Article

Sleep-Disordered Breathing in Chiari Malformation Type 1

Child Development Center, Department of Paediatric Neurology, Leicester Royal Infirmary, Leicester, United Kingdom.
Pediatric Neurology (Impact Factor: 1.5). 10/2008; 39(3):207-8. 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.

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    • "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 hypoventilation [9] [10] [11] [12] [13]. Sudden unexplained death has also been reported [14]. "
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    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.
    Clinical neurology and neurosurgery 06/2013; 115(9). DOI:10.1016/j.clineuro.2013.05.025 · 1.25 Impact Factor
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    • "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 hypoventilation [9] [10] [11] [12] [13] "
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    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.
  • Advances in Inorganic Chemistry 01/2005; 57:41-104. DOI:10.1016/S0898-8838(05)57002-8 · 2.56 Impact Factor
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