Recommended protocols for the Multiple Sleep Latency Test and Maintenance of Wakefulness Test in children: guidance from the American Academy of Sleep Medicine.

The American Academy of Sleep Medicine commissioned a task force of clinical experts in pediatric sleep medicine to review published literature on performing the Multiple Sleep Latency Test (MSLT) and Maintenance of Wakefulness Test for diagnosis and management of central disorders of hypersomnolence among children and adolescents. This paper follows a format similar to that of the paper "Recommended protocols for the Multiple Sleep Latency Test and Maintenance of Wakefulness Test in adults: guidance from the American Academy of Sleep Medicine" that was published in 2021. Since there is insufficient evidence to specify a recommended protocol for the Maintenance of Wakefulness Test in children and adolescents, this paper focuses only on the MSLT protocol. This protocol paper provides guidance to health care providers who order, sleep specialists who interpret, and technical staff who administer the MSLT to pediatric patients. Similar to the adult protocol paper, this document provides guidance based on pediatric expert consensus and evidence-based data when available. Topics include patient preparation, evaluation of medication and substance use, sleep needs before testing, scheduling considerations, optimal test conditions for youth, and documentation. Specific changes recommended for pediatric MSLT protocols include (1) provision of a minimum of 7 hours of sleep (with a minimum 8-hour recording time) on polysomnography the night before the MSLT, ideally meeting age-based needs; (2) use of clinical judgment to guide the need for sleep-disordered breathing treatments before polysomnography-MSLT testing; and (3) shared patient-health care provider decision-making regarding modifications in the protocol for children and adolescents with neurodevelopmental/neurological disorders, young age, and/or delayed sleep phase. CITATION: Maski KP, Amos LB, Carter JC, Koch EE, Kazmi U, Rosen CL. Recommended protocols for the Multiple Sleep Latency Test and Maintenance of Wakefulness Test in children: guidance from the American Academy of Sleep Medicine. J Clin Sleep Med. 2024;20(4):631-641.

Primary tracheocutaneous fistula closure with immediate transition to nocturnal noninvasive positive pressure ventilation in two children with Congenital Central Hypoventilation Syndrome.

Transitioning children with Congenital Central Hypoventilation Syndrome (CCHS) from nocturnal invasive ventilation via tracheostomy to noninvasive positive pressure ventilation (NIPPV) is challenging due to the leak caused by the tracheocutaneous fistula (TCF), resulting in insufficient ventilation. Decannulation and primary closure of the TCF with immediate transition to nocturnal NIPPV was performed in two children with CCHS at a tertiary care children's hospital. Neither child developed significant adverse effects such as pneumomediastinum or pneumothorax. This technique is a novel approach that may improve decannulation outcomes and aid transition to NIPPV in this patient population.

Treatment of pediatric restless legs syndrome.

OBJECTIVE: The primary aim was to determine if iron supplementation effectively treats children with restless legs syndrome (RLS), the time to improvement or resolution of symptoms, and patient characteristics (family history of RLS, secondary sleep disorders, medical diagnoses, and/or mental health diagnoses) that may affect outcome. METHODS.: This was a retrospective chart review of children between 5 and 18 years old who were diagnosed with RLS at the pediatric sleep disorders clinic at Children's Hospital of Wisconsin in Milwaukee, Wisconsin. Documented RLS treatment approaches included supplemental iron, nonpharmacologic interventions, melatonin, gabapentin, clonidine, and dopamine agonists (pramipexole and ropinirole). RESULTS: Ninety-seven children were diagnosed with RLS; 60.8% of children were between 5 and 11 years old. Most children (65%) received iron either as monotherapy or in combination with other treatments. Approximately 80% of the children who received iron and had follow-up had improvement or resolution of their symptoms. The median baseline ferritin level was 22.7 ng/mL, and 71% of children had a ferritin level less than 30 ng/mL. The median time to improvement or resolution of symptoms was 3.8 months. CONCLUSIONS: Supplemental iron as monotherapy or in combination with other treatments is effective in treating pediatric RLS. A prospective study could help determine if the initial ferritin level and degree of change in the ferritin level impact response to iron treatment. It is also important to study the long-term outcomes in these patients.

Severe central sleep apnea in a child with leukemia on chronic methadone therapy.

We describe a child with acute myeloid leukemia (AML) who developed severe central sleep apnea (CSA) on methadone therapy for chronic pain management. His chemotherapy-related cerebral atrophy and renal insufficiency with impaired methadone clearance may have also contributed to the severity of his sleep-disordered breathing. Maintenance methadone treatment is not a common pediatric practice; therefore, the adverse effects of methadone therapy, including CSA, are rarely reported in children.

Metabotropic glutamate receptors (mGluR5) activate transient receptor potential canonical channels to improve the regularity of the respiratory rhythm generated by the pre-Bötzinger complex in mice.

Metabotropic glutamate receptors (mGluRs) are hypothesized to play a key role in generating the central respiratory rhythm and other rhythmic activities driven by central pattern generators (e.g. locomotion). However, the functional role of mGluRs in rhythmic respiratory activity and many motor patterns is very poorly understood. Here, we used mouse respiratory brain-slice preparations containing the pre-Bötzinger complex (pre-BötC) to identify the role of group I mGluRs (mGluR1 and mGluR5) in respiratory rhythm generation. We found that activation of mGluR1/5 is not required for the pre-BötC to generate a respiratory rhythm. However, our data suggest that mGluR1 and mGluR5 differentially modulate the respiratory rhythm. Blocking endogenous mGluR5 activity with 2-Methyl-6-(phenylethynyl)pyridine (MPEP) decreases the inspiratory burst duration, burst area and frequency, whereas it increases the irregularity of the fictive eupneic inspiratory rhythm generated by the pre-BötC. In contrast, blocking mGluR1 reduces the frequency. Moreover, the mGluR1/5 agonist 3,5-dihydroxyphenylglycine increases the frequency and decreases the irregularity of the respiratory rhythm. Based on previous studies, we hypothesized that mGluR signaling decreases the irregularity of the respiratory rhythm by activating transient receptor potential canonical (TRPC) channels, which carry a non-specific cation current (ICAN). Indeed, 3,5-dihydroxyphenylglycine (DHPG) application reduces cycle-by-cycle variability and subsequent application of the TRPC channel blocker 1-[2-(4-methoxyphenyl)-2-[3-(4-methoxyphenyl)propoxy]ethyl]imidazole (SKF-96365) hydrochloride reverses this effect. Our data suggest that mGluR5 activation of ICAN-carrying TRPC channels plays an important role in governing the cycle-by-cycle variability of the respiratory rhythm. These data suggest that modulation of TRPC channels may correct irregular respiratory rhythms in some central neuronal diseases.

Chiari I malformation presenting as chronic cough.

We present a 9-month-old infant with persistent cough refractory to conventional asthma therapy. An extensive evaluation eventually revealed a Chiari I malformation with syringohydromyelia. His cough resolved one month after surgical decompression, suggesting that brainstem compression from the Chiari malformation directly caused his symptoms.