It has been previously reported that NPE can be considered an exotic form of ARDS[3]. Although the exact mechanisms responsible for the development of NPE are not entirely understood, the proposed etiology involves the release of vasoactive substances and a rapid massive sympathetic discharge after a sudden increase of intracranial pressure (ICP) by severe central nervous system insult[5, 6].
The clinical presentation of neurogenic NPE are non-specific and often include dyspnea, tachypnea, tachycardia, cyanosis, pink frothy sputum, and bilateral crackles and rales on auscultation. The chest radiograph or CT scan shows diffuse bilateral hyperintensive infiltrates. It has been described that there are two forms of NPE. The early form of NPE that develops within minutes to hours following neurologic injury is most common and a delayed form that develops 12 to 24 hours after the CNS insult[7]. Clinically, the possibility of developing NPE after aneurysmal SAH correlates with increasing the severity of clinical and radiographic presentation[8]. Patients with NPE have a higher incidence of vasospasm and mortality rate compared with patients without NPE [2, 9].
The key to the treatment of NPE is to control its underlying central neurologic insult in order to reduce the sympathetic discharge responsible for causing the lung injury [3]. Tracheal intubation, controlled ventilation with supplemental oxygen, and application of moderate PEEP are critical strategies to improve the pulmonary status in the acute stage[10]. In addition to these conventional treatments, it has been reported a woman with fulminant NPE following an aneurysmal SAH successfully treated by extracorporeal membrane oxygenation (ECMO) [11]. It has been reported that NPE may resolve in 48-72 hours with appropriate treatment, and patient’s prognosis dependents on the neurological condition[12]. In this case report, the patient was successfully treated with PEEP and early endovascular intervention, although NPE was developed rapidly and fatally after admission. Therefore, we believe that timely identification of NPE, early respiratory management and endovascular intervention at acute stage is essential to avoid poor clinical outcomes.