C. Rabec et al., Central sleep apnoea in Arnold-Chiari malformation: evidence of pathophysiological heterogeneity, EUR RESP J, 12(6), 1998, pp. 1482-1485
We report on the case of two young patients with type I Arnold-Chiari malfo
rmation (ACM), as revealed IF a central sleep apnoea (CSA) syndrome without
any other neurological defect.
Case 1 was a 14-yr-old male patient, who developed severe alveolar hypovent
ilation and needed long-term mechanical ventilation via,a tracheostomy.
Case 2 was a 39-yr-old male patient, who developed features suggestive of s
leep apnoea and responded to nasal continuous positive airway pressure vent
ilation despite the central type of apnoeas.
Those two cases illustrate the different pathophysiological mechanisms invo
lved in CSA, namely a blunted chemical drive (in hypercapnic patients) and
an increased chemical drive, which destabilizes the breathing pattern durin
g sleep (in normo/hypocapnic patients),
Central sleep apnoea can be the initial manifestation of Arnold-Chiari malf
ormation and can lead to a life-threatening condition.