neuropsychiatric symptom severity (40% to 55% reductions, respectively) for a group of severely ill children with OCD and/or tic disorders. Further research is required to determine why the treatments are helpful, as well as to ascertain whether or not antibiotic prophylaxis can help prevent post-streptococcal symptom exacerbations.

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Vitamin D Deficiency in Obsessive–Compulsive Disorder Patients with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections: A Case Control Study

Nöro Psikiyatri Arşivi ◽

10.5152/npa.2015.8763 ◽

2016 ◽

Vol 53 (1) ◽

pp. 33-37 ◽

Cited By ~ 9

Author(s):

Gonca Celik ◽

Didem Tas ◽

Aysegul Tahiroglu ◽

Ayse Avci ◽

Bilgin Yuksel ◽

...

Keyword(s):

Vitamin D ◽

Vitamin D Deficiency ◽

Obsessive Compulsive Disorder ◽

Case Control Study ◽

Neuropsychiatric Disorders ◽

Case Control ◽

Streptococcal Infections ◽

Obsessive Compulsive ◽

Compulsive Disorder ◽

Control Study

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Pediatric Acute-onset Neuropsychiatric Syndrome and Mycoplasma Pneumoniae Infection: A Case Report Analysis with a Metabolomics Approach

Current Pediatric Reviews ◽

10.2174/1573396315666191022102925 ◽

2020 ◽

Vol 16 (3) ◽

pp. 183-193 ◽

Cited By ~ 3

Author(s):

Cristina Piras ◽

Roberta Pintus ◽

Dario Pruna ◽

Angelica Dessì ◽

Luigi Atzori ◽

...

Keyword(s):

Case Report ◽

Protein Biosynthesis ◽

Neuropsychiatric Symptoms ◽

Acute Onset ◽

Point Of View ◽

Obsessive Compulsive ◽

Compulsive Disorder ◽

Before And After ◽

New Biomarkers ◽

The Individual

: Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) is a clinical condition characterized by a sudden and dramatic obsessive-compulsive disorder with a suggested post-infectious immune-mediated etiology. This condition is accompanied by an extensive series of relatively serious neuropsychiatric symptoms. The diagnosis of PANS is made by "exclusion", as the individual PANS symptoms overlap with a multiplicity of psychiatric disorders with the onset in childhood. A number of researchers accumulated evidence to support the hypothesis that PANS was closely associated with a number of infections. : In the last decade, metabolomics played an essential role in improving the knowledge of complex biological systems and identifying potential new biomarkers as indicators of pathological progressions or pharmacologic responses to therapy. The metabolome is considered the most predictive phenotype, capable of recognizing epigenetic differences, reflecting more closely the clinical reality at any given moment and thus providing extremely dynamic data. In the present work, the most recent hypothesis and suggested mechanisms of this condition are reviewed and the case of a 10 - year-old girl with PANS is described, before and after clarithromycin treatment. The main results of this case report are discussed from a metabolomics point of view. The alteration of several metabolic pathways concerning the microbial activity highlights the possible role of the microbiome in the development of PANS. Furthermore, different metabolic perturbations at the level of protein biosynthesis, energy and amino acid metabolisms are observed and discussed. Based on our observations, it is believed that metabolomics is a promising technology to unravel the mysteries of PANS in the near future.

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Oxidative stress and gut-derived lipopolysaccharides in children affected by Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections.

10.21203/rs.2.19372/v5 ◽

2020 ◽

Author(s):

Lorenzo Loffredo ◽

Alberto Spalice ◽

Francesca Salvatori ◽

De Castro Giovanna ◽

Cristiana Alessia Guido ◽

...

Keyword(s):

Linear Regression Analysis ◽

Prostaglandin F2α ◽

Neuropsychiatric Disorders ◽

Serum Levels ◽

Multiple Linear Regression Analysis ◽

Acute Onset ◽

Tic Disorders ◽

Bivariate Analysis ◽

Streptococcal Infections ◽

Obsessive Compulsive

Abstract Background: paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections syndrome (PANDAS) identifies patients with acute onset of obsessive-compulsive and tic disorders. The objective of this study was to evaluate serum NOX2 levels, as well as 8-iso-prostaglandin F2α (8-iso-PGF2α) and lipopolysaccharide (LPS) of PANDAS patients. Methods: in this study we wanted to compare serum levels of soluble NOX2-dp (sNOX-2-dp), iso-PGF2α and LPS in 60 consecutive subjects, including 30 children affected by PANDAS and 30 controls (CT) matched for age and gender. Serum zonulin was used as intestinal permeability assay.Results: compared with CT, PANDAS children had increased serum levels of sNOX-2-dp, 8-iso-PGF2α and LPS. Bivariate analysis showed that serum sNOX2-dp was signiﬁcantly correlated with LPS (Rs=0.359; p=0.005), zonulin (Rs=0.444; p<0.001) and 8-iso-PGF2α (Rs=0.704; p<0.001). Serum LPS signiﬁcantly correlated with zonulin (Rs=0.610; p<0.001), and 8-iso-PGF2α (Rs=0.591; p=0.001). Finally, a multiple linear regression analysis showed that serum 8-iso-PGF2α and zonulin were the only independent variables associated with sNOX2-dp (R2=68%).Conclusion: this study shows that children affected by PANDAS have high circulating levels of sNOX2-dp, isoprostanes and of LPS that could be involved in the process of neuroinflammation.

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