Document Detail

Startle syndromes.
MedLine Citation:
PMID:  21496599     Owner:  NLM     Status:  MEDLINE    
Startle refers to a sudden involuntary movement of the body in response to a surprising and unexpected stimulus. It is a fast twitch of facial and body muscles evoked by a sudden and intense tactile, visual, or acoustic stimulus. While startle can be considered to be a protective function against injury, startle syndromes are abnormal responses to startling events, consisting of three heterogeneous groups of disorders. The first is hyperekplexia, characterized by brisk and generalized startle in response to trivial stimulation. The major form of hereditary hyperekplexia has a genetic basis, frequently due to mutations in the α1 subunit of the glycine receptor (GLRA1) on chromosome 5q. In the second group, normal startle induces complex but stereotyped motor and/or behavioral abnormalities lasting several seconds, termed as startle epilepsy. It usually occurs in the setting of severe brain damage, particularly perinatal hypoxia. The third group is characterized by nonhabituating hyperstartling, provoked by loud noises, sudden commands, or gestures. The intensity of startle response tends to increase with frequency of stimulation, which often leads to injury. Interestingly, its occurrence is restricted to certain social or ethnic groups in different parts of the world, such as jumping Frenchmen of Maine among Franco-Canadian lumberjack communities, and Latah in Southeast Asia. So far, no neurological abnormalities have been reported in association with these neuropsychiatric startle syndromes. In this chapter, the authors discuss the clinical presentation, physiology, and the neuronal basis of the normal human startle as well as different groups of abnormal startle syndromes. The aim is to provide an overview of hyperstartling with some diagnostic hints and the distinguishing features among these syndromes.
Roongroj Bhidayasiri; Daniel D Truong
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Handbook of clinical neurology     Volume:  100     ISSN:  0072-9752     ISO Abbreviation:  Handb Clin Neurol     Publication Date:  2011  
Date Detail:
Created Date:  2011-04-18     Completed Date:  2011-07-12     Revised Date:  2013-03-18    
Medline Journal Info:
Nlm Unique ID:  0166161     Medline TA:  Handb Clin Neurol     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  421-30     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 Elsevier B.V. All rights reserved.
Chulalongkorn Comprehensive Movement Disorders Center, Chulalongkorn University Hospital, Bangkok, Thailand.
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MeSH Terms
Brain Diseases / physiopathology*
Movement Disorders / physiopathology*
Reflex, Abnormal*
Startle Reaction / physiology*

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