Document Detail


Effects of sedation on auditory brainstem response in Rett syndrome.
MedLine Citation:
PMID:  20399386     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Prolongation of the I-V interpeak latency intervals have been reported in Rett syndrome and other neurodevelopmental disorders. It has been suggested that the use of sedation may account for differences in the interpeak latency intervals when comparisons are made across diagnostic groups if sedated control groups are not used for the basis of comparison. This study examined the effects of sedation on auditory brainstem response interpeak latency intervals (i.e., I-III, III-V, and I-V) in two groups: (1) a group with Rett syndrome who were positive for mutations in the MECP2 gene and (2) a group negative for mutations in the MECP2 gene but who were severely to profoundly delayed with other causes of mental retardation. To further assess the effects of sedation, a third group of sedated and nonsedated female participants, taken from an in-house normative auditory brainstem response database was also included. An analysis of variance indicated (1) longer I-V interpeak latency intervals in the sedated participants with Rett syndrome; (2) longer III-V interpeak latency intervals in the mutation-positive participants as compared to non-Rett syndrome, mutation-negative participants; and (3) no significant effects of sedation on the I-III, III-V, or I-V interpeak latency intervals among the normative group participants, according to t tests. The findings suggest a possible biological basis for the discrepancy in the literature on auditory brain stem responses in Rett syndrome, and warrant cautious interpretation of auditory brainstem responses findings in sedated subjects with Rett syndrome, as well as in those with mental retardation and seizures.
Authors:
Joseph P Pillion; Genila Bibat; Sakkubai Naidu
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Pediatric neurology     Volume:  42     ISSN:  1873-5150     ISO Abbreviation:  Pediatr. Neurol.     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-04-19     Completed Date:  2010-10-25     Revised Date:  2011-07-28    
Medline Journal Info:
Nlm Unique ID:  8508183     Medline TA:  Pediatr Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  331-4     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Elsevier Inc. All rights reserved.
Affiliation:
Department of Audiology, Kennedy Krieger Institute, Baltimore, Maryland 21205, USA. pillion@kennedykrieger.org
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Cell Line
Child
Child, Preschool
Conscious Sedation* / methods
DNA Mutational Analysis
Evoked Potentials, Auditory, Brain Stem / physiology*
Female
Humans
Methyl-CpG-Binding Protein 2 / genetics
Rett Syndrome / genetics,  physiopathology*
Young Adult
Grant Support
ID/Acronym/Agency:
HD24448/HD/NICHD NIH HHS; P01 HD024448-20/HD/NICHD NIH HHS; RR-00052/RR/NCRR NIH HHS
Chemical
Reg. No./Substance:
0/MECP2 protein, human; 0/Methyl-CpG-Binding Protein 2
Comments/Corrections

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