Narcolepsy and emotions: Is there a place for a theory of mind approach?

Narcolepsy type 1 is a central disorder of hypersomnolence characterized by excessive daytime sleepiness, rapid eye movement sleep-related manifestations, and cataplexy. In the current literature there is general agreement regarding neural correlates of Narcolepsy type 1 that appear to be related to anatomical and functional abnormalities in the hypothalamic region. In the last two decades, researchers shed light on the neurological bases of cataplexy by focusing on the neurobiological correlates of emotions. Although the results of these studies differ, they all point to an impairment in the amygdala and hypothalamus functions that are known to be involved in emotional processing, suggesting an impairment in this domain in narcoleptic patients. Indeed, despite heterogeneous results, several studies showed that narcoleptic patients differed from healthy controls in processing emotional stimuli. From a behavioral point of view, these findings suggest that alterations in emotional processing may be driven, at least in part, by compensatory strategies to avoid or reduce the frequency of cataplexy attacks. Surprisingly, the only study exploring in NT1 the behavioural performances in emotional facial recognition found no differences between NT1 adults and controls. We hypothesize that narcoleptic patients may present an alteration in a more complex socio-cognitive ability that is related to emotional processing, namely Theory of Mind. This review aims to investigate the literature supporting this hypothesis and to propose possible future developments on this topic.

Pediatric sleep disorder medicine training in Canada: past, present and future.

Pediatric sleep medicine is rapidly evolving in Canada. As pediatric sleep disorders are increasingly recognized, there is a growing need for clinicians educated in the evaluation and treatment of pediatric sleep disorders. Current pediatric sleep resources in Canada are inadequate to meet the needs of the population. Until this year, there was no formal pathway or specific requirements for pediatric sleep disorder medicine training in Canada and exposure to this field of medicine in post-graduate training was limited. In 2018, the Royal College of Physicians of Canada approved an Area of Focused Competence program for certification and maintenance of competence in Sleep Disordered Medicine. It was designed to ensure adequate breadth and depth of training experiences in this diverse field. The goals of the program are to ensure competence in the assessment and management of adults and children with a range of sleep-wake disorders and sleep-disordered breathing, to interpret sleep investigations, to administratively manage a sleep laboratory and to advance the discipline of Sleep Disorder Medicine through research. The program follows a competency-based model in which, within the year of training, trainees accumulate a dossier of experiences which are evaluated by a Royal College of Physicians and Surgeons committee. This new program will ensure that a critical mass of trained sleep medicine physicians is developed to meet the needs of the Canadian pediatric population and to contribute to advancement of the field of pediatric sleep medicine.

Pediatric pulmonology year in review 2017: Part 4 (Sleep medicine).

Pediatric Pulmonology publishes original research, case reports and review articles on topics related to a wide range of children's respiratory disorders. In this article (Part 4 of a 5-part series), we summarize the past year's publications in sleep medicine, in the context of selected literature in this area from other journals. Articles are highlighted on topics including diagnosis and treatment of OSAS, sleep duration and position, and sleep disorders in chronic disease.

Outcomes of long-term iron supplementation in pediatric restless legs syndrome/periodic limb movement disorder (RLS/PLMD).

OBJECTIVES: Restless legs syndrome (RLS) and periodic limb movement disorder (PLMD) are thought to center around a genetically mediated sensitivity to iron insufficiency. Previous studies have shown the effectiveness of short-term iron therapy in children with low iron storage. Little is known, however, about long-term iron treatment in children with RLS and PLMD. Therefore, we performed this study to assess the long-term effect of iron therapy in children with RLS and PLMD. METHODS: A retrospective chart review was performed for children who met the following criteria: A) diagnosed as having either RLS or PLMD, B) started on iron supplementation, C) followed up for >2 years in a sleep clinic. Baseline values for iron, ferritin, and periodic limb movement of sleep index (PLMS index) were defined in the three months leading up to the initiation of iron therapy. Values were also computed for follow-up periods of 3-6 months, 1-2 years, and >2 years. Serum iron and ferritin levels and PLMS index were compared between baseline and all subsequent follow-ups. RESULTS: In total, 105 patients met inclusion criteria, of whom 64 were diagnosed with PLMD alone, seven with RLS alone, and 35 with both RLS and PLMD. The average age was 10.2 ± 5.3 years. Compared to the baseline (27.4 ± 12.1 ng/ml), the average ferritin values at 3-6 months (45.62 ± 21.2 ng/ml, p < 0.001, n = 34), 1-2 years (52.0 ± 48.3 ng/ml, p <0.001, n = 63), and >2 years (54.7 ± 40.5 ng/ml, p <0.001, n = 67) were all significantly increased. Inversely, compared to baseline (21 ± 27.0/h, n = 66), PLMS index values at 3-6 months (7.5 ± 9.5/h p < 0.05, n = 11), 1-2 years (6.9 ± 8.9/h, p <0.001, n = 29), and >2 years (10 ± 14.5/h, p <0.001, n = 31) were all significantly decreased. No significant change in serum iron levels was noted at any time point. CONCLUSION: While retrospective in nature, this study demonstrates a sustained improvement in PLMS index and maintenance of adequate ferritin levels >2 years after iron therapy initiation in our RLS/PLMD cohort with a long-term follow-up. Iron therapy appears to lead to long-lasting improvements in children with RLS/PLMD.

Transoral robotic surgery for sleep apnea in children: Is it effective?

OBJECTIVES: Children with obstructive sleep apnea/hypopnea syndrome (OSAHS) as a result of base of tongue (BOT) or lingual tonsillar hypertrophy do not improve following adenotonsillectomy. In adults, transoral robotic surgery (TORS) offers a means of treating such patients, however the efficacy of this technique for children is not known. In this study, we examine the effectiveness of TORS BOT reduction and lingual tonsillectomy for treatment of pediatric OSAHS. METHODS: This was a retrospective study of nine patients (5 non-syndromic; 4 syndromic) who underwent isolated BOT reduction and lingual tonsillectomy for OSAHS between 2012 and 2014 at a tertiary care pediatric medical center. Differences between pre and post surgical polysomnograms (PSGs) were utilized to measure the procedural effectiveness. Patient age, sex, body mass index (BMI), developmental status, and comorbid conditions were also examined. RESULTS: The average patient age was 10.5 years (range 5.2-18.5). There were 5 males and 4 females. The mean pre-operative obstructive AHI (O-AHI) was 27.1 compared to 10.9 post-operatively (mean difference=16.1, t=2.27, p≤0.05). Statistically significant reductions were also noted in hypopneic events (mean difference=61.3, t=2.64, p<0.05) and lowest oxygen saturation (mean difference=9.0, t=-3.29, p≤0.01). One patient developed a post-operative bleed that was controlled operatively. CONCLUSIONS: In children with airway obstruction associated with BOT and lingual tonsillar hypertrophy, TORS is a useful and effective tool. Patients' who underwent TORS demonstrated a significant decrease in obstructive events. All patients reviewed exhibited at least a 50% reduction in O-AHI.