Abstract
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.
Highlights
There is increasing evidence for an association between autoimmune disease (AD) and neuropsychiatric disorders (Najjar et al 2013; Mataix-Cols et al 2018)
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 Pediatric acute-onset neuropsychiatric syndrome (PANS) (Kurlan et al 2008; Leckman et al 2011; Brimberg et al 2012; Cutforth et al 2016; Mahony et al 2017a, 2017b)
All study participants were recruited from a specialist pediatric obsessivecompulsive disorder (OCD) and related disorders outpatient clinic in Stockholm, Sweden
Summary
There is increasing evidence for an association between autoimmune disease (AD) and neuropsychiatric disorders (Najjar et al 2013; Mataix-Cols et al 2018). Pediatric acute-onset neuropsychiatric syndrome (PANS) is a descriptive entity of disputed validity and for which there are currently no defined biomarkers (Chang et al 2015; Hesselmark and Bejerot 2017a, 2017b) It affects young children, often in temporal relation to an uncomplicated infection, resulting in abrupt onset of obsessivecompulsive disorder (OCD) and/or anorexia, emotional lability, and a wide range of somatic symptoms (Swedo et al 1998; Chang et al 2015; Hesselmark and Bejerot 2017a, 2017b). 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)
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