Progressive myotonia in foals resembling human dystrophia myotonica

A severe and progressive neuromuscular disorder accompanied by clinical, electrophysiological, and pathological features resembling human dystrophia myotonica was observed in three foals. This disorder was apparent as early as 1 month of age and involved progressive skeletal muscle dysfunction, init...

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Bibliographic Details
Published inMuscle & nerve Vol. 11; no. 4; p. 291
Main Authors Reed, S M, Hegreberg, G A, Bayly, W M, Brown, C M, Paradis, M R, Clemmons, R M
Format Journal Article
LanguageEnglish
Published United States 01.04.1988
Subjects
Animals
Animals, Newborn - anatomy & histology
Animals, Newborn - physiology
Electrophysiology
Female
Horse Diseases - pathology
Horse Diseases - physiopathology
Horses
Humans
Male
Muscles - pathology
Muscles - physiopathology
Myotonia - pathology
Myotonia - physiopathology
Myotonia - veterinary
Myotonic Dystrophy - pathology
Myotonic Dystrophy - physiopathology
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Summary:A severe and progressive neuromuscular disorder accompanied by clinical, electrophysiological, and pathological features resembling human dystrophia myotonica was observed in three foals. This disorder was apparent as early as 1 month of age and involved progressive skeletal muscle dysfunction, initially characterized by proximal muscle hypertrophy and hypertonicity with subsequent muscle stiffness, weakness, and atrophy. Multisystem involvement was manifested in one case by testicular hypoplasia, early cataract formation, and borderline glucose intolerance. Prolonged dimpling of these large rear-limb muscles was elicited by percussion. Myotonic discharges were identified by electromyography. Percussion dimpling and the typical myotonic discharges persisted after neuromuscular blockade. Distinctive histologic muscle changes included ring fibers, sarcoplasmic mass formation, variation in fiber diameter size, and internally positioned nuclei.
ISSN:0148-639X
1097-4598
DOI:10.1002/mus.880110403