"Nocturnal seizures" in idiopathic pulmonary arterial hypertension

Anthony Izzo1, Julia McSweeney, Thomas Kulik

  • 1Sleep Disorders Center of New England, St. Vincent Hospital, Worcester, MA ; Center for Pediatric Sleep Disorders, Boston Children's Hospital, Harvard Medical School, Boston, MA.

Insights

Idiopathic pulmonary arterial hypertension (IPAH) can mimic nocturnal seizures in children. Sleep physicians should consider IPAH in the differential diagnosis of unexplained nocturnal events.

Area of Science:

  • Pediatric Sleep Medicine
  • Pediatric Cardiology
  • Pulmonology

Background:

  • Nocturnal events in children often prompt differential diagnoses including parasomnias and seizures.
  • Accurate diagnosis is crucial for appropriate management of pediatric sleep disturbances.

Observation:

  • A previously healthy young girl presented with nocturnal events initially diagnosed as medically refractory nocturnal seizures.
  • A daytime syncopal event led to cardiac evaluation, revealing idiopathic pulmonary arterial hypertension (IPAH).

Findings:

  • The patient's nocturnal events were ultimately attributed to undiagnosed IPAH, not seizures.
  • This case highlights a potential diagnostic challenge where cardiac conditions mimic primary sleep disorders.

Implications:

  • Sleep physicians should broaden the differential diagnosis for nocturnal events in children to include conditions like IPAH.
  • Early consideration of IPAH can prevent misdiagnosis and delayed treatment of serious cardiopulmonary disease.
  • Integrating cardiac evaluation into the workup of refractory nocturnal events may be warranted.

Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
316
Seizures: Classification01:13

Seizures: Classification

Epilepsy is primarily characterized by unpredictable seizures, either provoked by an identifiable factor, such as injury or illness, or unprovoked, occurring spontaneously without apparent cause.
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
656
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers01:26

Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers

Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
277
Sleep Apnea01:21

Sleep Apnea

Sleep apnea is a condition where breathing stops intermittently during sleep, often leading to significant health issues. Each episode can last from 10 to 20 seconds or more and is frequently accompanied by a brief arousal from sleep. This disturbance, largely unnoticed by the individual, can lead to severe daytime fatigue. Commonly, individuals seek help after being informed by their partners about loud snoring and noticeable breathing pauses during sleep.
The condition is more prevalent among...
235
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
339
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
238