The Journal of Association of Chest Physicians

: 2016  |  Volume : 4  |  Issue : 1  |  Page : 18--20

Neurogenic pulmonary edema in a child with status epilepticus

Bindu T Nair, Sajith Surendran, Dinesh Yadav 
 Department of Paediatrics, Army College of Medical Sciences, New Delhi, India

Correspondence Address:
Bindu T Nair
Department of Paediatrics, Army College of Medical Sciences, Delhi Cantonment, New Delhi - 110 010


Neurogenic pulmonary edema (NPE) is defined as acute pulmonary edema after a sudden neurologic insult. It develops after a significant central nervous system insult such as trauma, hemorrhage or seizures and can occur both in adults and children. A 6-year-old male child, known case of cerebral palsy was brought to the emergency department in status epilepticus. He had severe respiratory distress with pink frothy secretions pouring from the mouth. Clinical and radiological examination was suggestive of NPE. Child was immediately ventilated and all supportive measures were started. Child showed marked improvement within 48 h of admission with diuresis and positive end-expiratory pressure (PEEP) assisted ventilation.

How to cite this article:
Nair BT, Surendran S, Yadav D. Neurogenic pulmonary edema in a child with status epilepticus.J Assoc Chest Physicians 2016;4:18-20

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Nair BT, Surendran S, Yadav D. Neurogenic pulmonary edema in a child with status epilepticus. J Assoc Chest Physicians [serial online] 2016 [cited 2022 Jan 27 ];4:18-20
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Full Text


Neurogenic pulmonary edema (NPE) is defined as acute pulmonary edema occurring shortly after a central neurologic insult. It has been reported in various injuries of the central nervous system (CNS) in both adults and children. However, this condition remains poorly understood. Neurologic conditions that cause abrupt, rapid, and extreme elevation in intracranial pressure (ICP) appear to be at greatest risk of being associated with NPE.[1] There are few reports in adults with NPE due to sudden neurologic insult. There is no case reported in children. This may be due to the sporadic nature and relatively an unpredictable nature of the presentation and definitive lack of etiology.[2] It may also be due to lack of specific diagnostic markers for NPE.

 Case Report

A 6-year-old male child, an old case of cerebral palsy was brought to the Pediatric Emergency Department of our hospital with a history of abnormal tonic posturing of the body for past 5 h. Parents gave a history of upper respiratory tract infection associated with low-grade fever, coryza, and cough for past 2 days. There was no history of any seizure disorder, trauma, discharge from ear or any erythematous rash. There was no history of any previous structural heart disease or pulmonary disease.

On initial presentation, the child was unresponsive and cyanosed. The patient had a blood pressure of 64/40 mmHg, heart rate of 130 beats/min, respiratory rate of 36 breaths/min, and temperature of 37.0 degrees Celsius. The SpO2 was 50% in room air and capillary refill time was >5 s. Child had cold, clammy peripheries, and peripheral pulses were not palpable. There was no pitting edema. Copious frothy pink secretions started coming from the mouth. Physical examination revealed suprasternal and substernal recessions. Chest auscultation revealed bilateral crackles. Neurologic examination revealed spastic quadriparesis. Cardiovascular examination and abdominal findings were within normal. He was immediately intubated and connected to a mechanical ventilator. Child was started on inotropes, antiepileptics (injection fosphenytoin, injection levetiracetam, and injection midazolam infusion).

Chest radiograph revealed bilateral patchy infiltrates consistent with acute pulmonary edema [Figure 1]. Arterial blood gases (ABGs) obtained before intubation showed pH 7.04 (N - 7.35–7.45), PaCO2 74 mmHg (N - 35–45 mm Hg), PaO2 56 mm Hg (N - 80–100 mmHg), and HCO3 16.6 mEq/L (N - 20–28 mEq/L). This ABG is suggestive of severe acidemia with mixed acid-base disorder due to a combined respiratory acidosis caused by ventilatory failure and metabolic acidosis caused by lactic acidosis, which may be secondary to shock or hypoxemia.{Figure 1}

Complete blood count, liver function tests, renal function tests, and serum electrolytes were all within normal limits. Other significant laboratory findings were creatinine kinase (CK) 230 U/L (normal - 39–308 U/L), CK-MB 135 U/L (normal - 7–25 U/L), and troponin-I 0.02 ng/mL (normal < 0.05 ng/mL). Electrocardiogram and two-dimensional echocardiogram were normal. Throat swab for H1N1 virus was negative as there was an epidemic during that period. A noncontrast enhanced brain computed tomography was performed which was reported as normal.

Screening test for poisons or drugs was not performed because history was not suggestive of intoxication. Other emergencies such as diabetic ketoacidosis and nonketotic hyperosmolar coma were excluded by normal blood osmolarity and the absence of ketone bodies in blood and urine. His ABGs were rapidly normalized by using PEEP-assisted ventilation and diuretics. With appropriate diuresis and mechanical ventilation, the patient's respiratory distress resolved within 48 h. The patient was discharged after 5 days and on follow-up has been found to have normal cardiac and pulmonary functions.


Neurogenic pulmonary edema can result from various CNS disorders such as brain malignancies, traumatic brain injuries, infections, and seizures. Epileptic seizures are also associated with NPE.[3] NPE was first reported following seizure activity in 1908. NPE following seizure remains an under-recognized diagnosis with a poorly understood pathogenesis.[4] Neurologic insults that cause sudden and severe increase in ICP are at greatest risk for NPE.[5]

Neurogenic pulmonary edema usually occurs within minutes to hours after a CNS insult.[6] An autopsy study found that 87% of patients with epilepsy and unexplained sudden death had NPE. It is not certain whether NPE was the proximate cause of death in these studies. NPE due to epileptic seizures usually occurs during the postictal period.[7] Clinical symptoms and signs of NPE are nonspecific. Dyspnea, tachypnea, tachycardia, cyanosis, pink frothy sputum, basal pulmonary crackles and rales are usual features of NPE.

Certain regions of the brain are considered responsible for the genesis of sympathetic hyperactivity. These parts are insular cortex, hypothalamus, and medulla. Insular cortex lesion is associated with both NPE and cardiac abnormality (stunned myocardium). Hypothalamus and medulla lesions are more likely to cause isolated NPE.[8] Following a neurological insult, these cases are characterized by an increase in extravascular lung fluid. There is a rapid accumulation of protein-rich fluid in the alveoli.[9] The mechanism for NPE is because of increased pulmonary capillary permeability combined with massive centrally mediated sympathetic discharge resulting in elevated pulmonary vascular resistance. This in turn leads to cardiopulmonary dysfunction.[10]

Clinical examination, prompt imaging, laboratory studies, and a high index of suspicion can help to establish the correct diagnosis. Chest X-ray typically shows bilateral diffuse alveolar infiltrates. With appropriate treatment, NPE often resolves within 48–72 h.[11] Diagnosis of NPE is difficult because of its relatively unpredictable nature. Besides, there is a lack of specific signs and diagnostic tests. Differential diagnoses include aspiration pneumonia, congestive heart failure, acute respiratory distress syndrome, and ventilation-induced lung injury.

The initial management should focus on treatment of the underlying neurological insult. Most NPE patients require supplemental oxygen or mechanical ventilation. The treatment is mainly intended toward hemodynamic and respiratory support. Mechanical ventilation is recommended to provide appropriate oxygenation.[12] Positive end-expiratory pressure (PEEP) should be judiciously applied because of hemodynamic consequences such as increased intra-thoracic pressure and reduced venous return. This in turn may result in hypotension and worsen cerebral perfusion. Several medications like alpha-adrenergic blockers have been used to treat NPE, but their efficacy has not been established.[13]

To conclude, even though this syndrome has been described for over a millennium, it remains underdiagnosed and underappreciated. NPE is a rapidly developing and life-threatening complication of CNS insult. Physicians should remember this clinical entity when caring for patients with acute respiratory distress following neurologic events to avoid misdiagnosis and unfavorable outcomes.


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