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J Vet Intern Med. 2010 Jul-Aug;24(4):882-9. doi: 10.1111/j.1939-1676.2010.0525.x. Epub 2010 May 11.

Clinical and electrophysiological characterization of myokymia and neuromyotonia in Jack Russell Terriers.

Author information

1
Department of Small Animal Medicine and Clinical Biology, Faculty of Veterinary Medicine, Ghent University, Ghent, Belgium. av354@cam.ac.uk

Abstract

BACKGROUND:

Generalized myokymia and neuromyotonia (M/NM) in Jack Russell Terriers (JRTs) is related to peripheral nerve hyperexcitability syndrome in humans, a symptom complex resulting from diverse etiologies.

OBJECTIVE:

Clinical and electrodiagnostic evaluation is used to narrow the list of possible etiological diagnoses in JRTs with M/NM.

ANIMALS:

Nine healthy JRTs and 8 affected JRTs.

METHODS:

A prospective study was conducted comparing clinical and electrophysiological characteristics in 8 JRTs affected by M/NM with 9 healthy JRT controls.

RESULTS:

All affected dogs except 1 had clinical signs typical of hereditary ataxia (HA). In 6 dogs, neuromyotonic discharges were recorded during electromyogram. Motor nerve conduction studies showed an axonal neuropathy in only 1 affected dog. Compared with controls, brainstem auditory-evoked potentials (BAEP) showed prolonged latencies (P<.05) accompanied by the disappearance of wave components in 3 dogs. Onset latencies of tibial sensory-evoked potentials (SEP) recorded at the lumbar intervertebral level were delayed in the affected group (P<.001). The BAEP and SEP results of the only neuromyotonic dog without ataxia were normal.

CONCLUSIONS AND CLINICAL IMPORTANCE:

The BAEP and spinal SEP abnormalities observed in JRTs with M/NM were associated with the presence of HA. Therefore, these electrophysiological findings presumably arise from the neurodegenerative changes characterizing HA and do not directly elucidate the pathogenesis of M/NM. An underlying neuronal ion channel dysfunction is thought to be the cause of M/NM in JRTs.

PMID:
20492485
DOI:
10.1111/j.1939-1676.2010.0525.x
[Indexed for MEDLINE]
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