Is pediatric catatonia on the rise?

In 2021, the American Academy of Pediatrics, American Academy of Child and Adolescent Psychiatry, and Children’s Hospital Association together declared a national State of Emergency in Children’s Mental Health.¹ Amidst a worsening pediatric mental health crisis, fueled by a bottleneck due to rising demand coupled with an extreme dearth of providers, greater attention has fallen upon mental health conditions in youth. One important and often underrecognized neuropsychiatric syndrome that often presents initially to the hospital setting is catatonia. Initially described over 150 years ago, its mechanism remains unclear, with much of our clinical understanding derived from adult data. Although both pediatric and adult catatonia are associated with significant morbidity and mortality, they demonstrate distinct demographics, symptoms, and associated diagnoses.^2,3 Those with autism, Trisomy 21, and neurodevelopmental disorders (NDDs) are disproportionately affected by catatonia.^2,4 Previous studies have attributed over 20% of pediatric catatonia to an underlying organic etiology, the prompt management of which is critical to treatment outcomes.² A limited number of studies have examined the incidence of pediatric catatonia (and are primarily focused on acute hospital discharges) prior to the COVID-19 pandemic, but to our knowledge no prior studies have examined post-pandemic pediatric-specific catatonia incidence, prevalence or trends over time.^3,5-7

In this issue, Lichtor et al describe the first known pediatric-focused study regarding recent trends in pediatric catatonia at a single quaternary pediatric hospital.⁸ Their retrospective analysis revealed a nearly 2.5-fold increase in the incidence of pediatric catatonia from 3.9 to 9.7 per 1000 psychiatry consults over a 6-year period from 2018 to 2023. The study indicated growth among those both with and without NDDs, though more so in those with NDDs. Particular growth occurred among medical etiology-based catatonia, 70% of which had autoimmune encephalitis. Among patients with an identifiable medical etiology, most had abnormal electroencephalograms, independent of benzodiazepine use. Patients with a medical etiology alone required a higher average daily benzodiazepine burden than those with a psychiatric or combined medical and psychiatric etiology.

This study begins to shed light on the incidence of an important, under-recognized diagnosis and supports many providers’ gestalt regarding the trend in pediatric catatonia cases in recent years. The generalizability and interpretability of these findings, however, remains limited to a single institution and to the emergency department or inpatient setting. The relatively small sample size limits the study’s power and ability to interpret comparison details between subgroups, thus raising questions for future studies to explore. The authors further relied upon clinical acumen to recognize the need for psychiatry expertise, and then upon accurate recognition of catatonia with documentation of an associated diagnosis code, with variable symptom documentation. Thus, the true eligible sample, and thereby the incidence of pediatric catatonia, may be underreported.

Setting-based sampling bias may influence overall catatonia incidence, prevalence and phenotypic presentation. This study offers an exciting glimpse into inpatient-managed catatonia, in which psychiatric consultation is more readily available, as compared to the outpatient non-urban setting with limited resources and psychiatry availability.⁹ Evidence suggests that access to psychiatric care and/or evaluation has a significant impact on time to catatonia diagnosis.¹⁰ Although this study focuses on those presenting to the inpatient setting, growing evidence suggests a substantial proportion of pediatric patients with catatonia are diagnosed and managed in the outpatient setting.¹¹ Furthermore, medical comorbidities, treatment-refractory illness, severe and/or acute presentations may be over-represented, and insidious onset or chronic symptomatology under-represented, among hospitalized individuals compared to those managed in the community setting, though formal comparisons have not been completed. This potential skew in existing epidemiologic data is highlighted in a recent multisite retrospective cohort in which over 90% of pediatric catatonia diagnoses occurred in inpatient settings.¹² This raises concern for potential underestimation of catatonia prevalence in those with NDDs, particularly in the outpatient setting, as those with non-medical primary diagnoses have been found to experience shorter hospitalizations and lower odds of clinical response compared to their neurotypical counterparts with catatonia.¹³ Broader sampling across outpatient, community, and specialty care settings is needed to capture the full spectrum of catatonia phenotypes, particularly among autistic individuals and those with co-occurring intellectual disability.

Regardless of setting, Lichtor et al highlight the importance of a thorough and thoughtful pediatric catatonia workup. This study corroborates previous findings that nearly one-fifth of catatonia cases are due to a medical etiology alone, with a similar number attributed to comorbid medical and psychiatric etiologies. Yet, notable variability existed in the rates at which laboratory, imaging, and procedural tests were performed, including significant differences between etiologic groups for a given test. While an individual’s presenting symptoms are prudent in guiding the medical workup, this variability highlights the lack of standardization in the assessment of pediatric catatonia. In fact, the first suggested single-institution standardized approach to the evaluation and management of pediatric catatonia was only recently published.¹²

Within the diagnostic workup, the role of genetic testing warrants particular attention, given a consistently high prevalence of syndromic NDDs in catatonia cohorts.^4,8 The authors’ finding that genetically determined NDDs were found in half of catatonia cases with comorbid medical and psychiatric etiologies builds upon prior work that identified pathogenic variants in over 40% of genetically tested individuals with catatonia and co-occurring NDDs.^4,8,14 As such, genetic testing may be particularly informative in catatonia with atypical features, early onset, or co-occurring intellectual disability. Additional studies utilizing a standardized approach to the evaluation of pediatric catatonia may help elucidate the diagnostic utility of various tests, many of which are costly and time intensive.

Objective data is critical to the initial assessment of catatonia, and too easily overlooked in those presenting with acute behavioral changes. Autonomic instability, in the setting of suspected or confirmed catatonia, should prompt consideration and swift management of possible malignant catatonia, a life-threatening form of catatonia with a significant mortality rate if untreated.¹⁵ A careful review of growth curves, particularly weight, can provide valuable insight into the possibility and severity of catatonia, often associated with significant weight changes due to decreased oral intake or increased energy expenditure, reflecting the catatonia subtype.¹⁶ Such an objective evaluation may be prescient, especially in autistic individuals, in whom overlapping features (e.g., mutism, stereotypy) can obscure catatonic symptoms if changes from baseline functioning are overlooked. Aggression, negativism, and other externalizing symptoms are also common in pediatric catatonia and may be falsely misattributed to oppositionality or resistance to care, especially in the inpatient setting.^10,17 Such misattribution, riddled by stigma and limited provider familiarity, may result in delayed diagnoses and associated morbidity, elevated stress upon health systems, and lack of recognition of an underlying medical etiology of a child’s catatonia.

Within the panorama of time, Lichtor et al’s⁸ finding of elevated rates of catatonia year after year is noteworthy. Could there be a historical link between pandemics? Their study spanned the COVID-19 pandemic, during which several pediatric catatonia cases were linked to COVID-19, suggesting that psychological and biological consequences of COVID-19 may have influenced the elevated catatonia rates, as was suspected during the Spanish Flu.^14,18 This is particularly relevant given the reported increase in catatonia due to underlying medical etiologies.⁸ The heightened awareness of catatonia in children and neurodivergent individuals may also contribute, as their symptoms are less well described in the DSM or Bush-Francis Catatonia Rating Scale, including urinary incontinence, acrocyanosis, recurrent self-injury, and nudism.^11,19,20 However, studies have yet to elucidate the extent to which recency bias and increasing awareness of catatonia contribute to the observed rise in diagnoses.

Lichtor et al provide important insights regarding the incidence and morphology of pediatric catatonia. The increasing rate of catatonia due to an underlying medical etiology, specifically autoimmune encephalitis, in their setting emphasizes the importance of considering a broad differential and targeted medical workup when patients present with a constellation of motor, affective and behavioral symptoms. Their findings also challenge clinicians to overcome implicit mental health stigma and bias which can lead to diagnostic overshadowing and treatment disparities.²¹ Although limited in scope, this study sets the stage for larger multi-site studies with greater power to determine the generalizability of this trend beyond that of a single institution, and to further delve into the factors contributing to these trends. The limited data available on the prevalence and trends in pediatric catatonia to date, augmented by this new literature to suggest a dramatically rising incidence, underscores the need for greater education and consistent data to facilitate more robust research in the field. Future studies may then build upon and integrate institutional pediatric catatonia clinical roadmaps toward the development of a much-needed, standardized evidence-based guideline for the evaluation and management of pediatric catatonia.

In summary, catatonia is a neuropsychiatric syndrome with significant morbidity and mortality rates, that is increasingly encountered by pediatric hospitalists. This study is the first to shed light on a remarkable multi-year upward trend in pediatric catatonia in the inpatient setting, especially that due to underlying medical etiologies, with associated evaluation and treatment implications. The observed pattern suggests multifactorial contributions and emphasizes the need for further investigation and education around this important diagnosis to promote optimal outcomes for these youth.

Abbreviations:

NDDs

neurodevelopmental disorders

COVID-19

Coronavirus disease 2019