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Risks and Dangers From Hyperekplexia and Other Startle Disorders

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Movement Disorder Emergencies

Part of the book series: Current Clinical Neurology ((CCNEU))

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Abstract

A neurological consultation is requested in the neonatal intensive care unit for a newborn infant who is noted to be extremely jittery. On examination, the baby is neurologically normal except for exaggerated response to tactile and auditory stimuli, lack of habituation on nose tap, and an exaggerated and sustained Moro response. Resting tone is markedly increased, particularly in axial muscles. On one occasion, a flurry of monitor noises triggers jerks and sustained stiffening that produce a 30-second apneic pause. Based on the examination, hyperekplexia is diagnosed, and clonazepam markedly attenuates the startle and stiffening. Screen of the patient’s family reveals one other affected child, who startles to loud noises.

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Authors and Affiliations

  1. Montreal Neurological Hospital and Institute, Montreal, Quebec, Canada

    Frederick Andermann MD, FRCP(c) & Eva Andermann MD, PHD, FCCMG

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  1. Frederick Andermann MD, FRCP(c)
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  2. Eva Andermann MD, PHD, FCCMG
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© 2005 Humana Press Inc.

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Andermann, F., Andermann, E. (2005). Risks and Dangers From Hyperekplexia and Other Startle Disorders. In: Movement Disorder Emergencies. Current Clinical Neurology. Humana Press. https://doi.org/10.1385/1-59259-902-8:187

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  • DOI: https://doi.org/10.1385/1-59259-902-8:187

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-305-3

  • Online ISBN: 978-1-59259-902-8

  • eBook Packages: MedicineMedicine (R0)

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