Sensitivity and Specificity of the Modified Epworth Sleepiness Scale in Children With Craniopharyngioma

Abstract

Study Objectives: Children with craniopharyngioma are at risk for excessive daytime sleepiness (EDS). Multiple Sleep Latency Testing (MSLT) is the gold standard for objective evaluation of EDS; however, it is time and resource intensive. We compared the reliability, sensitivity, and specificity of the modified Epworth Sleepiness Scale (M-ESS) and MSLT in monitoring EDS in children with craniopharyngioma. Methods: Seventy patients (ages 6 to 20 years) with craniopharyngioma completed the M-ESS and were evaluated by polysomnography and MSLT. Evaluations were made after surgery, if performed, and before proton therapy. Results: MSLT revealed that 66 participants (81.8%) had EDS, as defined by a mean sleep latency (MSL) < 10 minutes, with only 28.8% reporting EDS on the M-ESS by using a cutoff score of 10. The M-ESS demonstrated adequate internal consistency and specificity (91.7%) but poor sensitivity (33.3%) with the established cutoff score of 10. A cutoff score of 6 improved the sensitivity to 64.8% but decreased the specificity to 66.7%. Conclusions: Patients with craniopharyngioma are at high risk for EDS, as documented objectively on the MSLT, but they frequently do not recognize or accurately report their sleepiness. Future sleep studies should investigate whether specific items or alternative self-and parent-reported measures of sleepiness may have greater clinical utility in monitoring sleepiness in this population.

Sleep disturbances in craniopharyngioma: a challenging diagnosis

“…The cases detailed in our report highlight that the clinical manifestation of these dysfunctions can be difficult to diagnose and can lead to misdiagnosis and inappropriate treatment that can harm the patient’s health and the quality of life of patients and their families. Furthermore, the diagnosis can be challenging because these patients do not clinically report sleepiness even when it is objectively present [ 7 ]. These findings support the need to incorporate comprehensive sleep assessment in survivors from childhood brain tumors involving the suprasellar/hypothalamic region.…”

“…Growing data in the literature demonstrate the increased risk of sleep disorders after the onset of tumors involving the hypothalamic area or after their treatment. Sleep fragmentation, impaired sleep quality and excessive daytime sleepiness (EDS) are commonly reported in CP with a pattern of circadian rhythm characterized by early morning awakening, followed by an extra period of sleep during the afternoon [5][6][7]. Cases of secondary narcolepsy have also been reported, particularly in children (70% of cases of narcolepsy), with CP being the prevalent type (38%) (8).…”

Sleep Disorders in Patients With Craniopharyngioma: A Physiopathological and Practical Update

“…The (58). EDS was identified by M-ESS in 76% of pediatric CP and strictly related to hypothalamic involvement (31).…”

“…M-ESS is a quick screening tool derived from ESS to identify EDS in children by assessing their likelihood to fall asleep in different everyday situations (from 0, which is low probability to fall asleep, to 3, which is high probability to fall asleep). A cutoff of 10 is indicative for EDS ( 58 ). EDS was identified by M-ESS in 76% of pediatric CP and strictly related to hypothalamic involvement ( 31 ).…”

“…EDS was identified by M-ESS in 76% of pediatric CP and strictly related to hypothalamic involvement ( 31 ). However, M-ESS may not be sensitive enough to screen pediatric patients for EDS ( 58 ), so complete sleep evaluation is often recommended ( 25 ). In survivors of childhood brain tumors, hypersomnia/narcolepsy was diagnosed on a median of 6.1 years from diagnosis and 4.7 years from cranial irradiation, and tumor location and radiation therapy were potential risk factors ( 26 ).…”