Central Hypoventilation Syndrome
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Clinic
- CHS is a sleep-related breathing disorder that causes ineffective breathing, apnea, or respiratory arrest during sleep (and during wakefulness in severe cases).
- CHS can either be congenital or acquired later in life.
- The condition can be fatal if untreated.
- CCHS was once known as Ondine's curse.
- Acquired CHS can develop as a result of severe injury or trauma to the brain or brainstem.
Signs and symptoms
- Darkening of skin color from inadequate amounts of oxygen,
- Drowsiness, fatigue, headaches, and an inability to sleep at night.
- Sensitivity to sedatives and narcotics, which makes respiration even more difficult. A low concentration of oxygen in the red blood cells also may cause hypoxia-induced pulmonary vasoconstriction and pulmonary hypertension, culminating in cor pulmonale or a failure of the right side of the heart.
Related Disease / Entities
- CHS is associated with respiratory arrests during sleep and, in some cases, to neuroblastoma (tumors of the sympathetic ganglia)
- Hirschsprung disease
- Dysphagia
- Anomalies of the pupilla
- Gastro-esophageal reflux
- Ophthalmologic issues
- Seizures
- Recurrent pneumonia
- Developmental delays, learning disabilities
- Episodes of fainting
- Temperature dis regulation
Causes
- Severe brain or spinal trauma or injury
- Particular neurodegenerative conditions such as Parkinson's disease, MSA, or MS.
- CHS used to be classified as a "neurocristopathy", or disease of the neural crest because part of the autonomic nervous system (such as sympathetic ganglia) derives from the neural crest.