Clinical features of paediatric acute-onset neuropsychiatric syndrome: findings from a case- control study.
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ABSTRACT: Background
Paediatric 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.Method
A 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.Results
Illness 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.Conclusions
PANS may be a distinct clinical entity featuring an acute onset, an episodic course and multiple symptoms at onset.Declaration of interestNone.
SUBMITTER: Hesselmark E
PROVIDER: S-EPMC6401534 | biostudies-literature | 2019 Mar
REPOSITORIES: biostudies-literature
Publications
Clinical features of paediatric acute-onset neuropsychiatric syndrome: findings from a case- control study.
Hesselmark Eva E Bejerot Susanne S
BJPsych open 20190301 2
<h4>Background</h4>Paediatric 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 wi ...[more]
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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
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| S-EPMC8096321 | biostudies-literature
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: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: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: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
Project description:Objectives: Pediatric acute-onset neuropsychiatric syndrome (PANS) is a clinical diagnosis in children who have an acute manifestation of varied neuropsychiatric symptoms, including obsessive compulsive disorder, eating disorders, tics, anxiety, irritability, and problems with attention/concentration. PANS may develop as a result of a postinfectious syndrome and may represent a new form of postinfectious autoimmunity. To test the hypothesis that multiple, consecutive infusions of intravenous immunoglobulin (IVIG) for PANS can be efficacious, a multisite, open-label study was designed. Methods: The primary endpoint was evaluation of the efficacy of IVIG [Octagam 5%] in PANS over a period of 6 months (six infusions) based on mean changes in psychological evaluation scores using 6 different assessments, including the Children's Yale-Brown Obsessive Compulsive Scale (CY-BOCS), Clinical Global Impression of Severity, and the Parent-Rated Pediatric Acute Neuropsychiatric Symptom Scale (PANS Scale). Results: The final cohort consisted of 21 subjects (7 per site) with moderate to severe PANS. The mean age was 10.86 years (range: 4-16 years). Results demonstrated statistically significant reductions in symptoms from baseline to end of treatment in all six assessments measured. CY-BOCS results demonstrated statistically significant reductions in obsessive compulsive symptoms (p < 0.0001), resulting in >50% improvement sustained for at least 8 weeks after the final infusion and up to 46 weeks in a subset of subjects. Conclusions: In PANS, which may be associated with an underlying immune dysregulation, sequential infusions of IVIG [Octagam 5%] successfully ameliorated psychological symptoms and dysfunction, with sustained benefits for at least 8 weeks, and up to 46 weeks in a subset of subjects. In addition, baseline immune and autoimmune profiles demonstrated significant elevations in a majority of subjects, which requires further evaluation, characterization, and study to clarify the potential immune dysfunction by which PANS manifests and progresses.
| S-EPMC7984935 | biostudies-literature
Project description:IntroductionPediatric Acute-Onset Neuropsychiatric Syndrome (PANS) is characterized by abrupt onset of obsessive-compulsive disorder or eating restriction along with the abrupt onset of other co-occurring symptoms (tics, behavioral and cognitive regression, etc.). PANS is thought to be a post-infectious immunopsychiatric disorder, although as with most post-infectious disorders, it is challenging to establish a causal relationship with proposed infectious triggers. Intravenous immunoglobulin (IVIG) can modulate inflammation and support the elimination of infection and has been used for treatment of many post-infectious inflammatory disorders and autoimmune conditions. The aim of the study is to explore the pro-inflammatory state in PANS before and after administration of IVIG.MethodsChildren with moderate-to-severe PANS received six infusions of IVIG (Octagam 5%, Octapharma) every 3 weeks with post treatment follow-up. Blood samples and psychiatric measures were obtained at Visits 1 (pre-treatment), 7 and 8 (4 and 11 weeks after last infusion, respectively). Myeloid cell activation was assessed via flow cytometry.ResultsAll ten patients included in the study were male, White, with mean age 12.4 years (range 6-16). Statistically significant improvements following IVIG treatment were demonstrated in all psychometric assessments and parent questionnaires including CY-BOCS (obsessive compulsive scale), YGTSS (tic scale) and a parent PANS rating scale (for all scales p<0.001). The fraction of pro-inflammatory monocytes and dendritic cells decreased from pre-IVIG treatment levels. The proportional reductions were not compensated by increases in total white blood cells; pro-inflammatory monocytes post-IVIG were decreased as a proportion of CD14+ myeloid cells and in absolute number.ConclusionsThe results of this study suggest that active PANS is associated with a pro-inflammatory state. This pro-inflammatory profile and psychometric scores improved following IVIG treatment. Future work will aim to further elucidate the roles of innate and adaptive immune responses in PANS and the regulatory mechanism(s) of IVIG in PANS treatment.
| S-EPMC11484259 | biostudies-literature
Project description:ImportanceStudies of brain imaging and movements during rapid eye movement sleep indicate basal ganglia involvement in pediatric acute-onset neuropsychiatric syndrome (PANS). Characterizing neurological findings that commonly present among patients with PANS could improve diagnostic accuracy.ObjectivesTo evaluate the prevalence of neurological soft signs (NSSs) that may be associated with basal ganglia dysfunction among youths presenting with PANS and assess whether clinical characteristics of PANS correlate with NSSs that may be associated with basal ganglia dysfunction.Design, setting, and participantsThis cohort study included 135 new patients who met strict PANS criteria and were evaluated at the Stanford Children's Immune Behavioral Health Clinic between November 1, 2014, and March 1, 2020. Data on these patients were retrospectively reviewed between December 13, 2020, and September 25, 2023. Sixteen patients were excluded because they had no neurological examination within the first 3 visits and within 3 months of clinical presentation. Statistical analysis was conducted between September 26, 2023, and November 22, 2024.Main outcomes and measuresThe following NSSs that may be associated with basal ganglia dysfunction were recorded from medical record review: (1) glabellar tap reflex, (2) tongue movements, (3) milkmaid's grip, (4) choreiform movements, (5) spooning, and (6) overflow movements. Data from prospectively collected symptoms and impairment scales (Global Impairment Score [GIS; score range, 1-100, with higher scores indicating greater impairment] and Caregiver Burden Inventory [score range, 0-96, with higher scores indicating greater caregiver burden]) were included.ResultsThe study included 119 patients; the mean (SD) age at PANS onset was 8.2 (3.6) years, the mean (SD) age at initial presentation was 10.4 (3.6) years, and 66 (55.5%) were boys. At least 1 NSS that may be associated with basal ganglia dysfunction was observed in 95 patients (79.8%); the mean (SD) number of NSSs was 2.1 (1.6). Patients with 4 or more NSSs had higher GISs (mean [SD] score, 56.0 [22.6] vs 40.6 [26.7]; P = .05) and more symptoms (mean [SD] number, 15.1 [4.9] vs 11.5 [4.2]; P = .008) than patients with 0 NSSs. There was no significant difference in age at visit or in Caregiver Burden Inventory score. On Poisson and linear regression, the number of NSSs was associated with global impairment, with 1 more sign increasing the GIS by 2.86 (95% CI, 0.09-5.62; P = .04), and with the number of symptoms, with 1 more sign increasing the number of symptoms by 5% (1.05; 95% CI, 1.02-1.08; P = .002), but not with age or duration of PANS at presentation.Conclusions and relevanceThis cohort study of patients with PANS found a high prevalence of NSSs that may be associated with basal ganglia dysfunction and an association between these NSSs and disease severity that was not associated with younger age. PANS may have a unique profile, suggesting that targeted neurological examinations may support PANS diagnosis.
| S-EPMC11889471 | biostudies-literature
Project description:Background: The prevalence and impact of allergic and immune-mediated food disorders in pediatric acute-onset neuropsychiatric syndrome (PANS) are mostly unknown. Objective: We sought to explore the prevalence of atopic dermatitis (AD), asthma, allergic rhinitis (AR), IgE-mediated food allergies (FAs), and other immune-mediated food disorders requiring food avoidance in patients with PANS. In addition, to further understand the extent of food restriction in this population, we investigated the empiric use of dietary measures to improve PANS symptoms. Methods: Pediatric patients in a PANS Clinic and Research Program were given surveys regarding their caregiver burdens, allergic and food-related medical history, and whether food elimination resulted in perception of improvement of PANS symptoms. A review of health records was conducted to confirm that all responses in the survey were concordant with documentation of each patient's medical chart. Results: Sixty-nine (ages 4-20 years) of 80 subjects who fulfilled PANS criteria completed the surveys. Thirteen (18.8%) had AD, 11 (15.9%) asthma, 33 (47.8%) AR, 11 (15.9%) FA, 1 (1.4%) eosinophilic gastrointestinal disorders, 1 (1.4%) food protein-induced enterocolitis syndrome, 3 (4.3%) milk protein-induced proctocolitis syndrome, and 3 (4.3%) celiac disease. Thirty subjects (43.5%) avoided foods due to PANS; elimination of gluten and dairy was most common and was associated with perceived improvement of PANS symptoms (by parents). This perceived improvement was not confirmed with objective data. Conclusions: The prevalence of allergic and immune-mediated food disorders in PANS is similar to the general population as reported in the literature, with the exception of AR that appears to be more prevalent in our PANS cohort. More research will be required to establish whether diet or allergies influence PANS symptoms.
| S-EPMC6154445 | biostudies-literature