Sleep-Disordered Breathing in Chiari Malformation Type 1

ArticleinPediatric Neurology 39(3):207-8 · October 2008with11 Reads
DOI: 10.1016/j.pediatrneurol.2008.05.017 · Source: PubMed
Abstract
Type 1 Chiari malformation is defined as an elongation of the cerebellar tonsils >6 mm below the foramen magnum. Central sleep apnea is a well-recognized sign, and can be an initial presentation, of this malformation. Obstructive sleep apnea is not a widely recognized sign of Chiari type 1 malformation, though there were a few case reports. We present a 13-year-old girl who presented at our respiratory clinic with excessive nighttime snoring. Magnetic resonance imaging revealed a Chiari type 1 malformation requiring decompression. We emphasize the importance of including cervicomedullary junction disorders in the differential diagnosis of apnea, and we review the literature concerning mixed apneas and obstructive sleep apneas in Chiari type 1 malformation.
    • "A decision to proceed with sub-occipital decompression for relief of symptoms can therefore become subjective and vulnerable to bias of the treating neurologist or neurosurgeon [8]. Co-existing compression of the medullary respiratory control centers may lead to sleep related breathing disturbance in the form of central sleep apnea, obstructive sleep apnea or hypoven- tilation910111213. Sudden unexplained death has also been reported [14]. "
    [Show abstract] [Hide abstract] ABSTRACT: To evaluate nocturnal polysomnogram findings in children with suspected symptomatic Chiari type I malformation, correlate them with clinical and magnetic resonance imaging data and to determine if this information has value in clinical decision making process. A retrospective review identified 24 children with type I Chiari malformation, presumed symptomatic who had undergone neurological assessment, cranial magnetic resonance imaging and nocturnal polysomnography. Perimedullary subarachnoid space effacement on the magnetic resonance studies and the magnitude of cerebellar tonsillar descent in relation to the McRae line were correlated with frequency of obstructive or central sleep apnea, number of cortical arousals and evidence of impaired vocal mobility on laryngoscopy. The Wilcoxon rank sum test was applied for continuous variables and the Fisher exact test for categorical variables. The median age of the subjects was 6 years. The findings from 16/24 subjects with perimedullary subarachnoid space effacement (effaced group) were compared with those of 8/24 in the non-effaced group. The central apnea index [1.5 (IQR 1-3.5) versus 0.5 (IQR 0-1.5)] and cortical arousal index [12 (IQR 10-19) versus 8 (IQR 6.5-9)] were significantly higher in the effaced group than in the non-effaced group (p=0.0376 and 0.0036 respectively). Greater descent of tonsils as measured by distance from the McRae line to the tonsil tip was associated with significantly higher central apnea index, total arousal index and respiratory event related arousals. Measurements of clivus-canal angle, Klauss index and pB-C2 line did not correlate with abnormalities on polysomnography. The central apnea and arousal indices derived from the nocturnal polysomnogram correlate well with magnetic resonance imaging findings of subarachnoid space effacement and degree of tonsillar herniation. In children with Chiari type I malformation, the nocturnal polysomnogram findings provides important information that aids in the decision making process about proceeding with surgical decompression.
    Full-text · Article · Jun 2013
    • "A decision to proceed with sub-occipital decompression for relief of symptoms can therefore become subjective and vulnerable to bias of the treating neurologist or neurosurgeon [8]. Co-existing compression of the medullary respiratory control centers may lead to sleep related breathing disturbance in the form of central sleep apnea, obstructive sleep apnea or hypoven- tilation910111213. Sudden unexplained death has also been reported [14]. "
    [Show abstract] [Hide abstract] ABSTRACT: a b s t r a c t Objective: To evaluate nocturnal polysomnogram findings in children with suspected symptomatic Chiari type I malformation, correlate them with clinical and magnetic resonance imaging data and to determine if this information has value in clinical decision making process. Methods: A retrospective review identified 24 children with type I Chiari malformation, presumed symp-tomatic who had undergone neurological assessment, cranial magnetic resonance imaging and nocturnal polysomnography. Perimedullary subarachnoid space effacement on the magnetic resonance studies and the magnitude of cerebellar tonsillar descent in relation to the McRae line were correlated with frequency of obstructive or central sleep apnea, number of cortical arousals and evidence of impaired vocal mobility on laryngoscopy. The Wilcoxon rank sum test was applied for continuous variables and the Fisher exact test for categorical variables. Results: The median age of the subjects was 6 years. The findings from 16/24 subjects with perimedullary subarachnoid space effacement (effaced group) were compared with those of 8/24 in the non-effaced group. The central apnea index [1.5 (IQR 1–3.5) versus 0.5 (IQR 0–1.5)] and cortical arousal index [12 (IQR 10–19) versus 8 (IQR 6.5–9)] were significantly higher in the effaced group than in the non-effaced group (p = 0.0376 and 0.0036 respectively). Greater descent of tonsils as measured by distance from the McRae line to the tonsil tip was associated with significantly higher central apnea index, total arousal index and respiratory event related arousals. Measurements of clivus-canal angle, Klauss index and pB-C2 line did not correlate with abnormalities on polysomnography. Conclusion: The central apnea and arousal indices derived from the nocturnal polysomnogram correlate well with magnetic resonance imaging findings of subarachnoid space effacement and degree of ton-sillar herniation. In children with Chiari type I malformation, the nocturnal polysomnogram findings provides important information that aids in the decision making process about proceeding with surgical decompression.
    Full-text · Dataset · May 2013 · Clinical neurology and neurosurgery
    • "Down beating nystagmus is considered characteristic of lesions at the level of the foramen magnum. Sleep related disorders associated with CM 1 are well described in literature [4, 5]. Symptoms can vary from simple snoring, bilateral vocal cord palsy to stridor and sleep apnea. "
    [Show abstract] [Hide abstract] ABSTRACT: Chiari malformations type 1 is characterized by herniation of the hindbrain tissue below the foramen magnum. It’s more frequently recognised now due to better imaging modalities. The symptoms are typically subtle and evolve slowly. These could be related to the compression of neural structures at the cranio-cervical junction. We describe 5 children diagnosed with Chiari 1 malformation over last 1 year. Clinical features varied from snoring to spastic quadriplegia. Symptoms improved after surgery in four children. These cases highlight the varied clinical spectrum associated with it.
    Full-text · Article · Jan 2010 · Clinical neurology and neurosurgery
Show more