Equine muscular dystrophy with myotonia

P. Montagna, R. Liguori, L. Monari, P. N. Strong, R. Riva, V. Di Stasi, G. Gandini, M. Cipone

Research output: Contribution to journalArticlepeer-review

Abstract

Objectives: To describe a case of equine muscular dystrophy with myotonia. Methods: A 5-year-old horse presented with hypertrophy and delayed relaxation of the muscles of the hindlimbs from age 2 months. Testicular atrophy developed from 2 years of age. Action and percussion myotonia was associated with weakness in these muscles, and EMG showed diffuse myotonic discharges and myopathic features. Biopsy of the gluteal muscle showed adipose and connective tissue infiltration, marked variation in muscle fibre size, and moth-eaten, ring and whorled fibres. Results: Injection of apamin, a peptide blocker of calcium-activated potassium channels, which inhibits myotonia in human myotonic dystrophy, was ineffective in blocking myotonic discharges. Discharges promptly abated with 2% lidocaine injection. Conclusions: Myotonia in this horse is associated with dystrophic changes similar to human myotonic dystrophy, though there are some pharmacological differences.

Original languageEnglish
Pages (from-to)294-299
Number of pages6
JournalClinical Neurophysiology
Volume112
Issue number2
DOIs
Publication statusPublished - 2001

Keywords

  • Apamin
  • EMG in myotonia
  • Equine
  • Muscular dystrophy
  • Myotonia
  • Myotonic dystrophy

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Neurology
  • Sensory Systems
  • Physiology (medical)

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