title: Cortical processing of respiratory occlusion stimuli in children with central hypoventilation syndrome.
abstract: BACKGROUND
  The ability of patients with central hypoventilation syndrome (CHS) to produce and process mechanoreceptor signals is unknown.
  
  
  OBJECTIVE
  Children with CHS hypoventilate during sleep, although they generally breathe adequately during wakefulness. Previous studies suggest that they have compromised central integration of afferent stimuli, rather than abnormal sensors or receptors. Cortical integration of afferent mechanical stimuli caused by respiratory loading or upper airway occlusion can be tested by measuring respiratory-related evoked potentials (RREPs). We hypothesized that patients with CHS would have blunted RREP during both wakefulness and sleep.
  
  
  METHODS
  RREPs were produced with multiple upper airway occlusions and were obtained during wakefulness, stage 2, slow-wave, and REM sleep. Ten patients with CHS and 20 control subjects participated in the study, which took place at the Children's Hospital of Philadelphia. Each patient was age- and sex-matched to two control subjects. Wakefulness data were collected from 9 patients and 18 control subjects.
  
  
  RESULTS
  During wakefulness, patients demonstrated reduced Nf and P300 responses compared with control subjects. During non-REM sleep, patients demonstrated a reduced N350 response. In REM sleep, patients had a later P2 response.
  
  
  CONCLUSIONS
  CHS patients are able to produce cortical responses to mechanical load stimulation during both wakefulness and sleep; however, central integration of the afferent signal is disrupted during wakefulness, and responses during non-REM are damped relative to control subjects. The finding of differences between patients and control subjects during REM may be due to increased intrinsic excitatory inputs to the respiratory system in this state.
authors.affiliation: The Sleep Center, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
authors.forename: Jingtao
authors.initials: J
authors.lastname: Huang
authors.forename: Carole L
authors.initials: CL
authors.lastname: Marcus
authors.forename: Preetam
authors.initials: P
authors.lastname: Bandla
authors.forename: Michael S
authors.initials: MS
authors.lastname: Schwartz
authors.forename: Michelle E
authors.initials: ME
authors.lastname: Pepe
authors.forename: John M
authors.initials: JM
authors.lastname: Samuel
authors.forename: Howard B
authors.initials: HB
authors.lastname: Panitch
authors.forename: Ruth M
authors.initials: RM
authors.lastname: Bradford
authors.forename: Yael P
authors.initials: YP
authors.lastname: Mosse
authors.forename: John M
authors.initials: JM
authors.lastname: Maris
authors.forename: Ian M
authors.initials: IM
authors.lastname: Colrain
mesh_terms: D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005073:Evoked Potentials, Somatosensory / Q000502:physiology*; D005260:Female; D006801:Humans; D008297:Male; D017286:Polysomnography; D012129:Respiratory Function Tests; D020182:Sleep Apnea, Central / Q000503:physiopathology*; D012895:Sleep, REM / Q000502:physiology*; D014851:Wakefulness / Q000502:physiology*