Cortical processing of respiratory occlusion stimuli in children with central hypoventilation syndrome.
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.
Huang
Jingtao
J
The Sleep Center, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Marcus
Carole L
CL
Bandla
Preetam
P
Schwartz
Michael S
MS
Pepe
Michelle E
ME
Samuel
John M
JM
Panitch
Howard B
HB
Bradford
Ruth M
RM
Mosse
Yael P
YP
Maris
John M
JM
Colrain
Ian M
IM
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*