Abstract
Sleep disorders (SDs) represent an important issue in patients with craniopharyngioma (CP). Nearly 70% of these patients complain of sleep-wake cycle alterations and/or excessive diurnal somnolence due to sleep-related breathing disorders, such as obstructive sleep apnea (OSA) and/or central hypersomnia, including secondary narcolepsy. SDs may severely reduce quality of life, increase disease-related cardiorespiratory and cardiovascular morbidity, and finally play a major role in increased long-term mortality reported on patients with CP. A major risk factor for SDs is represented by the hypothalamic syndrome, which may develop because of direct hypothalamic damage by the tumor itself and/or complications of the treatments, neurosurgery and/or radiotherapy, and typically includes permanent neuroendocrine dysfunctions, morbid obesity, and secondary metabolic disorders. Despite increasing attention to SDs in the general population, and in particular to OSA as a risk factor for cardio-metabolic diseases and excessive daytime somnolence, sleep evaluation is still not routinely proposed to patients with CP. Hence, SDs are often underdiagnosed and undertreated. The aim of this paper is to update current knowledge of the pathogenesis and prevalence of SDs in patients with CP and propose practical algorithms for their evaluation and management in clinical practice. Particular attention is paid to screening and diagnostic tools for appropriate characterization of SDs, identification of risk factors, and potential role of hypothalamic sparing surgery in the prevention of morbid obesity and SDs. Available tools in sleep medicine, including lifestyle interventions, drugs, and respiratory devices, are discussed, as well as the importance of optimal hormone replacement and metabolic interventions. Current limits in the diagnosis and treatment of SDs in patients with CP and possible future avenues for research agenda are also considered.
Highlights
Craniopharyngiomas (CPs) are rare benign parasellar tumors derived from Rathke’s pouch rests, and are classified into two subtypes [1]
The most dramatic complication is the development of a hypothalamic syndrome (HS), which is typically associated with neuroendocrine disorders and includes neurocognitive changes [4] morbid hypothalamic obesity (HO) and related systemic complications [5], a variety of sleep disorders (SDs) including sleep-related breathing disorders (SBDs), central hypersomnia and abnormal wake-sleep circadian rhythms [6, 7], and less commonly abnormalities of thirst and central temperature or cardiovascular regulation
Almost 70% of complaints of SDs and/or excessive daytime sleepiness (EDS) are due to SBDs, central hypersomnias, and circadian rhythm sleepwake disorders (CRSWDs)
Summary
Craniopharyngiomas (CPs) are rare benign parasellar tumors derived from Rathke’s pouch rests, and are classified into two subtypes [1]. Within the methodological limits and heterogeneity of reported studies, the prevalence of SDs and/or excessive daytime sleepiness (EDS) approaches 70–80% [11, 13], with an adult prevalence of OSA around 40% [6]. Hypothalamic dysfunction plays an essential role in the development of SDs. Damage to the suprachiasmatic nucleus (SCN), the central biological “master clock,” leads to abnormal circadian rhythms and sleep-wake cycles. According to the Wisconsin Sleep Cohort, ∼13% of men and 6% of women have moderateto-severe sleep apnea (AHI>15/h), and 14% of men and 5% of women have AHI ≥ 5/h plus symptoms of daytime sleepiness, both increasing with age and body mass index (BMI) [43] As a consequence, these estimates have grown substantially over the last two decades, largely because of the rising obesity epidemic [43].
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