1/5. Arnold Chiari type 1 malformation presenting with sleep disordered breathing in well children.Healthy children, aged 3, 9, and 13 years referred with sleep disordered breathing had marked central apnoea and bradypnoea on polysomnography, necessitating the use of non-invasive bilevel ventilation in two cases. Each had normal neurological examinations and an Arnold Chiari type 1 malformation was confirmed on magnetic resonance imaging. All underwent urgent posterior fossa decompression which normalised their sleep breathing.- - - - - - - - - - ranking = 1keywords = apnoea (Clic here for more details about this article) |
2/5. Obstructive sleep apnoea with arnold-chiari malformation.A case of obstructive sleep apnoea associated with the arnold-chiari malformation is described, in which the loss of pharyngeal sensation seems to have played an important part in the aetiology of the obstruction of the upper airway.- - - - - - - - - - ranking = 5keywords = apnoea (Clic here for more details about this article) |
3/5. Acute respiratory failure as the first sign of arnold-chiari malformation associated with syringomyelia.We report a rare case of acute respiratory failure in a previously asymptomatic patient showing clinical signs of inferior cranial nerve palsy together with weakness and muscular atrophy of the upper limbs. magnetic resonance imaging revealed arnold-chiari malformation associated with platybasia, basilar impression, syringomyelia and klippel-feil syndrome. Episodes of apnoea required tracheostomy and recurred upon tentative closure of the tracheostome, but remitted upon decompression of the posterior fossa. This case involved both obstructive mechanisms and dysfunction of the respiratory centre. patients with respiratory failure not explained by pulmonary pathology should be checked for underlying neurological disease.- - - - - - - - - - ranking = 1keywords = apnoea (Clic here for more details about this article) |
4/5. Chronic respiratory failure in a patient with type I arnold-chiari malformation (ACM1) and syringomyelia.We present the case of a young adult with type I arnold-chiari malformation (AMC1) and syringomyelia who developed central sleep apnoea and chronic respiratory failure, successfully treated with nocturnal noninvasive positive pressure ventilation ventilation (NIPPV). An extensive syringomyelic cavity (from bulbar to L4 segment) with severe impairment of the IX cranial nerve was documented and remains, although reduced, after the neurosurgical treatment. At baseline evaluation, the patient showed a moderate restrictive ventilatory defect, severe hypercapnic respiratory failure, abnormal control of breathing characterized by the absence of response to hypoxia and hypercapnia, and severe nocturnal central apnoeas. Nocturnal NIPPV was then started in the A/C mode with an improvement in blood gas values. Further evaluations were performed 10 and 18 months later. A progressive significant improvement of lung volumes, both in sitting and supine position, associated with a slight improvement of blood-gas values were observed. Nonetheless, the breathing pattern abnormalities persisted. Polysomnographic evaluation during mechanical ventilation showed a normalization of breathing pattern with arterial oxygen saturation (SaO2) > 90% throughout the night.- - - - - - - - - - ranking = 2keywords = apnoea (Clic here for more details about this article) |
5/5. Central sleep apnoea in arnold-chiari malformation: evidence of pathophysiological heterogeneity.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.- - - - - - - - - - ranking = 8keywords = apnoea (Clic here for more details about this article) |