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We report a case of a 34 year old right handed woman seen at 29 weeks’ gestation who suffered from apnoea of unknown aetiology. This pregnancy, as well as her first gestation, was complicated by generalised oedema and high blood pressure. Starting at week 25, her husband noticed she had developed intermittent brief periods of apnoea only while sleeping, which lasted as long as one minute but of variable duration. Her husband awakened her each time she had a protracted episode of apnoea. She was asymptomatic while awake. In the 29th week she suffered a more severe apnoea. She was intubated in the field and taken to the hospital for an emergency caesarean section. There was no spontaneous labour. Inability to breathe spontaneously persisted for two weeks postpartum and a neurological consultation was requested.
On initial evaluation blood pressure was 140/90 and the heart rate was 90 beats/min. The neurological examination revealed upbeat nystagmus of small amplitude in the primary position which did not change with upward or downward gaze. She had lack of spontaneous breathing. She was fully awake and cooperative, sitting up in bed with no assistance. While intubated she had an obvious cough reflex but the gag reflex was not formally tested. Tongue examination showed normal movement and power with no evidence of atrophy or fasciculation. Otherwise, cranial nerve and sensorimotor examinations were entirely normal. There was normal tone, with downgoing plantar reflexes and no evidence of other pyramidal findings. There was no record of arrhythmia. No yawning, vomiting, or hiccups were present during the examination and they were not seen by nursing staff.
It was of interest that this patient had suffered from apnoea presenting immediately after her first vaginal delivery two years previously. This was treated with intubation and resolved spontaneously with successful extubation approximately four hours later.
Magnetic resonance imaging of the brain, brain stem, and cervical spinal cord was carried out, and showed a Chiari malformation with tonsillar herniation at C2 level and a cervical syrinx.
After posterior cranial fossa decompression the patient recovered spontaneous breathing and was off any ventilatory support within six hours. She and her child have been followed for two years following this event. She remains in good health with normal polysomnographic testing.
Ondine’s curse is a rare form of central respiratory failure which includes severe sleep apnoea.1 We describe the case of recurrent sleep apnoea associated with pregnancy and delivery and related to a Chiari malformation that became symptomatic only during pregnancy. We hypothesise that the generalised oedema that occurred during pregnancy, with a potentially mild increase in intracranial pressure, produced dysfunction of central chemoreceptors or the respiratory integrative system of the brain stem. Additionally there may have been compression of the lower cranial nerves, impairing input from peripheral chemoreceptors.2 Related to this, our patient suffered severe sequelae from a central malformation that was previously clinically inapparent. Although Ondine’s curse has rarely occasionally described in patients with Chiari malformation,3 the expression of secondary sleep apnoea uniquely during pregnancy has not been reported before. Finally we wish to highlight the complete recovery of this patient after posterior fossa decompression, as we found in another published case.4
Competing interests: none declared
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