Risks and Dangers From Hyperekplexia and Other Startle Disorders

  • Frederick Andermann
  • Eva Andermann
Part of the Current Clinical Neurology book series (CCNEU)


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.


Down Syndrome Inhibitory Glycine Receptor Startle Disease Movement Disorder Emergency Monitor Noise 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Humana Press Inc. 2005

Authors and Affiliations

  • Frederick Andermann
    • 1
  • Eva Andermann
    • 1
  1. 1.Montreal Neurological Hospital and InstituteMontrealCanada

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