Morelli Céline, Aeby Alec, Scaillet Sonia, Boitsios Grammatina, Vens Daphné, Prigogine Cynthia, Biarent Dominique, Vicinanza Alfredo
Department of Pediatrics, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles (ULB), Brussels, Belgium.
Division of Pediatric Neurology, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles (ULB), Brussels, Belgium.
Front Pediatr. 2020 Sep 25;8:547474. doi: 10.3389/fped.2020.547474. eCollection 2020.
Neuromyelitis optica spectrum disorder is a rare, relapsing autoimmune disease of the central nervous system. Various initial presentations can delay diagnosis and treatment. A 14-year-old girl was admitted to the emergency department owing to respiratory insufficiency. Repeated history-taking and neuroimaging revealed an area postrema syndrome. A diagnosis of neuromyelitis optica spectrum disorder with positive aquaporin-4 antibodies has finally been established. The patient improved significantly with immunosuppressive therapy. However, her 3-year follow-up still showed sleep-disordered breathing requiring nocturnal bilevel positive airway pressure therapy. We report an original case of NMOSD leading to persistent central sleep apnea syndrome.
视神经脊髓炎谱系障碍是一种罕见的、复发性的中枢神经系统自身免疫性疾病。各种初始表现可能会延迟诊断和治疗。一名14岁女孩因呼吸功能不全被收入急诊科。反复询问病史和神经影像学检查显示为最后区综合征。最终确诊为水通道蛋白-4抗体阳性的视神经脊髓炎谱系障碍。患者经免疫抑制治疗后明显好转。然而,她3年的随访仍显示存在睡眠呼吸紊乱,需要夜间双水平气道正压通气治疗。我们报告了一例导致持续性中枢性睡眠呼吸暂停综合征的视神经脊髓炎谱系障碍的原始病例。