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1 Department of Neurobiology, University Californai Los Angeles, Los Angeles, CA, USA
2 School of Nursing, University Californai Los Angeles, Los Angeles, CA, USA
3 Department of Radiology, University Californai Los Angeles, Los Angeles, CA, USA
4 Childrens Hospital, Los Angeles, CA, USA
* To whom correspondence should be addressed. E-mail: rharper{at}ucla.edu.
Congenital Central Hypoventilation Syndrome (CCHS) patients show impaired ventilatory responses to CO2 and hypoxia, and reduced drive to breathe during sleep, but retain appropriate breathing patterns to volition or increased exercise. Breath-by-breath influences on heart rate are also deficient. We examined, using functional magnetic resonance imaging techniques, responses over the brain to voluntary forced expiratory loading, a task which CCHS cases can perform, but which results in impaired rapid heart rate variation patterns normally associated with the challenge. Increased signals emerged in control (N = 14) over CCHS (N = 13; ventilator dependent during sleep but not waking) subjects in the cingulate and right parietal cortex, cerebellar cortex and fastigial nucleus, and basal ganglia, while anterior cerebellar cortical sites and deep nuclei, dorsal midbrain and dorsal pons showed increased signals in the patient group. The dorsal and ventral medulla showed delayed responses in CCHS cases. Primary motor and sensory areas bordering the central sulcus showed comparable responses in both groups. The delayed responses in medullary sensory and output regions, and the aberrant reactions in cerebellar and pontine sensorimotor coordination areas suggest that rapid cardiorespiratory integration deficits in CCHS may stem from defects in these sites. Additional autonomic and perceptual motor deficits may derive from cingulate and parietal cortex aberrations.
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