RT Journal Article
SR Electronic
T1 Central sleep apnoea in Arnold-Chiari malformation: evidence of pathophysiological heterogeneity
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1482
OP 1485
DO 10.1183/09031936.98.12061482
VO 12
IS 6
A1 Rabec, C
A1 Laurent, G
A1 Baudouin, N
A1 Merati, M
A1 Massin, F
A1 Foucher, P
A1 Brondel, L
A1 Reybet-Degat, O
YR 1998
UL https://publications.ersnet.org//content/12/6/1482.abstract
AB We report on the case of two young patients with type I Arnold-Chiari malformation (ACM), as revealed by a central sleep apnoea (CSA) syndrome without any other neurological defect. Case 1 was a 14-yr-old male patient, who developed severe alveolar hypoventilation and needed long-term mechanical ventilation via a tracheostomy. Case 2 was a 39-yr-old male patient, who developed features suggestive of sleep apnoea and responded to nasal continuous positive airway pressure ventilation despite the central type of apnoeas. These two cases illustrate the different pathophysiological mechanisms involved in CSA, namely a blunted chemical drive (in hypercapnic patients) and an increased chemical drive, which destabilizes the breathing pattern during sleep (in normo/hypocapnic patients). Central sleep apnoea can be the initial manifestation of Arnold-Chiari malformation and can lead to a life-threatening condition.