Sleep apnea and REM sleep behavior disorder in patients with Chiari malformations

Abstract

-Background: Chiari malformations (CM) may result in the appearance of REM sleep behavior disorder (RBD) and sleep apnea syndrome (SAS) that can be considered markers of brain stem dysfunction. Purpose: To evaluate the frequency of RBD and SAS in patients with CM type I and II. Method: Were evaluated 103 patients with CM by means of full night polysomnography. Were scoring different sleep stages, frequency of abnormal movements (through video monitoring) and abnormal respiratory events. Results: Of the 103 patients, 36 showed CM type I and 67 CM type II. Episodes of RBD were observed in 23 patients. Abnormal apnea-hypopnea index (AHI) was observed in 65 patients. Conclusion: The high rate of RBD suggests that this parassomnia and the increased frequency of central sleep apnea episodes, may be considered as a marker of progressive brain stem dysfunction.KEy WORDS: Chiari malformation, polysomnography, parasomnia, REM sleep behavior disorder, RBD, sleep apnea syndrome, SAS, central sleep apnea, obstructive sleep apnea. Apnéia do sono e distúrbio do comportamento da fase do sono com REM em pacientes com malformações de ChiariResumo -Introdução: Malformações de Chiari (MC) podem gerar o aparecimento de distúrbio comportamental da fase do sono com REM (DCR) e síndrome da apnéia do sono (SAS), sugerindo a ocorrência de disfunção do tronco cerebral. Objetivo: Avaliar a freqüência de DCR e SAS em pacientes com MC I ou II. Método: Utilizouse a polissonografia de noite inteira para a avaliação de 103 pacientes. Classificaram-se as diferentes fases do sono e analisou-se a freqüência de movimentos anormais (monitorada por vídeo) e de eventos respiratórios anormais. Resultados: Dos 103 pacientes analisados, 36 eram portadores de MC I e 67 de MC II. Episódios de DCR foram observados em 23 pacientes. O índice de apnéia/hipopnéia foi considerado anormal em 65 pacientes. Conclusão: A alta freqüência de DCR e o aumento da freqüência de episódios de apnéia central do sono podem ser considerados manifestação de disfunção progressiva do tronco cerebral.PAlAvRAS-CHAvE: malformação de Chiari, polissonografia, parassonia, distúrbio comportamental do sono REM, desordem respiratória do sono, apnéia central do sono, apnéia obstrutiva do sono.

Sudden unexpected nocturnal death in Chiari type 1 malformation and potential role of opioid analgesics

“…[1617] The most notable finding shared by the majority of these cases has been gross preservation of motor and sensory pathways, suggesting that a defect in the brainstem autonomic cardiorespiratory centers and inability of these centers to respond to typical homeostatic stress plays an important role in the etiopathology of sudden death associated with craniocervical junction disorders. [1415] Automatic respiration control, predominantly during sleep, depends on the activity of chemoreceptive neurons that respond to hypoxia, hypercapnia, and pH in the blood and cerebrospinal fluid (CSF) and is chemically regulated by both peripheral chemoreceptors and chemosensitive centers within the medulla. [3] With a partial impairment of the medullary chemosensitive centers, the stimulating action of carbon dioxide upon the brainstem respiratory center and ventilatory responses to hypercapnia and hypoxia-due to a greater hypercapnic ventilatory response during wakefulness as compared with sleep-remains preserved during alertness.…”

“…[381621] Nevertheless, ongoing compromises of autonomic centers in the brainstem related to the nonspecific structural lesions of the craniocervical junction can lead to a wide variety of autonomic disturbances including emesis, cardiorespiratory problems, sleep apnea, sudden death, and death during sleep. [48141621] Short and long-term opioid use is also associated with several potential adverse effects and toxicities, including respiratory depression and sleep-related breathing disorders, predominantly central sleep apnea. [2435] There is evidence that even addition of a small dose of opiates at night can precipitate central sleep apnea in patients who were receiving chronic opiate therapy without history of sleep apnea.…”

Sleep disorder: a possible cause of attention deficit in children and adolescents with Chiari malformation type II

“…In contrast, controls showed an increased frequency of obstructive apneas, predominantly caused by airway pathologies, allergic rhinitis being the most common (6/24). The increased frequency of sleep disorder in CM II patients, may be explained by the variable degree of brain stem abnormalities in CM II patients 21,22 , generally involving midbrain, pons and medulla oblongata, where are localized neurons of the respiratory regulation, and neurons of the REM sleep and of the movement control during sleep [23][24][25] . Analyzing these data, it is possible to conclude that sleep disorders are more prone to cause attention deficit than the presence of CM II alone, since in CM II patients without sleep disorders no attention deficit could be detected (Table 4).…”

Rapid Eye Movement Sleep Behavior Disorder During Childhood

“…From another standpoint, static neurodevelopmental disabilities such as autism, Moebius syndrome, and Smith Magenis syndrome; structural brainstem lesions such as neoplasms or Chiari type 1 malformation; narcolepsy; juvenile Parkinson disease; and the use of psychotropic medications predominate. [10][11][12][13][14][15][16][17] The common link between RBD of childhood and that of adults is the identical final common pathway leading up to event (ie, sleep state dissociation). 18 This is a process whereby elements of wakefulness such as tonic electromyographic activity, vocalizations, and bodily movement become superimposed on the phenomena of REM sleep.…”

“…A symptomatic Chiari type 1 malformation may be accompanied by dysphagia to liquids, paradoxic vocal cord motion on laryngoscopy, central sleep apnea on polysomnography, and MRI evidence of caudal displacement of the cerebellar tonsils in conjunction with brainstem compression. 12 In childhood narcolepsy-cataplexy, RBD may be the initial manifestation. 15 It may be accompanied by hypersomnolence, cataplexy, positive blood test for HLADQB1*0602, and low levels of spinal fluid hypocretin.…”