Document Detail


Congenital myasthenic syndrome with sleep hypoventilation.
MedLine Citation:
PMID:  10883011     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report the case of a 13-year-old boy with acetylcholinesterase deficiency, a congenital myasthenic syndrome, who developed sleep hypoventilation syndrome during a period of rapid growth. His symptoms were insidious and life-threatening despite changes in strength or lung volume measurements that were not marked. He responded well to noninvasive nocturnal ventilation, with reversal of symptoms and normalization of blood gases. His lung volumes, but not motor function, improved after treatment.
Authors:
S T Iannaccone; J K Mills; K M Harris; J H Herman; P Schochet; P Luckett
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Muscle & nerve     Volume:  23     ISSN:  0148-639X     ISO Abbreviation:  Muscle Nerve     Publication Date:  2000 Jul 
Date Detail:
Created Date:  2000-08-07     Completed Date:  2000-08-07     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1129-32     Citation Subset:  IM    
Copyright Information:
Copyright 2000 John Wiley & Sons, Inc.
Affiliation:
Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
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MeSH Terms
Descriptor/Qualifier:
Acetylcholinesterase / deficiency*
Humans
Hypoventilation / congenital*,  etiology*,  therapy
Infant
Lung Volume Measurements
Male
Neuromuscular Diseases / complications*,  congenital*,  therapy
Polysomnography
Respiration, Artificial
Syndrome
Chemical
Reg. No./Substance:
EC 3.1.1.7/Acetylcholinesterase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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