Elsevier

Pediatric Neurology

Volume 12, Issue 2, February 1995, Pages 149-151
Pediatric Neurology

Case report
Complete heart block in nonfamilial hyperekplexia

https://doi.org/10.1016/0887-8994(94)00111-EGet rights and content

Abstract

An infant with nonfamilial hyperekplexia had multiple episodes of tonic spasms that mimicked tonic seizures. They were accompanied by complete heart block and apnea. These episodes did not correlate with electroencephalographic epileptiform changes and were partially responsive to clonazepam, valproic acid, and cardiac pacemaker. Sudden death in hyperekplexia may be related to complete heart block and apnea during seizurelike episodes.

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Cited by (19)

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    2018, Volpe's Neurology of the Newborn
  • Ectodermal, skeletal, and genitourinary abnormalities with neonatal hyperekplexia

    2011, Pediatric Neurology
    Citation Excerpt :

    A recent study has proposed a new pathogenic mechanism of hyperekplexia involving a missense mutation resulting in spontaneous glycine receptor channel opening in the absence of an agonist [3]. Spontaneous channel activity is a pathogenesis in other disorders, including congenital myopathy and cardiac arrhythmias [3], both which have been associated with hyperekplexia (Table 1) [7,8]. We think that this patient manifested a unique clinical syndrome.

  • The effects of clonazepam and vigabatrin in hyperekplexia

    1997, Journal of the Neurological Sciences
  • Neonatal Seizures

    2017, Volpe's Neurology of the Newborn
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Presented in part at the Child Neurology Society Annual Meeting, Orlando, FL, October, 1993.

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