Palatal Petechiae in the Absence of Group A<i>Streptococcus</i>in Pediatric Patients with Acute-Onset Neuropsychiatric Deterioration: A Cohort Study

Mahony, Talia; Sidell, Douglas; Gans, Hayley A.; Cooperstock, Michael; Brown, Kayla; Cheung, Joanne M; Farhadian, Bahare; Gustafson, Melissa; Thienemann, Margo; Frankovich, Jennifer

doi:10.1089/cap.2016.0153

Cited by 5 publications

(5 citation statements)

References 30 publications

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“…That such mechanisms may indeed be critical in PANS is suggested by the efficacy of anti-inflammatory therapies, most commonly ibuprofen and steroids, in attenuating PANS symptoms among patients in this study. Although treatment with other anti-inflammatories, such as naproxen and celecoxib, appears to be rare in the PANS population, the positive reports collected in this survey suggest that this anti-inflammatory treatment strategy may deserve further study (Mahony et al 2017 ).…”

Section: Discussionmentioning

confidence: 90%

Treatment of Pediatric Acute-Onset Neuropsychiatric Disorder in a Large Survey Population

Calaprice

Tona

Murphy

2018

Journal of Child and Adolescent Psychopharmacology

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Objective: The goal of this study was to investigate treatment histories and outcomes in a large community sample of youth with Pediatric Acute-onset Neuropsychiatric Syndrome (PANS), and, where appropriate, to examine the impact of immune deficiency on treatment outcomes.Methods: A comprehensive internet-based survey was completed by parents or guardians of youth who had received physician diagnoses of PANS, or by young adults (age 18+) who had themselves been diagnosed by a physician (N = 698). Data regarding the treatment histories of these patients, including the variety of medical and psychological treatments employed and the caregiver- or self-reported response to each, are presented.Results: The PANS patients in this study had commonly been treated with antibiotic (N = 675), anti-inflammatory (N = 437), and/or psychotropic therapy (N = 378). Response to antibiotic treatment was best when treatment was relatively aggressive, with broad-spectrum antibiotics and courses of >30 days generally producing the best results (i.e., up to 52% of patients achieving a “very effective” response). For immune-deficient patients (caregiver-reported laboratory studies below normal limits; N = 108), use of broad-spectrum antibiotics appeared to be particularly desirable. Anti-inflammatory therapies, including over-the-counter medications such as ibuprofen, were at least “somewhat effective” for most patients. Intravenous immunoglobulin (IVIG) had been used to treat PANS in 193 (28%) of the patients and was at least “somewhat effective” for 89%, although for 18% of these, the effect was not sustained. The highest rate of sustained response to IVIG treatment was seen in immune-deficient patients who received doses of at least 0.8 g/kg IVIG on a regular basis. Psychotropic medications, most commonly SSRIs (38% reported a trial), were commonly employed, but were often ineffective (e.g., 44% found SSRIs “somewhat” to “very effective”). Many patients (N = 473) had received some form of psychotherapy with some benefit, with cognitive behavioral therapy found to be at least somewhat effective in a majority of those treated with this modality.Conclusion: Among the PANS patients represented in this study, relatively aggressive treatment courses targeted at eradicating infection and modulating the inflammatory response appeared to provide the best caregiver-reported therapeutic results, and to be generally well tolerated. Given its relative efficacy and tolerability, treatment targeting the inflammatory response may represent an underutilized approach in this population. The results of this study should be considered in light of the limitations inherent in a self-selected and administered online survey.

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Section: Discussionmentioning

confidence: 90%

Treatment of Pediatric Acute-Onset Neuropsychiatric Disorder in a Large Survey Population

Calaprice

Tona

Murphy

2018

Journal of Child and Adolescent Psychopharmacology

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“…Slightly fewer than half of the patients, 46%, exhibited ear, nose, and throat (ENT) infection signs at onset/first assessment. Nine percent had palatal petechiae as a suspected sign of streptococcal infection (Mahony et al 2017b). Skin abnormalities, including dermatographia, livedo, rashes, and/or eczema, were present in 61% of patients.…”

Section: Resultsmentioning

confidence: 99%

“…Other diseases of known etiology, but with a similar clinical picture, such as Sydenham's chorea, systemic lupus erythematosus, or other inflammatory encephalitides, such as anti-NMDA receptor encephalitis, need to be excluded (Dalmau et al 2008; Dale and Brilot 2012; Hacohen et al 2013; Ramanathan 2014). While the etiology is unknown, infectious agents such as group A streptococci (GAS), mycoplasma, and Epstein–Barr virus as well as AD and inflammatory disorders have been described as contributing to the pathogenic mechanisms and potential triggers for the constellation of symptoms that constitute PANS (Kurlan et al 2008; Leckman et al 2011; Brimberg et al 2012; Cutforth et al 2016; Mahony et al 2017a, 2017b).…”

Section: Introductionmentioning

confidence: 99%

Establishing a Pediatric Acute-Onset Neuropsychiatric Syndrome Clinic: Baseline Clinical Features of the Pediatric Acute-Onset Neuropsychiatric Syndrome Cohort at Karolinska Institutet

Gromark

Harris

Wickström

et al. 2019

Journal of Child and Adolescent Psychopharmacology

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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.

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“…Nonetheless, it is still hard to reach a homogeneous, validated diagnostic pathway because of the difference between the conceptual frames and the diagnostic tools used by diverse groups. A recent review 105 suggested that, taken individually, no single biomarker is specific for the diagnosis of PANS.…”

Section: Clinical Managementmentioning

confidence: 99%

Pediatric Acute-Onset Neuropsychiatric Syndrome: Current Perspectives

Gagliano¹,

Carta²,

Tanca³

et al. 2023

NDT

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Pediatric acute-onset neuropsychiatric syndrome (PANS) features a heterogeneous constellation of acute obsessive-compulsive disorder (OCD), eating restriction, cognitive, behavioral and/or affective symptoms, often followed by a chronic course with cognitive deterioration. An immune-mediated etiology is advocated in which the CNS is hit by different pathogen-driven (auto)immune responses. This narrative review focused on recent clinical (ie, diagnostic criteria, pre-existing neurodevelopmental disorders, neuroimaging) and pathophysiological (ie, CSF, serum, genetic and autoimmune findings) aspects of PANS. We also summarized recent points to facilitate practitioners with the disease management. Relevant literature was obtained from PubMed database which included only English-written, full-text clinical studies, case reports, and reviews. Among a total of 1005 articles, 205 were pertinent to study inclusion. Expert opinions are converging on PANS as the effect of post-infectious events or stressors leading to “brain inflammation”, as it is well-established for anti-neuronal psychosis. Interestingly, differentiating PANS from either autoimmune encephalitides and Sydenham’s chorea or from alleged “pure” psychiatric disorders (OCD, tics, Tourette’s syndrome), reveals several overlaps and more analogies than differences. Our review highlights the need for a comprehensive algorithm to help both patients during their acute distressing phase and physicians during their treatment decision. A full agreement on the hierarchy of each therapeutical intervention is missing owing to the limited number of randomized controlled trials. The current approach to PANS treatment emphasizes immunomodulation/anti-inflammatory treatments in association with both psychotropic and cognitive-behavioral therapies, while antibiotics are suggested when an active bacterial infection is established. A dimensional view, taking into account the multifactorial origin of psychiatric disorders, should suggest neuro-inflammation as a possible shared substrate of different psychiatric phenotypes. Hence, PANS and PANS-related disorders should be considered as a conceptual framework describing the etiological and phenotypical complexity of many psychiatric disorders.

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Palatal Petechiae in the Absence of Group AStreptococcusin Pediatric Patients with Acute-Onset Neuropsychiatric Deterioration: A Cohort Study

Cited by 5 publications

References 30 publications

Treatment of Pediatric Acute-Onset Neuropsychiatric Disorder in a Large Survey Population

Treatment of Pediatric Acute-Onset Neuropsychiatric Disorder in a Large Survey Population

Establishing a Pediatric Acute-Onset Neuropsychiatric Syndrome Clinic: Baseline Clinical Features of the Pediatric Acute-Onset Neuropsychiatric Syndrome Cohort at Karolinska Institutet

Pediatric Acute-Onset Neuropsychiatric Syndrome: Current Perspectives

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