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.

Stability of nocturnal wake and sleep stages defines central nervous system disorders of hypersomnolence.

STUDY OBJECTIVES: We determine if young people with narcolepsy type 1 (NT1), narcolepsy type 2 (NT2), and idiopathic hypersomnia (IH) have distinct nocturnal sleep stability phenotypes compared to subjectively sleepy controls. METHODS: Participants were 5- to 21-year old and drug-naïve or drug free: NT1 (n = 46), NT2 (n = 12), IH (n = 18), and subjectively sleepy controls (n = 48). We compared the following sleep stability measures from polysomnogram recording between each hypersomnolence disorder to subjectively sleepy controls: number of wake and sleep stage bouts, Kaplan-Meier survival curves for wake and sleep stages, and median bout durations. RESULTS: Compared to the subjectively sleepy control group, NT1 participants had more bouts of wake and all sleep stages (p ≤ .005) except stage N3. NT1 participants had worse survival of nocturnal wake, stage N2, and rapid eye movement (REM) bouts (p < .005). In the first 8 hours of sleep, NT1 participants had longer stage N1 bouts but shorter REM (all ps < .004). IH participants had a similar number of bouts but better survival of stage N2 bouts (p = .001), and shorter stage N3 bouts in the first 8 hours of sleep (p = .003). In contrast, NT2 participants showed better stage N1 bout survival (p = .006) and longer stage N1 bouts (p = .02). CONCLUSIONS: NT1, NT2, and IH have unique sleep physiology compared to subjectively sleepy controls, with only NT1 demonstrating clear nocturnal wake and sleep instability. Overall, sleep stability measures may aid in diagnoses and management of these central nervous system disorders of hypersomnolence.

Sleep-Dependent Memory Consolidation in Children.

In the past 30 years, much research has been conducted elucidating the role of sleep in memory and learning; however, the interaction between sleep and cognitive functioning may be unknown in clinical realms. This article serves to provide a primer on sleep-dependent memory consolidation, a process in which memory is stabilized or even enhanced over a period of sleep. Given the increased amounts of sleep needed in infancy and childhood, the link between sleep and neuronal plasticity is highlighted in this article. Furthermore, sleep disruptions are common to children with neurodevelopmental disorders such as attention-deficit hyperactivity disorder; thus, recent studies showing direct relationships between sleep and memory functioning in such vulnerable groups are discussed.

Quality measures for the care of patients with narcolepsy.

ABSTRACT: The American Academy of Sleep Medicine (AASM) commissioned a Workgroup to develop quality measures for the care of patients with narcolepsy. Following a comprehensive literature search, 306 publications were found addressing quality care or measures. Strength of association was graded between proposed process measures and desired outcomes. Following the AASM process for quality measure development, we identified three outcomes (including one outcome measure) and seven process measures. The first desired outcome was to reduce excessive daytime sleepiness by employing two process measures: quantifying sleepiness and initiating treatment. The second outcome was to improve the accuracy of diagnosis by employing the two process measures: completing both a comprehensive sleep history and an objective sleep assessment. The third outcome was to reduce adverse events through three steps: ensuring treatment follow-up, documenting medical comorbidities, and documenting safety measures counseling. All narcolepsy measures described in this report were developed by the Narcolepsy Quality Measures Work-group and approved by the AASM Quality Measures Task Force and the AASM Board of Directors. The AASM recommends the use of these measures as part of quality improvement programs that will enhance the ability to improve care for patients with narcolepsy.

Functional neurological symptom disorders in a pediatric emergency room: diagnostic accuracy, features, and outcome.

BACKGROUND: In children, functional neurological symptom disorders are frequently the basis for presentation for emergency care. Pediatric epidemiological and outcome data remain scarce. OBJECTIVE: Assess diagnostic accuracy of trainee's first impression in our pediatric emergency room; describe manner of presentation, demographic data, socioeconomic impact, and clinical outcomes, including parental satisfaction. METHODS: (1) More than 1 year, psychiatry consultations for neurology patients with a functional neurological symptom disorder were retrospectively reviewed. (2) For 3 months, all children whose emergency room presentation suggested the diagnosis were prospectively collected. (3) Three to six months after prospective collection, families completed a structured telephone interview on outcome measures. RESULTS: Twenty-seven patients were retrospectively assessed; 31 patients were prospectively collected. Trainees' accurately predicted the diagnosis in 93% (retrospective) and 94% (prospective) cohorts. Mixed presentations were most common (usually sensory-motor changes, e.g. weakness and/or paresthesias). Associated stressors were mundane and ubiquitous, rarely severe. Families were substantially affected, reporting mean symptom duration 7.4 (standard error of the mean ± 1.33) weeks, missing 22.4 (standard error of the mean ± 5.47) days of school, and 8.3 (standard error of the mean ± 2.88) of parental workdays (prospective cohort). At follow-up, 78% were symptom free. Parental dissatisfaction was rare, attributed to poor rapport and/or insufficient information conveyed. CONCLUSIONS: Trainees' clinical impression was accurate in predicting a later diagnosis of functional neurological symptom disorder. Extraordinary life stressors are not required to trigger the disorder in children. Although prognosis is favorable, families incur substantial economic burden and negative educational impact. Improving recognition and appropriately communicating the diagnosis may speed access to treatment and potentially reduce the disability and cost of this disorder.

Increased pediatric functional neurological symptom disorders after the Boston marathon bombings: a case series.

BACKGROUND: Functional neurological symptom disorders are frequently the basis for acute neurological consultation. In children, they are often precipitated by high-frequency everyday stressors. The extent to which a severe traumatic experience may also precipitate functional neurological abnormalities is unknown. METHODS: For the 2-week period after the Boston Marathon bombings, we prospectively collected data on patients whose presentation suggested a functional neurological symptom disorder. We assessed clinical and demographic variables, duration of symptoms, extent of educational impact, and degree of connection to the Marathon bombing. We contacted all patients at 6 months after presentation to determine the outcome and accuracy of the diagnosis. RESULTS: In a parallel study, we reported a baseline of 2.6 functional neurological presentations per week in our emergency room. In the week after the Marathon bombings, this frequency tripled. Ninety-one percent of presentations were delayed by 1 week, with onset around the first school day after a city-wide lockdown. Seventy-three percent had a history of a prior psychiatric diagnosis. At the 6 months follow-up, no functional neurological symptom disorder diagnoses were overturned and no new organic diagnosis was made. CONCLUSIONS: Pediatric functional neurological symptom disorder may be precipitated by both casual and high-intensity stressors. The 3.4-fold increase in incidence after the Boston Marathon bombings and city-wide lockdown demonstrates the marked effect that a community-wide tragedy can have on the mental health of children. Care providers must be aware of functional neurological symptom disorders after stressful community events in vulnerable patient populations, particularly those with prior psychiatric diagnoses.

Development and implementation of a quality improvement curriculum for child neurology residents: lessons learned.

BACKGROUND: Quality improvement is a major component of the Accreditation Council for Graduate Medical Education core competencies required of all medical trainees. Currently, neither the Neurology Residency Review Committee nor the Accreditation Council for Graduate Medical Education defines the process by which this competency should be taught and assessed. We developed a quality improvement curriculum that provides mentorship for resident quality improvement projects and is clinically relevant to pediatric neurologists. METHODS: Before and after implementation of the quality improvement curriculum, a 14-item survey assessed resident comfort with quality improvement project skills and attitudes about implementation of quality improvement in clinical practice using a 5-point Likert scale. We used the Kruskal-Wallis and Fisher exact tests to evaluate pre to post changes. RESULTS: Residents' gained confidence in their abilities to identify measures (P = 0.02) and perform root cause analysis (P = 0.02). Overall, 73% of residents were satisfied or very satisfied with the quality improvement curriculum. CONCLUSIONS: Our child neurology quality improvement curriculum was well accepted by trainees. We report the details of this curriculum and its impact on residents and discuss its potential to meet the Accreditation Council for Graduate Medical Education's Next Accreditation System requirements.

Sleep deprivation and neurobehavioral functioning in children.

Sleep deprivation can result in significant impairments in daytime neurobehavioral functioning in children. Neural substrates impacted by sleep deprivation include the prefrontal cortex, basal ganglia and amygdala and result in difficulties with executive functioning, reward anticipation and emotional reactivity respectively. In everyday life, such difficulties contribute to academic struggles, challenging behaviors and public health concerns of substance abuse and suicidality. In this article, we aim to review 1) core neural structures impacted by sleep deprivation; 2) neurobehavioral problems associated with sleep deprivation; 3) specific mechanisms that may explain the relationship between sleep disturbances and neurobehavioral dysfunction; and 4) sleep problems reported in common neurodevelopmental disorders including attention deficit hyperactivity disorder (ADHD) and autistic spectrum disorders (ASDs).

Common neurological co-morbidities in autism spectrum disorders.

PURPOSE OF REVIEW: Autism spectrum disorders (ASDs) are heterogeneous neurodevelopmental disorders associated with various co-morbidities. Neurological co-morbidities include motor impairments, epilepsy, and sleep dysfunction. These impairments have been receiving more attention recently, perhaps because of their significant impact on the behavior and cognitive function of children with ASDs. Here, we review the epidemiology, etiology, and clinical approach to these neurological co-morbidities and highlight future research directions. RECENT FINDINGS: Motor impairments include stereotypies, motor delays, and deficits, such as dyspraxia, incoordination, and gait problems. Sleep dysfunction typically presents as difficulty with sleep onset and prolonged awakenings during the night. Recent data suggest that abnormalities in melatonin may affect sleep and may be a potential treatment target. There is no classic epilepsy syndrome associated with ASDs. Intellectual disability, syndromic autism, and female sex are specific risk factors. Recent research has focused on identifying the overlapping pathways between these neurological co-morbidities and the core deficits in ASDs, which may have direct and powerful implications for treatment and prognosis. SUMMARY: Motor impairment, epilepsy, and sleep dysfunction are common neurological co-morbidities in ASDs. Clinicians should be aware that recognition and treatment of these issues may improve the function and outcome of children with ASDs.

Intracranial artery dissection in an adolescent with Marfan syndrome.

Marfan syndrome is an autosomal dominant connective tissue disorder commonly due to mutation of the fibrillin-1 (FBN-1) gene that causes disruption of elastic fibers in large- and medium-size arteries and predisposes to aneurysm formation and arterial dissection. Cardiovascular complications occur in most patients with Marfan syndrome, but interestingly, neurovascular complications of Marfan syndrome are rare. We present a novel case of an adolescent with Marfan syndrome with spontaneous intracranial cerebral artery dissection and ischemic stroke with hemorrhagic transformation. This case is novel in that it reports spontaneous intracranial dissection in a young patient with Marfan syndrome and highlights the rare intrinsic neurovascular complications that can occur in these patients.

New onset epilepsy in Prader-Willi syndrome: semiology and literature review.

Prader-Willi syndrome is a chromosomal disorder caused by absence of expression of the paternal active genes in the 15q11∼q13 chromosome region; it is associated with an increased incidence of epilepsy and narcolepsy. Presented here is the case of a 2.5-year-old boy with Prader-Willi syndrome and a history of neonatal superior sagittal sinus thrombosis with new onset of atonic seizures with electrographic onset from the parasagittal region. It is postulated that microscarring from neonatal venous sinus thrombosis, history of febrile seizures, and Prader-Willi syndrome are factors predisposing him to epilepsy. The importance of video electroencephalography with electromyography electrodes is emphasized for Prader-Willi syndrome patients with drop episodes, to differentiate cataplexy from seizures. This being a novel report of a Prader-Willi syndrome patient with atonic seizures, the literature on seizure semiology among patients with Prader-Willi syndrome is reviewed.

Epilepsy in Prader-Willi syndrome: clinical characteristics and correlation to genotype.

Prader-Willi syndrome (PWS) is a genomic imprinting disease secondary to the loss of a functional paternal copy of 15q11-q13. Unlike its related imprinting disorder, Angelman syndrome, PWS has not been regarded as a risk factor for epilepsy. A retrospective analysis of 92 patients with PWS identified 24 (26%) with seizures. Twenty-two of these (92%) were affected by focal epilepsy and only two (8%) had generalized epilepsy. The most common seizure type was staring spells (67%). Correlation to genotype analysis showed deletions were more common in patients with epilepsy than in patients without epilepsy. The epilepsy syndromes were easy to control with a single antiepileptic drug in most cases. Three patients (11%) had had febrile seizures. These findings suggest that PWS may be a risk factor for epilepsy, which can manifest with focal features. Patients with PWS with a deletion genotype showed a trend toward developing seizures compared with patients with other genotypes in our series, even though this difference did not achieve statistical significance.