Alveolar Hypoventilation Syndrome in Brainstem Glioma With Improvement After Surgical Resection

Hui, Shuk Yi Annie; Wing, Yun Kwok; Poon, Wai-sang; Chan, Y. L.; Buckley, Tom

doi:10.1378/chest.118.1.266

Cited by 35 publications

(15 citation statements)

References 6 publications

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“…Watershed infarcts in the brainstem tegmentum in the human fetal and neonatal brainstem can present with multiple cranial neuropathies, central hypoventilation and apnea, dysphagia and aspiration, Möbius syndrome, and Pierre Robin sequence 91. Tumors in the region of the brain stem, such as glioma and acoustic neuroma, can lead to central hypoventilation 92,93. Children with brain tumors often report sleepiness that may be related to central apneas or hypoventilation 94.…”

Section: Other Acquired Alveolar Hypoventilation Disordersmentioning

confidence: 99%

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Central Alveolar Hypoventilation Syndromes

Muzumdar

Arens

2008

Sleep Medicine Clinics

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KeywordsCentral alveolar hypoventilation; Congenital central hypoventilation syndromeThe ventilatory control system is tightly regulated. Three elements known to regulate this system include (1) sensors, such as peripheral and central chemoreceptors, and mechanoreceptors; (2) central controllers that receive input and integrate the response from the above sensors; and (3) effectors, including the muscles of respiration, that respond to the commands of the central controllers.Thus, alveolar ventilation is regulated most of the time involuntarily by the respiratory centers located beneath the ventral surfaces of the pons and medulla, that receive afferent input from the sensors and control ventilation automatically (chemically) via the respiratory muscles. This chemical control regulates breathing during non-rapid eye movement (NREM) sleep. Voluntary control by cortical centers can also occur, particularly during wakefulness and during rapid eye movement (REM) sleep.Central alveolar hypoventilation disorders denote conditions resulting from underlying neurologic disorders affecting the sensors, the central controller, or the integration of the signals. Such disorders can lead to insufficient ventilation and an increase in PaCO 2 (hypercarbia), as well as a decrease in PaO 2 (hypoxemia). The condition may be congenital or acquired, and affected children may be at risk from the neonatal period. Central alveolar hypoventilation may be present during sleep alone or in more severe cases during sleep and wakefulness.It is important to make an early diagnosis of central hypoventilation to prevent the deleterious effects of hypercapnia, acidosis, and hypoxemia on cardiovascular and neurocognitive function. This review discusses the current knowledge on central alveolar hypoventilation syndromes, particularly in children, with special emphasis describing the recent knowledge about congenital central hypoventilation syndrome (CCHS) and rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD). CONGENITAL ALVEOLAR HYPOVENTILATION DISORDERS Congenital Central Hypoventilation SyndromeCCHS or congenital central alveolar hypoventilation syndrome has also been referred to as Ondine's curse and has been recognized in the literature for more than 30 years.1 Ondine's curse (also known as Undine) was referenced to the story of a water sprite from European lore who cursed her unfaithful lover to lose all automatic functions and therefore stop breathing * Corresponding author: rarens@montefiore.org (R. Arens). NIH Public Access NIH-PA Author ManuscriptNIH-PA Author Manuscript NIH-PA Author Manuscript when he fell asleep. This rather apt description of the plight of subjects with CCHS has now become a part of medical mythology, but does not accurately reproduce the fable of Ondine. 2 The identification of associated features including Hirschsprung's disease, which is considered a neurocristopathy or developmental anomaly of the neural crest,3 neural crest tumors, and autonomic nervous system (...

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Section: Other Acquired Alveolar Hypoventilation Disordersmentioning

confidence: 99%

“…Children with brain tumors often report sleepiness that may be related to central apneas or hypoventilation 94. Hypoventilation may improve after tumor resection 93. Bulbar polio and viral and paraneoplastic encephalitis have can occasionally result in central hypoventilation 95–97.…”

Section: Other Acquired Alveolar Hypoventilation Disordersmentioning

confidence: 99%

Central Alveolar Hypoventilation Syndromes

Muzumdar

Arens

2008

Sleep Medicine Clinics

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“…Acquired central hypoventilation, leading to loss of automatic respiration with preserved voluntary breathing (Ondine’s curse), occurs with medullary lesions, usually ischaemic infarctions,1 Chiari malformation,2 brainstem glioma3 or cervical spinal damage 4. Although central hypoventilation related to anti-Hu associated paraneoplastic encephalomyelitis has been reported,5 6 the frequency of this complication among these patients is not known.…”

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confidence: 99%

Central hypoventilation as the presenting symptom in Hu associated paraneoplastic encephalomyelitis

Gómez-Choco

Zarranz

Sáiz

et al. 2007

Journal of Neurology, Neurosurgery & Psychiatry

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“…Sleep related central hypoventilation represents a separate type of sleep related breathing disorder that often cooccurs with CSA and arises through similar mechanisms [8,37,77]. Like CSA, it is usually reported in relation to tumors of the brain stem and posterior fossa [8,76,78,79].…”

Section: Sleep Related Breathing Disordersmentioning

confidence: 99%

“…Treatment of CSA and sleep-related hypoventilation is often challenging. Surgical tumor resection may lead to improvement in symptoms but does not guarantee complete resolution [78]. Patients may require long-term positive pressure ventilation during sleep, either non-invasively using bilevel positive pressure ventilation (BPAP) or invasively via tracheostomy and home mechanical ventilation.…”

Section: Sleep Related Breathing Disordersmentioning

confidence: 99%

Sleep Disorders in Children with Central Nervous System Tumors

Maloney¹,

Lewinter²,

Ward³

et al. 2018

obm neurobiol

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Sleep complaints are common in pediatric patients with central nervous system (CNS) tumors. These problems may result from disruption of normal homeostatic, circadian, neuroendocrine, and cardiorespiratory pathways and vary by tumor location and treatment received. Children with tumors within the hypothalamus and surrounding regions are prone to excessive daytime sleepiness. Sleep-related breathing disorders, especially those involving abnormal control of breathing, may occur in patients with tumors of the brainstem and posterior fossa. Maintaining a high index of suspicion for sleep disorders in children with CNS tumors is essential for early recognition and treatment. In this article, we will review the various sleep problems reported in pediatric brain tumor survivors, explore underlying neurobiological mechanisms, and discuss approaches to screening and diagnosis.Sleep is an essential physiologic function for growth, cognition, and physical and emotional well-being. It is particularly important in childhood, when the brain and body are undergoing rapid development. Normal sleep occurs through a complex interplay between homeostatic, circadian, neuroendocrine, and cardiorespiratory pathways. Cancer and cancer treatment may cause disruptions and imbalances in these pathways, resulting in abnormal sleep. Compared to the general population, pediatric cancer survivors are more likely to experience sleep problems. Patients with a history of brain tumors are particularly prone to sleep issues. In this article, we will review the physiology of normal sleep, describe the sleep problems and pathophysiologic mechanisms behind disrupted sleep in children with central nervous system (CNS) tumors, and discuss strategies for screening, diagnosis, and management. Sleep Mechanisms and PhysiologySleep and wakefulness. Sleep is fundamentally important in promoting growth, repair, and neurocognitive functioning. The timing, duration, and quality of sleep depend on complex, balanced interactions between sleep-promoting and wake-promoting pathways. The majority of these pathways are housed in the basal forebrain, diencephalon, and brainstem. The balance between sleep and wakefulness is governed by two distinct biologic processes: sleep-wake homeostasis (Process S) and circadian rhythm (Process C) [1]. Homeostatic drive to sleep intensifies with continued wakefulness over time and subsides as an individual sleeps. During the day, the sleep-dependent homeostatic drive is counteracted by sleep-independent circadian arousal mechanisms. Circadian rhythms are coordinated by an internal pacemaker housed in the bilateral suprachiasmatic nuclei (SCN) of the anterior hypothalamus [1]. The near-24 hour circadian rhythm is externally entrained by the light-dark cycle on Earth through direct input received by the SCN from retinal ganglion cells via the retinohypothalamic tract, which runs through the optic chiasm [2,3]. Additional input from the intergeniculate leaflet and median raphe nucleus modulate circadian phase shifts [3]. The SCN sends...

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Alveolar Hypoventilation Syndrome in Brainstem Glioma With Improvement After Surgical Resection

Cited by 35 publications

References 6 publications

Central Alveolar Hypoventilation Syndromes

Central Alveolar Hypoventilation Syndromes

Central hypoventilation as the presenting symptom in Hu associated paraneoplastic encephalomyelitis

Sleep Disorders in Children with Central Nervous System Tumors

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