Dataset Information

Continued Presence of Period Limb Movements During REM Sleep in Patients With Chronic Static Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS).

ABSTRACT:

Study objectives

A major component of pediatric acute-onset neuropsychiatric syndrome (PANS) is disruption of sleep. These disturbances have been reported in the acute phase of diagnosis but it is unknown if these sleep disruptions persist, especially in patients with chronic static symptoms. This retrospective chart review sought to review polysomnography (PSG) tests of patients in whom PANS has been clinically diagnosed in order to assess sleep architecture, periodic limb movements, and presence of rapid eye movement (REM) sleep without atonia (RSWA) after a chronic static course of symptoms, which were refractory to immunomodulatory interventions.

Methods

Patients were retrospectively identified through the PANS clinic at our institution and had to have fully completed a PSG study and be younger than 18 years. PSG with video were reviewed and scored based on established criteria.

Results

We identified 9 patients who met inclusion criteria. The median time from presentation to PSG was 4 years. This study identified PSG-measured periodic limb movement index (PLMI) > 5 events/h in REM sleep in 7 of 9 patients. Two patients with elevated PLMI also demonstrated RSWA, although neither fit a clinical diagnosis of REM sleep behavior disorder. This cohort also demonstrated increased onset of REM sleep (median 134 minutes), insomnia (median total sleep time of 389 minutes), and decreased sleep efficiency (77%).

Conclusions

This study identifies continued sleep disturbances in patients with refractory PANS symptoms several years after diagnosis and treatment. Continued sleep disturbances after presentation and treatment in patients with chronic static PANS may be a contributing factor in prolonged symptomatology of this disease process.

SUBMITTER: Santoro JD

PROVIDER: S-EPMC6040797 | biostudies-literature | 2018 Jul

REPOSITORIES: biostudies-literature

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Publications

Continued Presence of Period Limb Movements During REM Sleep in Patients With Chronic Static Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS).

Santoro Jonathan D JD Frankovich Jennifer J Bhargava Sumit S

Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine 20180715 7

<h4>Study objectives</h4>A major component of pediatric acute-onset neuropsychiatric syndrome (PANS) is disruption of sleep. These disturbances have been reported in the acute phase of diagnosis but it is unknown if these sleep disruptions persist, especially in patients with chronic static symptoms. This retrospective chart review sought to review polysomnography (PSG) tests of patients in whom PANS has been clinically diagnosed in order to assess sleep architecture, periodic limb movements, an ...[more]

PMID: 29991427

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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.

Project description:BackgroundPediatric obsessive-compulsive disorder (OCD) and eating disorder symptoms frequently overlap, clouding diagnostic certainty and hypothesized etiologic factors. Pediatric acute-onset neuropsychiatric syndrome (PANS) is defined by abrupt emergence of core obsessive-compulsive behaviours and/or food restriction with concurrent, ancillary cognitive and behavioral symptoms. Inflammatory and immune processes have putative roles in both PANS and a related described condition with cardinal obsessive-compulsive or tic symptoms, known as pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS). While prevalence of PANS and PANDAS has been examined in tic, movement disorder and OCD populations, this has not yet been systematically examined in a pediatric eating disorder sample.ObjectivesTo identify the lifetime prevalence of those meeting PANS and/or PANDAS criteria within a pediatric eating disorder cohort.MethodsConvenience sampling method was utilized to select consecutive youth (ages 8-18-years) presenting to an interdisciplinary pediatric eating disorder subspecialty program with a confirmed eating disorder and completed parent-report PANS/PANDAS questionnaire (n = 100). A parent-reported measure was used to establish lifetime prevalence rates for PANS and PANDAS. Descriptive and exploratory comparative analyses were conducted between PANS and non-PANS groups. Continuous measures were analyzed using two-tailed independent sample t-tests and categorical measures were analyzed using two-tailed Fisher's exact tests.ResultsAmong participants, 52% (n = 52) met PANS criteria and 0% (n = 0) met PANDAS diagnostic criteria. Core, abrupt-onset PANS symptoms included both food restriction and obsessive-compulsive symptoms in 63.5% (n = 33), food restriction only in 25% (n = 13), and obsessive-compulsive symptoms only in 11.5% (n = 6) of participants. In comparison to those who did not meet PANS criteria, those in the PANS subgroup were less likely to be male and more commonly prescribed a selective serotonin reuptake inhibitor medication. Significant group differences did not emerge for onset age, body mass index, eating disorder type or comorbid psychiatric/medical/autoimmune illness.ConclusionLifetime prevalence of symptoms in keeping with PANS diagnostic criteria within a pediatric eating disorder cohort was notably higher than that previously reported in OCD or tic disorder cohorts. The overlap between starvation effects and ancillary PANS symptoms may challenge the practical utility of this putative syndrome within the eating disorder population.

Project description:ObjectiveTo assess effects of treatment against a hypothesized neuroinflammation in children with symptoms corresponding to the research condition Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) which is not included in current diagnostic systems.MethodsSystematic literature searches were performed (1998 to June 2020) in PubMed, Embase, the Cochrane Library, CINAHL, PsycInfo, and HTA databases. Inclusion criteria: patients (P) were children (<18 years) with PANS; intervention (I)/comparison (C) was use of, versus no use of, anti-inflammatory, antibacterial or immunomodulating treatments; outcomes (O) were health-related quality of life (HRQL), level of functioning, symptom change, and complications.ResultsFour randomised controlled trials (RCTs) and three non-RCTs, including 23 to 98 patients, fulfilled the PICO. HRQL was not investigated in any study. Regarding level of functioning, two RCTs investigated antibiotics (penicillin V, azithromycin) and one RCT investigated immunomodulating treatments (intravenous immunoglobulins (IVIG), plasma exchange). Regarding symptoms, two non-RCTs investigated anti-inflammatory treatment (cyclooxygenase (COX) inhibitors, corticosteroids), two RCTs and one non-RCT investigated antibiotics (penicillin V, azithromycin), and two RCTs investigated immunomodulating treatments (IVIG, plasma exchange). Complications, reported in five studies, were consistent with those listed in the summary of products characteristics (SPC). All studies were assessed to have some or major problems regarding directness, the absence of an established diagnosis contributing to clinical diversity in the studied populations. All studies were assessed to have major risk of bias, including selection and detection biases. Due to clinical and methodological diversity, meta-analyses were not performed.ConclusionThis systematic review reveals very low certainty of evidence of beneficial effects, and moderate certainty of evidence of adverse effects, of anti-inflammatory, antibacterial or immunomodulating treatments in patients with symptoms corresponding to the research condition PANS. Available evidence neither supports nor excludes potential beneficial effects, but supports that such treatment can result in adverse effects.RegistrationPROSPERO (CRD42020155714).

Project description:When rhythmically moving two limbs in either the same or in opposite directions, one coupling mode meets constraints that are absent in the other mode. Isodirectional (ISO) flexion-extensions of the ipsilateral hand and foot can be easily performed with either the hand prone or supine. Instead, antidirectional (ANTI) movements require attentive effort and irresistibly tend to reverse into ISO when frequency increases. Experimental evidence indicates that the direction dependent easy-difficult dichotomy is caused by interference of the anticipatory postural commands associated to movements of one limb with voluntary commands to the other limb. Excitability of the resting wrist muscles is subliminally modulated at the period of ipsilateral foot oscillations, being phase-opposite in the antagonists and distributed so as to facilitate ISO and obstacle ANTI coupling of the hand (either prone or supine) with the foot. Modulation is driven by cortical signals dispatched to the forearm simultaneously with the voluntary commands moving the foot. If right foot oscillations are performed when standing on the left foot with the right hand touching a fixed support, the subliminal excitability modulation is replaced by overt contractions of forearm muscles conforming the APAs features. This suggests that during hand-foot ANTI coupling the voluntary commands to forearm muscles are contrasted by APAs commands of opposite sign linked to foot oscillations. Correlation between the easy-difficult dichotomy and the APAs distribution is also found in coupled adduction-abduction of the arms or hands in the transverse plane and in coupled flexion-extension of the arms in the parasagittal plane. In all these movements, APAs commands linked to the movement of each limb reach the motor pathways to the contralateral muscles homologous to the prime movers and can interfere during coupling with their voluntary activation. APAs are also generated in postural muscles of trunk and lower limbs and size-increase when the movement frequency is incremented. The related increase in postural effort apparently contributes in destabilizing the difficult coupling mode. Motor learning may rely upon more effective APAs. APAs and focal contraction are entangled within the same voluntary action. Yet, neural diseases may selectively impair APAs, which represent a potential target for rehabilitation.

Project description:Introduction: In March 2020, SARS-CoV-2 declared a pandemic by the World Health Organization. Restrictive isolation measures have also brought psychological distress to the pediatric population. Building on the syndrome's characteristics, the present study explored the impact of lockdown on the clinical course of young people with PANDAS/PANS. The initial hypothesis considered both the reduced exposure to viral agents and the strategies of the parents and other containment actions as protective factors against the worsening of symptoms. Methods: One hundred and eight children, adolescents, and young adults were recruited according to the multicenter PANDAS/PANS research program. Parents participated in a web-based survey. Results: contrary to our hypothesis, the study results show an increase in symptoms during the block in 71% of the sample. Psychometric analyzes allowed us to exclude a relationship between the main symptoms of PANDAS and the increase in symptoms or the presence of symptoms before the block and their increase over time. The increase in symptoms is best explained by the presence of sleep disturbances and emotional lability. The exacerbation also appears to be linked to the onset of new symptoms in children and adolescents with depressed moods and eating problems. Furthermore, irritability and oppositionality are significant predictors of acute exacerbation. Equally statistically significant is the factor linked to the effects of pandemic stress, such as the fear of contracting the virus. No significant associations for symptom reduction have been identified between parental strategies or other parent-initiated actions, but the study demonstrates that caregiver perceived efficacy on the strategies used can reduce the risk of exacerbation. Conclusion: This preliminary study highlights the importance of studying the causes of increased symptoms in children with PANDAS/PANS. Life events can exacerbate the clinical condition or generate new symptoms in young patients. In particular, environmental, family, and social changes in the course of clinical symptoms in PANDAS/PANS patients should be investigated. It highlights the importance of emotional and behavioral management, which can be improved by enhancing coping strategies in young people with PANDAS/PANS and their caregivers through a combination treatment in which CBT and PMT are included, in line with guidelines. Limits: An experimental proxy-report questionnaire not yet standardized and validated on the PANS/PANDAS pediatric clinical sample was used for the exploratory study. There is also a small sample size (N = 108) and the absence of a control group (pre-lockdown or children without PANDAS/PANS). It would be interesting to evaluate the exact long-term dimensions to see the course of symptoms after covid and conduct a new study focusing on the impact of stressful events on the clinical course of the syndrome.

Dataset Information

Continued Presence of Period Limb Movements During REM Sleep in Patients With Chronic Static Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS).

Study objectives

Methods

Results

Conclusions

Publications

Continued Presence of Period Limb Movements During REM Sleep in Patients With Chronic Static Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS).

Similar Datasets

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