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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    ABSTRACT: Introduction Chiari type I malformation (CM-I) is characterised by caudal ectopia of the cerebellar tonsils through the foramen magnum. This is associated with brain stem, high spinal cord, and cranial nerve compression phenomena. The most frequent symptoms are occipital headaches and dizziness. Less well-known symptoms are sleep disorders and nocturnal respiratory abnormalities. Sources MEDLINE and information from patients evaluated at the Neurosurgery and Clinical Neurophysiology Departments at Hospital Universitario Vall d’Hebron. Development Review article based on data obtained from MEDLINE articles since 1966, using combinations of the following keywords: «Chiari malformation» or «Arnold-Chiari malformation» and «sleep apnea» or «sleep disorders». Conclusions CM-I patients show a higher prevalence of sleep disorders than that observed in the general population. Some studies report a 50% prevalence of sleep apnea-hypopnea syndrome (SAHS), probably associated with sudden death in some cases. These results support analysing sleep respiratory parameters in theses patients. Identifying SAHS symptoms may help optimise treatment, thereby improving quality of life and prognosis.
    Neurologia (Barcelona, Spain) 06/2014; 29(5):294–304. DOI:10.1016/j.nrl.2011.01.008 · 1.35 Impact Factor
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    ABSTRACT: Chiari malformation (CM) type-1 frequently causes obstructive or central sleep-disordered breathing (SDB) in both adults and children, although SDB is relatively rare as a presenting manifestation in the absence of other neurological symptoms. The definitive treatment of symptomatic CM is surgical decompression. We report a case that is, to our knowledge, a novel manifestation of central sleep apnea (CSA) due to CM type-1 with severe exacerbation and initial clinical presentation during pregnancy.
    Frontiers in Neurology 10/2014; 5:195. DOI:10.3389/fneur.2014.00195
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    ABSTRACT: The prevalence of obstructive sleep apnea-hypopnea syndrome in the general childhood population is 1-2% and the most common cause is adenotonsillar hypertrophy. However, beyond adenotonsillar hypertrophy, there are other highly prevalent causes of this syndrome in children. The causes are often multifactorial and include muscular hypotonia, dentofacial abnormalities, soft tissue hypertrophy of the airway, and neurological disorders). Collaboration between different specialties involved in the care of these children is essential, given the wide variability of conditions and how frequently different factors are involved in their genesis, as well as the different treatments to be applied. We carried out a wide literature review of other causes of obstructive sleep apnea-hypopnea syndrome in children, beyond adenotonsillar hypertrophy. We organised the prevalence of this syndrome in each pathology and the reasons that cause it, as well as their interactions and management, in a consistent manner.
    Acta Otorrinolaringológica Española 08/2014; 66(2). DOI:10.1016/j.otoeng.2014.05.024