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Related Experiment Video

Updated: Dec 13, 2025

Habituation and Prepulse Inhibition of Acoustic Startle in Rodents
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Hyperekplexia and other startle syndromes.

Arushi Gahlot Saini1, Sanjay Pandey2

  • 1Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.

Journal of the Neurological Sciences
|July 30, 2020
PubMed
Summary

Abnormal startle syndromes, including hereditary hyperekplexia, are treatable neurogenetic disorders. Early diagnosis and interventions like the Vigevano maneuver improve outcomes for affected newborns and adults.

Keywords:
ClonazepamExaggerated startleGlycineHyperekplexiaStartle

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Area of Science:

  • Neurology
  • Neurogenetics

Background:

  • Abnormal startle syndromes encompass hyperekplexia, stimulus-induced, and neuropsychiatric types.
  • Hyperekplexia can be genetic, idiopathic, or symptomatic, with hereditary forms being treatable neurogenetic disorders.

Purpose of the Study:

  • To outline the classification and diagnostic approach to abnormal startle syndromes.
  • To emphasize the importance of identifying secondary causes in infants and acquired causes in older individuals.
  • To highlight effective management strategies and prognosis for hereditary hyperekplexia.

Main Methods:

  • Clinical characterization of hyperactive startle responses.
  • Evaluation of neonates and infants for specific symptoms like stiffness, seizures, apnea, and stimulus sensitivity.
  • Routine examination including the head-retraction reflex to nose tapping and teaching the Vigevano maneuver.
  • Investigation of acquired causes (e.g., brainstem injury, antibodies) for later-onset startle syndromes.
  • Assessment of gait and falls in adults for undiagnosed startle disorders.

Main Results:

  • Secondary causes of startle syndromes in children offer clues to neurodevelopmental or neurodegenerative disorders.
  • The head-retraction reflex to nose tapping and the Vigevano maneuver are key diagnostic and emergency tools.
  • Late-onset startle syndromes warrant investigation for acquired causes like brainstem injury or autoimmune conditions.
  • Undiagnosed startle syndromes should be considered in adults with unexplained gait issues or falls.

Conclusions:

  • Early recognition and characterization of abnormal startle responses are crucial for timely diagnosis and management.
  • Hereditary hyperekplexia has a good prognosis with interventions like oral clonazepam and behavioral support.
  • Comprehensive evaluation, considering both genetic and acquired etiologies, is essential across all age groups.