Acquired central hypoventilation syndrome

Acquired Central Hypoventilation Syndrome (ACHS), also known as Ondine's Curse, is a rare respiratory disorder that affects the control of breathing, causing individuals to hypoventilate, particularly during sleep.

Overview

ACHS is characterized by the body's inability to control respiration autonomously. This means that affected individuals must consciously control their breathing. When they fall asleep or lose consciousness, their breathing can become shallow or even stop, leading to life-threatening consequences.

Causes

The exact cause of ACHS is not well understood. However, it is believed to be due to damage or dysfunction in the brainstem, which is responsible for controlling automatic functions such as breathing. This damage can be caused by various factors, including brain injury, stroke, tumor, or neurodegenerative diseases.

Symptoms

The primary symptom of ACHS is hypoventilation, particularly during sleep. Other symptoms can include daytime sleepiness, headaches, shortness of breath, and cyanosis (bluish color of the skin due to lack of oxygen). In severe cases, ACHS can lead to respiratory failure or cardiac arrest.

Diagnosis

Diagnosis of ACHS is often challenging due to its rarity and the nonspecific nature of its symptoms. It typically involves a thorough medical history, physical examination, and various tests such as polysomnography (sleep study), blood gas analysis, and neuroimaging studies.

Treatment

Treatment for ACHS is primarily supportive and aims to ensure adequate ventilation, particularly during sleep. This often involves the use of mechanical ventilation or positive pressure ventilation devices. In some cases, tracheostomy may be required.

See also

• Central hypoventilation syndrome
• Congenital central hypoventilation syndrome
• Respiratory failure
• Sleep apnea

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