Congenital myasthenia gravis in 13 smooth fox terriers.

L. M. Miller, Vanda A Lennon, E. H. Lambert, S. M. Reed, G. A. Hegreberg, J. B. Miller, R. L. Ott

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

In 13 Smooth Fox Terriers with a congenital form of myasthenia gravis, clinical signs included intermittent, progressive muscle weakness that became more pronounced with exercise; muscle wasting; megaesophagus; and aspiration pneumonia. Neurologic abnormalities were apparent only during periods of weakness and included inability to retract the fore- and hindlimbs from painful stimuli. A decrement of the compound muscle action potential was evident during repetitive supramaximal nerve stimulation. Intravenous injection of a short-acting cholinesterase inhibitor evoked immediate improvement of clinical and electromyographic signs. Intracellular microelectrode studies of a biopsied external intercostal muscle revealed reduced amplitude of miniature end-plate potentials, as occurs in acquired myasthenia gravis. However, in contrast to acquired myasthenia gravis, antibodies directed against acetylcholine receptors were not demonstrable in serum and were not bound to acetylcholine receptors in muscle. Despite lack of complexing with immunoglobulin, the amount of acetylcholine receptor protein in biopsied external intercostal muscles from 9 affected pups was less than 25% of the amount in 5 unaffected littermates. The latter finding accounted for the reduction in amplitude of miniature end-plate potential and the failure of neuromuscular transmission. Treatment with a long-acting cholinesterase inhibitor in 6 cases resulted in temporary improvement in muscle strength.

Original languageEnglish (US)
Pages (from-to)694-697
Number of pages4
JournalJournal of the American Veterinary Medical Association
Volume182
Issue number7
StatePublished - Apr 1 1983

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Congenital Myasthenic Syndromes
terriers
Cholinergic Receptors
foxes
Miniature Postsynaptic Potentials
Intercostal Muscles
Myasthenia Gravis
Cholinesterase Inhibitors
Muscles
muscles
cholinergic receptors
cholinesterase inhibitors
Nervous System Malformations
Aspiration Pneumonia
Esophageal Achalasia
Forelimb
Muscle Weakness
Muscle Strength
Microelectrodes
Hindlimb

ASJC Scopus subject areas

  • veterinary(all)

Cite this

Miller, L. M., Lennon, V. A., Lambert, E. H., Reed, S. M., Hegreberg, G. A., Miller, J. B., & Ott, R. L. (1983). Congenital myasthenia gravis in 13 smooth fox terriers. Journal of the American Veterinary Medical Association, 182(7), 694-697.

Congenital myasthenia gravis in 13 smooth fox terriers. / Miller, L. M.; Lennon, Vanda A; Lambert, E. H.; Reed, S. M.; Hegreberg, G. A.; Miller, J. B.; Ott, R. L.

In: Journal of the American Veterinary Medical Association, Vol. 182, No. 7, 01.04.1983, p. 694-697.

Research output: Contribution to journalArticle

Miller, LM, Lennon, VA, Lambert, EH, Reed, SM, Hegreberg, GA, Miller, JB & Ott, RL 1983, 'Congenital myasthenia gravis in 13 smooth fox terriers.', Journal of the American Veterinary Medical Association, vol. 182, no. 7, pp. 694-697.
Miller LM, Lennon VA, Lambert EH, Reed SM, Hegreberg GA, Miller JB et al. Congenital myasthenia gravis in 13 smooth fox terriers. Journal of the American Veterinary Medical Association. 1983 Apr 1;182(7):694-697.
Miller, L. M. ; Lennon, Vanda A ; Lambert, E. H. ; Reed, S. M. ; Hegreberg, G. A. ; Miller, J. B. ; Ott, R. L. / Congenital myasthenia gravis in 13 smooth fox terriers. In: Journal of the American Veterinary Medical Association. 1983 ; Vol. 182, No. 7. pp. 694-697.
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