Equine muscular dystrophy with myotonia

MONTAGNA, P., LIGUORI, R., MONARI, L., STRONG, P. N., RIVA, R., DI STASI, V., GANDINI, G. and CIPONE, M. (2001). Equine muscular dystrophy with myotonia. Clinical neurophysiology, 112 (2), 294-299.

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Link to published version:: 10.1016/S1388-2457(00)00511-3

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.

Item Type: Article
Research Institute, Centre or Group: Biomedical Research Centre
Identification Number: 10.1016/S1388-2457(00)00511-3
Depositing User: Ann Betterton
Date Deposited: 12 Mar 2008
Last Modified: 09 Dec 2009 18:23
URI: http://shura.shu.ac.uk/id/eprint/376

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