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CENTRAL NERVOUS SYSTEM GANGLIOGLIOMA PRESENTING WITH SEVERE CENTRAL AND OBSTRUCTIVE SLEEP APNEA IN A CHILD: A CASE REPORT

RESUMO

Case Presentation: we report the case of a four-year-old girl who has experienced, according to her mother, snoring, breathing pauses lasting for 8-10 seconds, and a breathing pattern suggestive of Cheyne-Stokes ventilation during sleep since she was two years old. At the age of three, she presented headaches, vomiting and mental confusion, being diagnosed with Grade I ganglioglioma in brainstem and cerebellum. She was submitted to tumor resection and maintained under vinblastine chemotherapy. The sleep complaints got progressively worse, and she was referred to evaluation in the Neurology Sleep Medicine service. This child was obese (BMI = 37.6 kg/m²), usually sleeping from 11 p.m. to 9 a.m., waking up multiple times to urinate, but without any complaints of daytime sleepiness. She also had a small oral cavity (Mallampati 4, Brodsky tonsil scale 3, and an ogival palate). Polysomnography (PSG) evidenced severe sleep apnea [Apnea Hypopnea Index (AHI) = 84.1 events/h] with both obstructive (AHI = 13.3 events/h) and central (AHI = 70.8 events/h) components. Baseline SatO2 was 90% and it decreased until 68% during apnea episodes. The central sleep apnea (CSA) showed up in the second half of the night, and Cheyne-Stokes breathing was observed. CPAP titration was effective to stop obstructive sleep apneas at the pressure of 8cm of H2O, yet central apneas remained. In this second PSG, CSA also appeared in the second part of the night.

Discussion: This case is about a rare condition, associated to severe central (also rare in children) and obstructive sleep apnea, not to mention the Cheyne-Stokes breathing. The prevalence of CSA is 4-6% in children with underlying conditions (using a cutoff > 5 events/h). Brainstem lesions may lead to several sleep modifications, such as reduced sleep efficiency, excessive daytime sleepiness, decreased stage 3 and REM sleep, and narcolepsy symptoms. These lesions might cause alveolar hypoventilation, having an impact on sleep, which may be severe enough to cause respiratory insufficiency and require mechanical ventilation, especially when lesions extend to the medulla. Brainstem lesions are a rare yet possible cause of CSA and the prevalence of central nervous system (CNS) tumors is higher in individuals with CSA when compared to controls.

Final Comments: The presence of brainstem lesions increases the risk of multiple sleep pathologies. CSA is a rare condition in children and CNS tumors should be investigated as a possible cause.

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