SARS-CoV-2 related paediatric acute-onset neuropsychiatric syndrome.
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ABSTRACT:
SUBMITTER: Pavone P
PROVIDER: S-EPMC8096321 | biostudies-literature | 2021 Jun
REPOSITORIES: biostudies-literature
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SARS-CoV-2 related paediatric acute-onset neuropsychiatric syndrome.
Pavone Piero P Ceccarelli Manuela M Marino Silvia S Caruso Daniela D Falsaperla Raffaele R Berretta Massimiliano M Rullo Emmanuele Venanzi EV Nunnari Giuseppe G
The Lancet. Child & adolescent health 20210504 6
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Project description:IntroductionPaediatric acute-onset neuropsychiatric syndrome (PANS) is a neuroimmune condition characterised by episodic exacerbations and remissions. This study investigates the impact of PANS on children's occupational performance during these phases, using the Vineland Adaptive Behaviour Scale (VABS). Understanding these variations is crucial for developing tailored interventions and managing the condition effectively.MethodsA two-period bidirectional case-crossover design was employed to assess occupational performance in children with PANS at exacerbation (T-E) and remission (T-R) phases. Parents of children aged 4.6-13.1 years with PANS were recruited globally, with data collected via online surveys and monthly updates. The VABS evaluated occupational performance, with analysis using linear mixed models and Least Squares Means (LSMeans) for accurate representation.ResultsData from 27 participants showed significantly lower scores in the Adaptive Behaviour Composite and socialisation domain of the VABS at T-E compared with T-R, indicating a decline in occupational performance and social skills during exacerbations. During remission, participants demonstrated typical performance across all domains.ConclusionThe study highlights a marked decline in occupational performance and socialisation during PANS exacerbations, with improvements to typical levels during remission. These findings emphasise the need for targeted interventions to address occupational and social challenges in children with PANS during exacerbations, underscoring the episodic nature of the condition and the risk of misinterpreting behaviours if assessed during exacerbations. Multidisciplinary allied health support may benefit children with PANS.Consumer and community involvementAlthough there was no direct consumer and community involvement, two researchers on our team have family members with PANS. Their personal experiences provided vital insights into the challenges faced by children with PANS, deeply influencing our study's design, focus, and interpretation, ensuring it reflects the realities of those affected.Plain language summaryOur study looked at how PANS affects the things children do in their daily lives. We asked parents of children ages 4.6-13.1 years old to complete surveys measuring this at two times: once during a symptom flare-up and once during a period of wellness. Our results showed that during symptom flare-ups, children's social skills and general ability to do daily tasks were much lower compared with periods of wellness. When children were well, their abilities were similar to those of typically developing children of the same age. This highlights the need for occupational therapy during flare-ups to help improve the lives of children with PANS.
| S-EPMC11650011 | biostudies-literature
Project description:Background: Individuals with generalised joint hypermobility (GJH, present in 10-20% of the general population) are at increased risk of being diagnosed with a range of psychiatric and rheumatological conditions. It is unknown whether Paediatric acute-onset neuropsychiatric syndrome (PANS), characterised by childhood onset obsessive-compulsive disorder or restricted eating and typically associated with several comorbid neuropsychiatric symptoms, is associated with GJH. It is also unknown whether extensive psychiatric comorbidity is associated with GJH. Method: This is a case-control study including 105 participants. We compared three groups: Individuals with PANS, individuals with other mental disorders and healthy controls. Joint mobility was assessed with the Beighton scoring system, psychiatric comorbidity with the M.I.N.I. or MINI-KID interview and symptoms of PANS with the PsychoNeuroInflammatory related Signs and Symptoms Inventory (PNISSI). Results: Hypermobility was similar across groups, and high rates of psychiatric comorbidity was not associated with higher Beighton scores. Conclusion: Although GJH is associated with several psychiatric conditions, such as ADHD and anxiety, this does not seem to be the case for PANS according to this preliminary study.
| S-EPMC8678126 | biostudies-literature
Project description:We report the case of a 12-years-old patient who subacutely developed a positive and negative myoclonus of limbs and face, drowsiness and memory deficits after getting infected by SARS-CoV-2. On admission, nasopharyngeal swab for SARS-CoV-2, brain and spinal MRI with and without contrast, EEG, chest X-ray and abdominal ultrasound were negative. CSF physical-chemical examination, culture, PCR testing for SARS-CoV-2 and other pathogens, and oligoclonal IgG bands were negative as well. A full panel blood test, including clotting, autoimmunity and paraneoplastic blood studies, did not show any alteration. The neuropsychological examination showed an impairment in memory, visual-motor coordination, inductive reasoning skills, attention, and concentration. The patient was first treated with clonazepam and then with intravenous methylprednisolone for five days, with poor response. For this reason, he 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.
| S-EPMC8739821 | biostudies-literature
Project description:BackgroundPaediatric acute-onset neuropsychiatric syndrome (PANS), an umbrella term that includes PANDAS (paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) is suggested to be a psychiatric disorder of autoimmune aetiology. PANS is characterised by an acute onset of obsessive-compulsive disorder or restricted eating with multiple comorbid symptoms. The specificity of the PANS criteria is not fully understood.AimsTo describe a cohort of patients with PANS and to determine if PANS features relating to symptoms, onset and course are more common in PANS than in other psychiatric conditions.MethodA case-control study comparing patients with interview-confirmed PANS with patients with suspected PANS and patients with a psychiatric condition but with no suspicion of PANS. Validated and non-validated measures of symptoms, onset and episodic course were used.ResultsIllness in patients with interview-confirmed PANS featured an episodic course and multiple symptoms present at onset compared with the psychiatric controls. However, individuals with interview-confirmed PANS did not present a specific symptom profile.ConclusionsPANS may be a distinct clinical entity featuring an acute onset, an episodic course and multiple symptoms at onset.Declaration of interestNone.
| S-EPMC6401534 | biostudies-literature
Project description:This SuperSeries is composed of the SubSeries listed below.
2025-09-07 | GSE278680 | GEO
Project description:BackgroundTreatment with intravenous immunoglobulin (IVIG) in children with Paediatric Acute-onset Neuropsychiatric Syndrome (PANS) has for many years been used on clinical indications, but the research evidence for its efficacy is insufficient.MethodsOpen-label prospective in-depth trial including ten children (median age 10.3 years) with PANS, who received IVIG treatment 2 g/kg monthly for three months. Primary outcomes were changes in symptom severity and impairment from baseline to first and second follow-up visits one month after first and one month after third treatment, using three investigator-rated scales: Paediatric Acute Neuropsychiatric Symptom (PANS) scale, Clinical Global Impression - Severity and Improvement (CGI-S and CGI-I) scales. Secondary outcomes reported here were changes in Children's Yale-Brown Obsessive Compulsive Scale (CY-BOCS) scores, and side effects.ResultsAll ten children received three treatments at one-month intervals according to study plan. From baseline to second follow-up marked reductions were seen in mean total PANS scale scores (p = .005), and CGI-S scores (p = .004). CGI-I ratings showed much to very much global improvement (mean CGI-I 1.8). Nine children had clinical response defined as > 30% reduction in PANS Scale scores. Improvements were also noted for CY-BOCS scores (p = .005), and in school attendance. Three children suffered moderate to severe temporary side effects after the first treatment, and the remaining seven had mild to moderate side effects. Side effects were much less severe after second and third treatments.ConclusionsConsiderable and pervasive improvements in symptoms and clinical impairments were seen in these ten children after three monthly IVIG treatments. Moderate to severe transient side effects occurred in three cases.Trial registrationEudraCT no. 2019-004758-27, Clinicaltrials.gov no. NCT04609761, 05/10/2020.
| S-EPMC9357317 | biostudies-literature
Project description:Paediatric Acute Neuropsychiatric Syndrome (PANS) is characterised by abrupt onset obsessive compulsive disorder and regression in neurodevelopmental skills, triggered by infection or stress. Whether PANS is a distinct entity or part of a neurodevelopmental spectrum is uncertain, and its pathophysiology unclear. We show that children with PANS and other non-PANS neurodevelopmental disorders have higher reported early childhood infections and loss of previously acquired developmental skills compared to neurotypical controls. Children with PANS have normal routine immune testing, however bulk RNA-sequencing revealed upregulated pathways in ribosomal biogenesis and RNA methyltransferases, and downregulated pathways in diverse cellular functions such as mitochondrial activity, cell signalling, endocytosis, and immune responses. Single-cell RNA-sequencing confirmed these findings but showed heterogeneity across immune cell types. Toll-like receptor stimulation assay using peripheral blood mononuclear cells revealed reduced TNF and interleukin-6 responses in PANS patients compared to controls. RNA sequencing before and after intravenous immunoglobulin treatment in PANS patients revealed reversal of the dysregulated ribosomal, epigenetic, and cell signaling pathways. Given the central role of the immune system in synaptic pruning and neurodevelopment, these insights provide rationale for novel epigenetic and immune modulating therapies to optimize neurodevelopmental trajectories and minimize neuropsychiatric impairment.
2025-09-11 | GSE278679 | GEO
Project description:Paediatric Acute Neuropsychiatric Syndrome (PANS) is characterised by abrupt onset obsessive compulsive disorder and regression in neurodevelopmental skills, triggered by infection or stress. Whether PANS is a distinct entity or part of a neurodevelopmental spectrum is uncertain, and its pathophysiology unclear. We show that children with PANS and other non-PANS neurodevelopmental disorders have higher reported early childhood infections and loss of previously acquired developmental skills compared to neurotypical controls. Children with PANS have normal routine immune testing, however bulk RNA-sequencing revealed upregulated pathways in ribosomal biogenesis and RNA methyltransferases, and downregulated pathways in diverse cellular functions such as mitochondrial activity, cell signalling, endocytosis, and immune responses. Single-cell RNA-sequencing confirmed these findings but showed heterogeneity across immune cell types. Toll-like receptor stimulation assay using peripheral blood mononuclear cells revealed reduced TNF and interleukin-6 responses in PANS patients compared to controls. RNA sequencing before and after intravenous immunoglobulin treatment in PANS patients revealed reversal of the dysregulated ribosomal, epigenetic, and cell signaling pathways. Given the central role of the immune system in synaptic pruning and neurodevelopment, these insights provide rationale for novel epigenetic and immune modulating therapies to optimize neurodevelopmental trajectories and minimize neuropsychiatric impairment.
2025-09-11 | GSE278678 | GEO
Project description:Objectives: Pediatric acute-onset neuropsychiatric syndrome (PANS) is a descriptive clinical entity defined by the abrupt onset of psychiatric and somatic symptoms leading to significant loss of function. Data on well-characterized PANS patients are limited, biomarkers have yet to be identified, and a solid evidence base to guide treatment is lacking. In this study, we present our experience of a systematic evaluation of the first 45 patients included in a Swedish cohort. Methods: During the period 2014-2018, our clinic received 100 referrals regarding suspected PANS. All patients underwent a standardized psychiatric/medical evaluation by a child/adolescent psychiatrist and a clinical psychologist or a nurse. Those with severe symptoms were also assessed by a pediatric neurologist and a pediatric rheumatologist. Laboratory tests were obtained at different time points in an attempt to capture an active disease state. Results: Of the 100 referrals, 45 met strict PANS criteria and consented to participate in a long-term follow-up study. The median age at intake was 7.2 years (range 3.0-13.1) and 56% were male. Ninety-three percent fulfilled both criteria for acute/atypical onset of PANS symptoms and having had an infection in relation to onset. Sixteen percent had an onset of an autoimmune or inflammatory disorder in temporal relation to the onset of PANS-related symptoms. The most common onset symptoms were obsessive-compulsive disorder (89%), anxiety (78%), and emotional lability (71%). Twenty-four percent had a preexisting autoimmune disease (AD) and 18% a preexisting psychiatric/neuropsychiatric diagnosis. Sixty-four percent of biological relatives had at least one psychiatric disorder and 76% at least one AD or inflammatory disorder. Complement activation (37%), leukopenia (20%), positive antinuclear antibodies (17%), and elevated thyroid antibodies (11%) were the most common laboratory findings. Conclusions: In our PANS cohort, there was a strong indication of an association with AD. Further work is needed to establish whether any of the potential biomarkers identified will be clinically useful. Long-term follow-up of these patients using the Swedish national registers will enable a deeper understanding of the course of this patient group.
| S-EPMC6786340 | biostudies-literature
Project description:IntroductionPANS is a controversial clinical entity, consisting of a complex constellation of psychiatric symptoms, adventitious changes, and expression of various serological alterations, likely sustained by an autoimmune/inflammatory disease. Detection of novel biomarkers of PANS is highly desirable for both diagnostic and therapeutic management of affected patients. Analysis of metabolites has proven useful in detecting biomarkers for other neuroimmune-psychiatric diseases. Here, we utilize the metabolomics approach to determine whether it is possible to define a specific metabolic pattern in patients affected by PANS compared to healthy subjects.DesignThis observational case-control study tested consecutive patients referred for PANS between June 2019 to May 2020. A PANS diagnosis was confirmed according to the PANS working criteria (National Institute of Mental Health [NIMH], 2010). Healthy age and sex-matched subjects were recruited as controls.MethodsThirty-four outpatients referred for PANS (mean age 9.5 years; SD 2.9, 71% male) and 25 neurotypical subjects matched for age and gender, were subjected to metabolite analysis. Serum samples were obtained from each participant and were analyzed using Nuclear Magnetic Resonance (NMR) spectroscopy. Subsequently, multivariate and univariate statistical analyses and Receiver Operator Curves (ROC) were performed.ResultsSeparation of the samples, in line with the presence of PANS diagnosis, was observed by applying a supervised model (R2X = 0.44, R2Y = 0.54, Q2 = 0.44, p-value < 0.0001). The significantly altered variables were 2-Hydroxybutyrate, glycine, glutamine, histidine, tryptophan. Pathway analysis indicated that phenylalanine, tyrosine, and tryptophan metabolism, as well as glutamine and glutamate metabolism, exhibited the largest deviations from neurotypical controls.ConclusionWe found a unique plasma metabolic profile in PANS patients, significantly differing from that of healthy children, that suggests the involvement of specific patterns of neurotransmission (tryptophan, glycine, histamine/histidine) as well as a more general state of neuroinflammation and oxidative stress (glutamine, 2-Hydroxybutyrate, and tryptophan-kynurenine pathway) in the disorder. This metabolomics study offers new insights into biological mechanisms underpinning the disorder and supports research of other potential biomarkers implicated in PANS.
| S-EPMC8194687 | biostudies-literature