Shahar Eli, Postovsky Sergey, Bennett Odeya
Child Neurology Unit and Epilepsy Service, Haifa, Israel.
Pediatr Neurol. 2004 Apr;30(4):287-90. doi: 10.1016/j.pediatrneurol.2003.10.003.
Central neurogenic hyperventilation refers to progressive tachypnea leading to hypocarbia and respiratory alkalosis caused by cortical disorders, initially reported in comatose patients with mainly pontine infarction. Central neurogenic hyperventilation in conscious patients is even rarer, numbering around 30 reported cases including seven children, mainly associated with infiltrative gliomas and lymphomas of the brainstem and pons. We report the evolution of central neurogenic hyperventilation in a conscious child associated with an infiltrative glioblastoma multiforme diagnosed 1 year before admission. He presented with progressive tachypnea and dyspnea of 1 week duration. On examination he was fully alert and aware of his respiratory disorder. Respiratory rate was 56 breaths per minute using accessory respiratory muscles. Hyperventilation was unchanged during sleep. Arterial blood gases disclosed marked hypocarbia: Pco(2) of 8 mm Hg resulting in severe respiratory alkalosis at pH of 7.8. Central neurogenic hyperventilation was therefore suggested after exclusion of other respiratory or cardiac disorders. The exaggerated tachypnea persisted along with respiratory alkalosis. Over a period of 2 months his overall state markedly deteriorated; he lapsed into coma, and finally succumbed after involvement of medullary cardiovascular centers. Although extremely rare in the pediatric age group, central neurogenic hyperventilation should be suspected in any alert child presenting with unexplained increasing tachypnea and hypocarbia leading to respiratory alkalosis. The evolution of such a disorder may be an alarming sign of ensuing deterioration in patients with tumors of the brainstem and medulla before cardiovascular derangement.
中枢性神经源性通气过度是指由皮质疾病引起的进行性呼吸急促,导致低碳酸血症和呼吸性碱中毒,最初报道于主要为脑桥梗死的昏迷患者。意识清醒患者出现中枢性神经源性通气过度更为罕见,报道病例约30例,其中包括7名儿童,主要与脑干和脑桥的浸润性胶质瘤和淋巴瘤有关。我们报告了一名意识清醒儿童的中枢性神经源性通气过度的病情演变,该儿童在入院前1年被诊断为浸润性多形性胶质母细胞瘤。他出现了持续1周的进行性呼吸急促和呼吸困难。检查时,他完全清醒,意识到自己的呼吸障碍。使用辅助呼吸肌时,呼吸频率为每分钟56次。睡眠期间通气过度无变化。动脉血气显示明显的低碳酸血症:二氧化碳分压为8毫米汞柱,导致pH值为7.8的严重呼吸性碱中毒。因此,在排除其他呼吸或心脏疾病后,提示为中枢性神经源性通气过度。过度呼吸急促和呼吸性碱中毒持续存在。在2个月的时间里,他的整体状况明显恶化;陷入昏迷,最终在延髓心血管中枢受累后死亡。虽然在儿童年龄组中极为罕见,但对于任何出现不明原因的呼吸急促增加和低碳酸血症导致呼吸性碱中毒的清醒儿童,都应怀疑中枢性神经源性通气过度。这种疾病的演变可能是脑干和延髓肿瘤患者在心血管紊乱之前病情恶化的一个警示信号。