Autism spectrum disorder in young patients with congenital central hypoventilation syndrome: role of the autonomic nervous system dysfunction.


Journal

Orphanet journal of rare diseases
ISSN: 1750-1172
Titre abrégé: Orphanet J Rare Dis
Pays: England
ID NLM: 101266602

Informations de publication

Date de publication:
03 Jul 2024
Historique:
received: 07 11 2023
accepted: 16 06 2024
medline: 4 7 2024
pubmed: 4 7 2024
entrez: 3 7 2024
Statut: epublish

Résumé

Congenital central hypoventilation syndrome (CCHS) is a rare condition characterized by alveolar hypoventilation and autonomic nervous system (ANS) dysfunction requiring long-term ventilation. CCHS could constitute a risk factor of autism spectrum disorder (ASD) due to birth injury related to respiratory failure, which remains to be determined. ANS dysfunction has also been described in ASD and there are indications for altered contribution of ANS-central nervous system interaction in processing of social information; thus, CCHS could be a risk factor for ASD based on pathophysiological background also. Our study aimed to determine the prevalence of ASD among CCHS patients, identify risk factors, and explore the relationship between the ANS, evaluated by heart rate variability indices, and adaptative functioning. Our retrospective study, based on the analysis of records of a French national center of patients with CCHS under 20 years of age, determined that the prevalence of ASD (diagnosed by a psychiatrist, following the criteria of DSM-4 or DSM-5) was 6/69 patients, 8.7% (95% confidence interval: 3.3-18.0%). In a case (CCHS with ASD, n = 6) - control (CCHS without ASD, n = 12) study with matching on sex, longer neonatal hospitalization stay and glycemic dysfunction were associated with ASD. Adaptative functioning was assessed using Vineland Adaptative behavioral scales (VABS) and heart rate variability indices (including daytime RMSSD as an index of parasympathetic modulation) were obtained from ECG Holter performed the same day. In 19 young subjects with CCHS who had both ECG Holter and VABS, significant positive correlations were observed between RMSSD and three of four sub-domains of the VABS (communication: R = 0.50, p = 0.028; daily living skills: R = 0.60, p = 0.006; socialization: R = 0.52, p = 0.021). Our study suggests a high prevalence of ASD in patients with CCHS. Glycemic dysfunction and longer initial hospitalization stays were associated with ASD development. A defect in parasympathetic modulation was associated with worse adaptative functioning.

Sections du résumé

BACKGROUND BACKGROUND
Congenital central hypoventilation syndrome (CCHS) is a rare condition characterized by alveolar hypoventilation and autonomic nervous system (ANS) dysfunction requiring long-term ventilation. CCHS could constitute a risk factor of autism spectrum disorder (ASD) due to birth injury related to respiratory failure, which remains to be determined. ANS dysfunction has also been described in ASD and there are indications for altered contribution of ANS-central nervous system interaction in processing of social information; thus, CCHS could be a risk factor for ASD based on pathophysiological background also. Our study aimed to determine the prevalence of ASD among CCHS patients, identify risk factors, and explore the relationship between the ANS, evaluated by heart rate variability indices, and adaptative functioning.
RESULTS RESULTS
Our retrospective study, based on the analysis of records of a French national center of patients with CCHS under 20 years of age, determined that the prevalence of ASD (diagnosed by a psychiatrist, following the criteria of DSM-4 or DSM-5) was 6/69 patients, 8.7% (95% confidence interval: 3.3-18.0%). In a case (CCHS with ASD, n = 6) - control (CCHS without ASD, n = 12) study with matching on sex, longer neonatal hospitalization stay and glycemic dysfunction were associated with ASD. Adaptative functioning was assessed using Vineland Adaptative behavioral scales (VABS) and heart rate variability indices (including daytime RMSSD as an index of parasympathetic modulation) were obtained from ECG Holter performed the same day. In 19 young subjects with CCHS who had both ECG Holter and VABS, significant positive correlations were observed between RMSSD and three of four sub-domains of the VABS (communication: R = 0.50, p = 0.028; daily living skills: R = 0.60, p = 0.006; socialization: R = 0.52, p = 0.021).
CONCLUSION CONCLUSIONS
Our study suggests a high prevalence of ASD in patients with CCHS. Glycemic dysfunction and longer initial hospitalization stays were associated with ASD development. A defect in parasympathetic modulation was associated with worse adaptative functioning.

Identifiants

pubmed: 38961480
doi: 10.1186/s13023-024-03257-z
pii: 10.1186/s13023-024-03257-z
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

249

Informations de copyright

© 2024. The Author(s).

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Auteurs

Benjamin Dudoignon (B)

Service de Physiologie Pédiatrique-Centre du Sommeil, Université de Paris-Cité, AP-HP, Hôpital Robert Debré, CRMR Maladies respiratoires rares - Hypoventilations alvéolaires rares - Syndrome d'Ondine, INSERM NeuroDiderot, Paris, F-75019, France. benjamin.dudoignon@aphp.fr.

Anna Maruani (A)

Service de psychiatrie de l'enfant et l'adolescent, Université de Paris-Cité, AP-HP, Hôpital Robert Debré, Paris, 75 019, France.

Richard Delorme (R)

Service de psychiatrie de l'enfant et l'adolescent, Université de Paris-Cité, AP-HP, Hôpital Robert Debré, Paris, 75 019, France.

Maxime Patout (M)

Service des Pathologies du Sommeil (Département R3S) - CRMR Hypoventilations centrales congénitales, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Sorbonne Université, AP-HP, Hôpital de la Pitié-Salpêtrière, INSERM, Paris, F-75005, France.

Mylene Fefeu (M)

Service de psychiatrie de l'enfant et l'adolescent, Université de Paris-Cité, AP-HP, Hôpital Robert Debré, Paris, 75 019, France.

Pierre Ellul (P)

Service de psychiatrie de l'enfant et l'adolescent, Université de Paris-Cité, AP-HP, Hôpital Robert Debré, Paris, 75 019, France.

Plamen Bokov (P)

Service de Physiologie Pédiatrique-Centre du Sommeil, Université de Paris-Cité, AP-HP, Hôpital Robert Debré, CRMR Maladies respiratoires rares - Hypoventilations alvéolaires rares - Syndrome d'Ondine, INSERM NeuroDiderot, Paris, F-75019, France.

Christophe Delclaux (C)

Service de Physiologie Pédiatrique-Centre du Sommeil, Université de Paris-Cité, AP-HP, Hôpital Robert Debré, CRMR Maladies respiratoires rares - Hypoventilations alvéolaires rares - Syndrome d'Ondine, INSERM NeuroDiderot, Paris, F-75019, France.

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