A prospective controlled study of sleep respiratory events in patients with craniovertebral junction malformation

Abstract

Object. Craniovertebral junction malformation (CVJM) or Chiari malformation in adults, with or without syringomyelia and basilar invagination, produces neuronal dysfunction of the brainstem, cerebellum, cranial nerves, and upper spinal cord. The respiratory center and some of its afferent and efferent components can be altered in these diseases. The authors studied patients with and without CVJM to determine whether this physical feature contributed to sleep disturbances. Methods. Respiratory manifestations during sleep were studied prospectively, by using whole-night polysomnography, in 32 symptomatic patients (CVJM group) and 16 healthy volunteers (control group). Patients with CVJM presented with more sleep disturbances (reports of snoring and apnea) than those in the control group. The apnea/hypopnea index values were higher in patients with CVJMs than in the control group (13 ± 15 compared with 3 ± 6; p = 0.007) and the rate of central sleep apneas was higher in the CVJM than in the control group (22 ± 30 compared with 4 ± 8%; p = 0.009). The apnea/hypopnea index was highest in the subgroup with basilar invagination than in the other subgroups. The central apneic episodes were more frequent in the patients with basilar invagination (35 ± 40%; p = 0.001) and in those with syringomyelia (17.6 ± 24.6%; p = 0.003) than in the control group (4 ± 8%). Patients with symptomatic CVJM, especially those with basilar invagination, presented with more sleep respiratory compromise than did those in the control group. Conclusions. The incidence of sleep apnea/hypopnea syndrome is significantly higher in patients with CVJM.

Evaluation of operative procedures for symptomatic outcome after decompression surgery for Chiari type I malformation

“…Systematic studies with polysomnography in patients with a Chiari malformation have shown that the rate of sleep-disordered breathing ranges from 59% to 70%. 10,11 Furthermore, recent reports have shown that decompressive surgery decreased sleep-disordered breathing, improving the sleep apnea index in a significant number of patients. 12 The presence of a syrinx usually causes additional deficits such as dissociated sensory loss, segmental weakness, hyperactive reflexes, long tract symptoms, or cranial nerve palsy.…”

Upper airway dimensions in patients with craniocervical junction malformations with and without sleep apnea. A pilot case-control study

“…Cases of spontaneous respiratory collapse in adult and infant Chiari type I patients have been well documented, resulting in some deaths 11 .…”

Chiari malformation and sleep related breathing disorders

“…Accordingly, the association between CM and SAS has been described previously in case report studies6 – 10 ranging from acute respiratory failure to central and obstructive apnoeas and hypopnoeas during sleep. Only one study of 23 CM affected patients included a control group 11. In this latter report, including only adults and CM type I, the presence of SAS (defined as apnoea/hypopnoea index values greater than 5) was observed in 44% and 60% of CMI patients, respectively, with and without syringomyelia, and therefore higher than in the control group with 12% of patients affected 11.…”

“…Only one study of 23 CM affected patients included a control group 11. In this latter report, including only adults and CM type I, the presence of SAS (defined as apnoea/hypopnoea index values greater than 5) was observed in 44% and 60% of CMI patients, respectively, with and without syringomyelia, and therefore higher than in the control group with 12% of patients affected 11. Another recent study on 16 adults affected with CM type I reported the presence of SAS (defined as apnoea/hypopnoea index values greater than 10) in 75% of cases 12.…”