NEUROMUSCULAR CASE OF THE MONTH - JUNE 2003

Rippling muscles in a 2 year old FS Jack Russell Terrier
Contributed by Dr. William Thomas
University of  Tennessee
Knoxville, TN


Clinical History
A 2-year-old spayed female Jack Russell terrier was presented for evaluation of episodes of muscle twitching and rigidity starting at one year of age. The dog had suffered two severe episodes lasting about one hour and several milder episodes. The attacks appeared to be triggered by excitement and were characterized by visible rippling movements (myokymia) Go To Video Clip in all skeletal muscle groups that progressed to generalized muscle rigidity. She remained alert and did not vocalize or seem painful during these attacks. Rectal temperature was usually elevated (as high as 107° F) during the episodes. A detailed family history was unavailable.

Physical and Neurological Examination
There was rippling in the gluteal muscles and mild generalized tremor.  The dog was alert with appropriate mentation. With the exception of the muscle contractions and tremor, her gait was normal with no evidence of ataxia or weakness. Postural reactions, spinal reflexes and cranial nerves were normal. There was no pain, atrophy, or hypertrophy of the muscles. The clinical signs resolved with diazepam (2.5 mg intravenously) and a calm environment.

Diagnostic Testing
CBC, serum chemistry profile,  electrolytes, and serum creatine kinase: No significant abnormalities

Electromyography and motor nerve conduction velocity: Normal with the exception of occasional positive sharp waves in the biceps femoris muscles and occasional fibrillation potentials in the middle gluteal muscles. The motor nerve conduction velocity of the sciatic-tibial nerve was normal (83 m/s) with a normal M-wave.

Muscle biopsy: No abnormalities identified within either the biceps femoris or gastrocnemius muscles

Clinical Course
Diazepam (5 mg PO every 6-8 hours as needed) was dispensed in an attempt to help control the episodes. Despite therapy, the dog suffered several more attacks. Mexiletine (6 mg/kg PO every 8 hours) was started. The severity of the episodes decreased although the dog still had mild episodes with excitement.

Comments
Several Jack Russell terriers with similar clinical signs have been evaluated at the University of Tennessee. Dr. Luc Van Ham has reported 12 Jack Russell terriers with episodes of "myokymia" progressing to muscle stiffness, hyperthermia and collapse. In this case the term rippling muscle is preferred over myokymia since the muscles were in general electrically silent, unlike that of myokymia. Episodes may be preceded by intense facial rubbing. Membrane stabilizing agents (procainamide, mexiletine, phenytoin) may be helpful. A similar syndrome has been reported by Reading et al in a single Yorkshire terrier. The pathophysiology of this syndrome is not fully understood.

References
Reading MJ, McKerrell RE. Suspected myokymia in a Yorkshire Terrier. Vet Rec 1993;132:587-588

Van Ham L. Suspected neuromyotonia in 12 Jack Russell terriers. Proceedings European Society of Veterinary Neurology 1998, pgs 26-27.



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