Sleep disturbances in craniopharyngioma: a challenging diagnosis

J Neurol. 2021 Nov;268(11):4362-4369. doi: 10.1007/s00415-021-10794-1. Epub 2021 Sep 14.

Abstract

Craniopharyngiomas are rare solid or mixed solid and cystic tumors that arise from Rathke's pouch remnants along the pituitary-hypothalamic axis, from the sella turcica to the brain third ventricle. Both the tumor and its treatment can lead to significant neurological and endocrinological complications. Due to the essential role of the hypothalamus in the complex neurophysiologic process of sleep, tumors involving the hypothalamic area may be responsible for disturbances in sleep-wake regulation with alterations in the circadian rhythm, sleep fragmentation, and increased daytime sleepiness. We report two cases of patients with craniopharyngioma, who came to our attention due to the occurrence of episodes characterized by psychomotor slowing and afinalistic limb movements, temporal and spatial disorientation, psychomotor agitation, and oneiric stupor like episodes. A comprehensive clinical data collection and a targeted diagnostic work-up led to a diagnosis of severe sleep disorder characterized by hypersomnia, altered sleep-wake rhythm, and sleep-related breathing disorder. In addition, the polysomnography revealed peculiar alterations in the sleep structure. The diagnostic work-up lead to an accurate differential diagnosis between epileptic seizures and episodes expressions of sleep disturbances. These clinical features can be challenging to diagnose and can lead to misdiagnosis and inappropriate treatment. Diagnosis of sleep disorders is crucial, considering the impact of sleep on general health, cognition, and neuropsychological functioning. These findings support the need to incorporate a comprehensive sleep evaluation in childhood brain tumor involving the suprasellar/hypothalamic region.

Keywords: Craniopharyngioma; Hypersomnolence; Hypothalamic dysfunction; Sleep; Sleep-related breathing disorders; Narcolepsy; Pitolisant.

MeSH terms

  • Craniopharyngioma* / diagnosis
  • Craniopharyngioma* / diagnostic imaging
  • Disorders of Excessive Somnolence*
  • Humans
  • Pituitary Neoplasms* / diagnosis
  • Pituitary Neoplasms* / diagnostic imaging
  • Sleep
  • Sleep Wake Disorders* / etiology