REM sleep atonia in patients with pediatric acute-onset neuropsychiatric syndrome: implications for pathophysiology
Patrizia Congiu1,2, Antonella Gagliano3, Sara Carucci4,5
1Department of Medical Sciences and Public Health, Sleep Disorder Research Center, University of Cagliari, Cagliari, Italy.
Insights
Children with pediatric acute-onset neuropsychiatric syndrome have significantly reduced REM sleep atonia. This REM sleep motor disinhibition may indicate brainstem dysfunction in these patients.
Area of Science:
- Neurology
- Sleep Medicine
- Pediatric Psychiatry
Background:
- Sleep disorders are common in pediatric acute-onset neuropsychiatric syndrome (PANS), affecting up to 80% of children.
- Rapid eye movement (REM) sleep motor disinhibition is a frequent feature in PANS.
- Limited polysomnographic studies exist for PANS, particularly concerning REM sleep atonia.
Purpose of the Study:
- To evaluate polysomnographic characteristics in children with PANS.
- To specifically assess REM sleep without atonia using the REM atonia index (RAI).
- To compare RAI in PANS patients with age- and sex-matched healthy controls.
Main Methods:
- Sixty-nine children diagnosed with PANS underwent polysomnography.
- Chin muscle tone during REM sleep was quantified using the REM atonia index (RAI).
- Forty-four healthy children served as controls.
Main Results:
- Patients with PANS had a significantly lower RAI compared to controls.
- In controls, RAI increased with age; this trend was absent in PANS patients.
- 25 out of 57 PANS patients (44%) had RAI below the normal range, versus 1 out of 43 controls (2%).
Conclusions:
- REM sleep abnormalities are significant in pediatric acute-onset neuropsychiatric syndrome.
- Reduced RAI and lack of age correlation suggest impaired brainstem regulation of REM sleep atonia in PANS.
- Findings point to potential pathophysiology involving brainstem dysfunction in PANS.
Study Objectives:
Sleep disorders and/or disordered sleep represent common clinical presentations of pediatric acute-onset neuropsychiatric syndrome, occurring in up to 80% of affected children, with rapid eye movement (REM) sleep motor disinhibition being a prevalent feature. To date, limited polysomnographic studies have been conducted. Therefore, the objective of this study was to evaluate the polysomnographic characteristics of a cohort of children with pediatric acute-onset neuropsychiatric syndrome, focusing particularly on REM sleep without atonia as assessed by the REM atonia index (RAI), and to compare these characteristics with those of a control group.
Methods:
Sixty-nine patients diagnosed with pediatric acute-onset neuropsychiatric syndrome were consecutively recruited who underwent comprehensive polysomnographic examinations following standard criteria. Chin muscle tone during REM sleep was evaluated using RAI. Forty-four healthy individuals, matched for age and sex, were used as controls.
Results:
RAI was significantly lower in patients compared to controls. In controls, RAI showed a significant progressive increase with age from preschool to adolescence. Conversely, this age-related trend was absent in patients, with no significant correlation between RAI and age, with a random distribution of values. Only 1 control individual (1/43) but 25 patients (25/57) exhibited a RAI below the lower limit of the predicted range obtained from controls.
Conclusions:
Our findings underscore the significance of REM sleep abnormalities in children with pediatric acute-onset neuropsychiatric syndrome. The substantial reduction in RAI and the lack of correlation between RAI and age observed in patients suggest a potential pathological mechanism leading to dysfunction in brainstem structures regulating REM sleep atonia in this population.
Citation:
Congiu P, Gagliano A, Carucci S, Lanza G, Ferri R, Puligheddu M. REM sleep atonia in patients with pediatric acute-onset neuropsychiatric syndrome: implications for pathophysiology. J Clin Sleep Med. 2025;21(5):757-764.
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