Characteristics of non-sleep related apneas in children with alternating hemiplegia of childhood.

Autor: Thamby J; Department of Pediatrics, Division of Neurology and Developmental Pediatrics, Duke University School of Medicine, Durham, NC, United States., Prange L; Department of Pediatrics, Division of Neurology and Developmental Pediatrics, Duke University School of Medicine, Durham, NC, United States., Boggs A; Department of Pediatrics, Division of Neurology and Developmental Pediatrics, Duke University School of Medicine, Durham, NC, United States., Subei MO; Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Duke University School of Medicine, Durham, NC, United States., Myers C; Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Duke University School of Medicine, Durham, NC, United States., Uchitel J; Department of Pediatrics, Division of Neurology and Developmental Pediatrics, Duke University School of Medicine, Durham, NC, United States; Stanford University Medical School, Palo Alto, CA, United States., ElMallah M; Department of Pediatrics, Division of Pulmonary Medicine, Duke University School of Medicine, United States., Bartlett-Lee B; Department of Pediatrics, Baylor College of Medicine, Houston, TX, United States., Riviello JJ; Department of Pediatrics, Baylor College of Medicine, Houston, TX, United States., Mikati MA; Department of Pediatrics, Division of Neurology and Developmental Pediatrics, Duke University School of Medicine, Durham, NC, United States. Electronic address: mohamad.mikati@duke.edu.
Jazyk: angličtina
Zdroj: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society [Eur J Paediatr Neurol] 2024 Jan; Vol. 48, pp. 101-108. Date of Electronic Publication: 2023 Dec 10.
DOI: 10.1016/j.ejpn.2023.12.002
Abstrakt: Background: Non-sleep related apnea (NSA) has been observed in alternating hemiplegia of childhood (AHC) but has yet to be characterized.
Goals: Investigate the following hypotheses: 1) AHC patients manifest NSA that is often severe. 2) NSA is usually triggered by precipitating events. 3) NSA is more likely in patients with ATP1A3 mutations.
Methods: Retrospective review of 51 consecutive AHC patients (ages 2-45 years) enrolled in our AHC registry. NSAs were classified as mild (not needing intervention), moderate (needing intervention but not perceived as life threatening), or severe (needing intervention and perceived as life threatening).
Results: 19/51 patients (37 %) had 52 NSA events (6 mild, 11 moderate, 35 severe). Mean age of onset of NSA (± Standard Error of the Mean (SEM)): 3.8 ± 1.5 (range 0-24) years, frequency during follow up was higher at younger ages as compared to adulthood (year 1: 2.2/year, adulthood: 0.060/year). NSAs were associated with triggering factors, bradycardia and with younger age (p < 0.008 in all) but not with mutation status (p = 0.360). Triggers, observed in 17 patients, most commonly included epileptic seizures in 9 (47 %), anesthesia, AHC spells and intercurrent, stressful, conditions. Management included use of pulse oximeter at home in nine patients, home oxygen in seven, intubation/ventilatory support in seven, and basic CPR in six. An additional patient required tracheostomy. There were no deaths or permanent sequalae.
Conclusions: AHC patients experience NSAs that are often severe. These events are usually triggered by seizures or other stressful events and can be successfully managed with interventions tailored to the severity of the NSA.
Competing Interests: Declaration of competing interest Dr. Mohamad Mikati reports that he has intellectual property interest in gene therapy of ATP1A3 related disease.
(© 2023 Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.)
Databáze: MEDLINE
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