Congenital Myasthenic Syndrome in a Mixed Breed Dog

Frontiers in Veterinary Science
Theresa J BlakeyG D Shelton

Abstract

A 6-month-old, male, intact mixed breed dog was presented for a 3-month history of progressive generalized weakness. Neurologic examination revealed non-ambulatory tetraparesis, weakness of the head and neck, and decreased withdrawal reflexes in all limbs consistent with a generalized neuromuscular disorder. Electromyography and motor nerve conduction velocity were normal. Repetitive nerve stimulation showed a decremental response of the compound muscle action potential with improvement upon intravenous administration of edrophonium chloride. The serum acetylcholine receptor (AChR) antibody titer was within reference range. Cerebrospinal fluid analysis was unremarkable. A presumptive diagnosis of post-synaptic congenital myasthenic syndrome (CMS) was made. Treatment with pyridostigmine bromide was initiated with titrated increases in dosage resulting in an incomplete improvement in clinical signs. The dog was euthanized 2 months after initiation of treatment due to poor quality of life. Immunostaining for localization of antibodies against end-plate proteins in muscle biopsies was negative. Immunofluorescence staining for AChRs in external intercostal muscle biopsies showed absence of AChRs and biochemical quantitation showed a...Continue Reading

References

Mar 1, 1997·Journal of Veterinary Internal Medicine·C W DeweyG H Cardinet
Jan 12, 2002·The Veterinary Clinics of North America. Small Animal Practice·G Diane Shelton
Mar 21, 2015·Lancet Neurology·Andrew G EngelSteven M Sine
Sep 17, 2015·Animal Genetics·Barbara GandolfiLeslie A Lyons
Oct 3, 2015·Neuromuscular Disorders : NMD·Caitlin J RinzG Diane Shelton

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Citations

Jul 16, 2020·Journal of Veterinary Internal Medicine·Thomas MignanMark Lowrie

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Methods Mentioned

BETA
biopsies
biopsy

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