Chiari Malformation and Sleep-Disordered Breathing: A Review of Diagnostic and Management Issues

Abstract

Chiari Malformation (CM) encompasses several patterns of congenital or acquired cerebellar herniation through the foramen magnum. This may result in brain-stem compression that impacts control of breathing and is associated with obstructive and central apneas. A high clinical suspicion for sleep-disordered breathing is needed in the care of such patients after as well as before corrective surgery. To introduce a review of CM with a focus on the relevance to sleep medicine, we present a case of a 13-yearold female who was diagnosed with CM Type 1 in the course of an evaluation of symptomatic central sleep apnea. After initial improvement following surgery there was recurrence of brain-stem compression. The only clinical expression of which was polysomnographically evident recurrence of sleep apnea.

Obstructive and central sleep apnoea in Arnold–Chiari malformation: resolution following surgical decompression

“…There are several hypotheses regarding the possible causes of central sleep apnoea. Physical compression of the brainstem may compress the respiratory centre in the medulla and/or the reticular activating system [8]. The response to decompressive surgery as observed by us and reported by others [1,8] would be consistent with this hypothesis.…”

“…Physical compression of the brainstem may compress the respiratory centre in the medulla and/or the reticular activating system [8]. The response to decompressive surgery as observed by us and reported by others [1,8] would be consistent with this hypothesis. Patients with ACM may also have impaired peripheral chemosensitivity [9], possibly due to stretching of the glossophar- [3].…”

Sleep apnea and REM sleep behavior disorder in patients with Chiari malformations

“…Abnormal respiratory events have been previously described in CM 10,27,31 but there is poor information regarding the associate presence of RBD in this disorder and, as far as we know (aside from the isolated case reported by lapierre and Montplaisir 9 , that suggested the dysfunction of lower brain stem neural structures responsible for muscle atonia as a possible cause), RBD has not been previously mentioned as a possible consequence of CM. Sforza et al 24 , in an extensive review of RBD clinical and physiopathological findings, stated that the association between sleep apnea and RBD is uncommon, and when present, the apneas are essentially of the obstructive type.…”

“…These neurons are represented by the "pontis oralis", "pontis caudalis" and magnocelular nucleous, located in the reticular formation in the pons tegment. There is another subgroup with the same function, the subgroup alpha of the locus coeruleus located in the ventral and medial region of the medulla oblongata, which could be related to this brain stem dysfunction [27][28][29] .…”

Zentrale Schlafapnoe –Pathophysiologie, Relevanz, Diagnostik und Therapie

“…Diese sind mitunter einziges Zeichen der zugrunde liegenden Hirnstammsymptomatik, bessern sich nach operativer Behandlung und deuten bei Wiederauftreten auf eine erneute Hirnstammkompression hin [10, 20,38,95].…”