Obstructive sleep apnea in people with intellectual disabilities: adherence to and effect of CPAP.

PURPOSE: Obstructive sleep apnea (OSA) is common in people with intellectual disabilities (ID), but in practice continuous positive airway pressure (CPAP) is often deemed unfeasible. We investigated adherence to and effect of CPAP in patients with ID and OSA. METHODS: Patients with ID were started on CPAP using an intensive training program. Acceptable adherence was defined as use of ≥ 4 h/night during ≥ 70% of the nights. Treatment effect was measured with a patient global impression scale and customized questionnaires. Reasons for not starting CPAP, factors influencing treatment, and reasons for terminating CPAP were explored. RESULTS: Of 39 patients with ID, 87% after 8-10 weeks and 70% at 8 months still used CPAP, of whom 74% and 77% showed acceptable adherence. Baseline apnea-hypopnea (AHI) index decreased from 41.2/h to 5.3/h after 8-10 weeks (p < 0.001), and 4.3/h after 8 months (p < 0.001). At 8-10 weeks and after 8 months, there was an improvement in the most restrictive reported complaint (both p < 0.0005), difficulty waking up (p < 0.01; p < 0.0005), handling behavior (p < 0.03; p < 0.02), presence of irritability (p < 0.01), and sleepiness (p < 0.05). The expectation that CPAP would not be tolerated was the main reason for not starting. CPAP use in the first 2 weeks predicted adherence at 8-10 weeks and 8 months (r = 0.51, p < 0.01; r = 0.69, p < 0.01). Of 13 patients who terminated CPAP, the reasons for termination included behavioral problems, comorbid insomnia, anxiety, discomfort, or other side effects. CONCLUSIONS: With adequate guidance, CPAP is both feasible and effective in people with ID and OSA.

The importance of specialized sleep investigations in children with a suprasellar tumor.

PURPOSE: Disruption of sleep has great impact on quality of life. In children with a suprasellar tumor and hypothalamic-pituitary dysfunction, the circadian rhythm may be disturbed causing sleep problems. However, also other factors may influence sleep. Awareness of these different etiologies and careful history taking with appropriate additional diagnostics will aid in restoring sleep quality. METHODS: We present the workup of 4 cases with a suprasellar tumor and disturbances of sleep initiation, sleep maintenance, and daytime sleepiness. In parallel, we developed a flowchart, to aid clinicians in the diagnostics of sleep problems in children after treatment for a (supra) sellar brain tumor. RESULTS: All four patients, known with hypopituitarism, presented with sleep complaints and increased daytime sleepiness. In all four, the cause of sleep problems showed to be different. In the first case, sleep evaluation revealed a severe obstructive sleep apnea, whereupon nocturnal ventilation was started. The second case revealed poor sleep hygiene in combination with an obsessive compulsive disorder. Sleep hygiene was addressed and psychiatric consultation was offered. Dexamphetamine treatment was started to reduce her obsessive compulsive complaints. The third case showed a delayed sleep phase syndrome, which improved by educational support. The fourth case revealed a secondary organic hypersomnia for which modafinil treatment was started. CONCLUSION: Sleep disturbances in children with hypopituitarism due to a (supra) sellar tumor can have different entities which require specific therapy. Awareness of these different entities is important to enable appropriate counseling. Referral to an expertise sleep center may be advised, if standard educational support is insufficient.

[Kleine Levin Syndrome: more than just periodic hypersomnia]. / Kleine-Levin-syndroom: meer dan periodieke hypersomnie.

BACKGROUND: Kleine Levin Syndrome (KLS) is a rare disease with periodic hypersomnia as its main feature. Hyperphagia and hypersexuality are also described as classical symptoms, although quite recently it has become clear that the full triad is absent in the majority of patients. CASE DESCRIPTION: A 14-year-old boy developed KLS after a period of flu-like symptoms. Over the course of three years he suffered from seven one-week episodes of extreme hypersomnia (sleeping 18 hours a day), depersonalisation, apathy, anxiety, paranoia, confusion, hallucinations and uninhibited sexual behaviour. He ate little. Ancillary investigations did not reveal any abnormalities. In between these episodes he had no symptoms. CONCLUSION: From this case description and a summary of the symptoms of twelve other patients with KLS, it appears that neuropsychiatric symptoms are much more prominent than hyperphagia and hypersexuality. It is important that the typical KLS phenotype be reappraised, so that the condition can be recognised early and patients managed appropriately.

Postoperative day 3 serum human chorionic gonadotropin decline as a predictor of persistent ectopic pregnancy after linear salpingotomy.

OBJECTIVE: To evaluate the ability of preoperative clinical, ultrasonographic, intraoperative findings and pre-postoperative serum human chorionic gonadotropin (hCG) levels to predict persistent ectopic pregnancy (EP). STUDY DESIGN: Retrospective cohorts study. SETTING: Tertiary care, university hospital. In all, 61 women with EP treated with laparoscopic linear salpingostomy between January 1995 and December 1999. RESULT: Out of 61 patients, 10 (9%) were diagnosed with a persistent EP. When compared with 51 (91%) successfully treated patients there were no differences in preoperative clinical and ultrasonographic findings, preoperative serum hCG levels and intraoperative findings. The postoperative decline of hCG levels were different in both groups. No case of persistent EP was found if the postoperative day 3 decline of hCG was more than 55%. CONCLUSION: Postoperative serum hCG follow-up is important after salpingotomy to prevent persistent EP. A decline of less than 55% at day 3 predicts persistent EP and may select early cases for second line methotrexate therapy.