Abstract

Sixteen patients, including 1 neonate, in a large 5 generation family with hyperekplexia (startle disease or congenital stiff-man syndrome) were treated with clonazepam at the University of Texas Health Science Center, San Antonio, TX.

Highlights

  • HYPEREKPLEXIA; RESPONSE TO CLONAZEPAM Sixteen patients, including 1 neonate, in a large 5 generation family with hyperekplexia (startle disease or congenital stiff-man syndrome) were treated with clonazepam at the University of Texas Health Science Center, San Antonio, TX

  • Clonazepam was introduced at a dose of 0.5 mg (0.125 - 0.25 mg for children at bedtime)

  • The total daily dose in children did not exceed 1.5 mg and increases of .125 to .25 mg were made every third day until the symptoms improved. (Ryan SG et al Startle disease, or hyperekplexia: response to clonazepam and assignment of the gene (STHE) to chromosome 5q by linkage analysis

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Summary

Introduction

HYPEREKPLEXIA; RESPONSE TO CLONAZEPAM Sixteen patients, including 1 neonate, in a large 5 generation family with hyperekplexia (startle disease or congenital stiff-man syndrome) were treated with clonazepam at the University of Texas Health Science Center, San Antonio, TX. The total daily dose in children did not exceed 1.5 mg and increases of .125 to .25 mg were made every third day until the symptoms improved. (Ryan SG et al Startle disease, or hyperekplexia: response to clonazepam and assignment of the gene (STHE) to chromosome 5q by linkage analysis.

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