Neurotrophic factor expression in three infants with Ondine's curse.

Abstract:

:This study investigates the expression of some neurotrophic factors (brain-derived neurotrophic factor, glial-derived neurotrophic factor, and nerve growth factor) in the cerebrospinal fluid of infants suffering from idiopathic congenital central hypoventilation syndrome and determines their correlations with this syndrome. Cerebrospinal fluid samples were collected from three infants suffering from idiopathic congenital central hypoventilation syndrome and 15 control subjects with obstructive hydrocephalus to measure the expression of brain-derived neurotrophic factor, glial-derived neurotrophic factor, and nerve growth factor using an immunoenzymatic assay. In the cerebrospinal fluid of patients, analysis of neurotrophic factors expression indicated a reduction, not statistically significant, of brain-derived neurotrophic factor compared with the mean level of the control group (1554 pg/mL, 1509 pg/mL, and 1582 pg/mL respectively, in comparison to 1954 +/- 103 pg/mL), whereas nerve growth factor and glial-derived neurotrophic factor did not undergo significant variations in either group. Neurotrophic factors, namely brain-derived neurotrophic factor, regulate the maturation and differentiation of respiratory neurons. The reduced expression of brain-derived neurotrophic factor in the cerebrospinal fluid samples of infants with Ondine's curse, although not statistically significant, is suggestive of a dysregulation in the brain-derived neurotrophic factor synthesis that could play an important role in the breathing disorders observed in patients with idiopathic congenital central hypoventilation syndrome.

journal_name

Pediatr Neurol

journal_title

Pediatric neurology

authors

Chiaretti A,Zorzi G,Di Rocco C,Genovese O,Antonelli A,Piastra M,Polidori G,Aloe L

doi

10.1016/j.pediatrneurol.2005.05.014

subject

Has Abstract

pub_date

2005-11-01 00:00:00

pages

331-6

issue

5

eissn

0887-8994

issn

1873-5150

pii

S0887-8994(05)00273-0

journal_volume

33

pub_type

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