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Clinical and electrophysiological characterization of myokymia and neuromyotonia in Jack Russell terriers

An Vanhaesebrouck UGent, Iris Van Soens UGent, L Poncelet, Luc Duchateau UGent, Sofie Bhatti UGent, Ingeborgh Polis UGent, Seppe Diels UGent and Luc Van Ham UGent (2010) JOURNAL OF VETERINARY INTERNAL MEDICINE. 24(4). p.882-889
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.
Please use this url to cite or link to this publication:
author
organization
year
type
journalArticle (original)
publication status
published
subject
keyword
EVOKED-POTENTIALS, MOTOR UNIT-ACTIVITY, PERIPHERAL-NERVE HYPEREXCITABILITY, Potassium channels, Hereditary ataxia, MACHADO-JOSEPH-DISEASE, Electrodiagnostics, Dog, SPINAL-CORD, GENERALIZED MYOKYMIA, FRIEDREICHS ATAXIA, NORMAL DOGS, STIMULATION, SCA3
journal title
JOURNAL OF VETERINARY INTERNAL MEDICINE
J. Vet. Intern. Med.
volume
24
issue
4
pages
882 - 889
Web of Science type
Article
Web of Science id
000279449900014
JCR category
VETERINARY SCIENCES
JCR impact factor
2.277 (2010)
JCR rank
11/145 (2010)
JCR quartile
1 (2010)
ISSN
0891-6640
DOI
10.1111/j.1939-1676.2010.0525.x
language
English
UGent publication?
yes
classification
A1
copyright statement
I have transferred the copyright for this publication to the publisher
id
1235162
handle
http://hdl.handle.net/1854/LU-1235162
date created
2011-05-24 21:27:10
date last changed
2011-05-30 14:42:33
@article{1235162,
  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 {\textlangle} .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 {\textlangle} .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.},
  author       = {Vanhaesebrouck, An and Van Soens, Iris and Poncelet, L and Duchateau, Luc and Bhatti, Sofie and Polis, Ingeborgh and Diels, Seppe and Van Ham, Luc},
  issn         = {0891-6640},
  journal      = {JOURNAL OF VETERINARY INTERNAL MEDICINE},
  keyword      = {EVOKED-POTENTIALS,MOTOR UNIT-ACTIVITY,PERIPHERAL-NERVE HYPEREXCITABILITY,Potassium channels,Hereditary ataxia,MACHADO-JOSEPH-DISEASE,Electrodiagnostics,Dog,SPINAL-CORD,GENERALIZED MYOKYMIA,FRIEDREICHS ATAXIA,NORMAL DOGS,STIMULATION,SCA3},
  language     = {eng},
  number       = {4},
  pages        = {882--889},
  title        = {Clinical and electrophysiological characterization of myokymia and neuromyotonia in Jack Russell terriers},
  url          = {http://dx.doi.org/10.1111/j.1939-1676.2010.0525.x},
  volume       = {24},
  year         = {2010},
}

Chicago
Vanhaesebrouck, An, Iris Van Soens, L Poncelet, Luc Duchateau, Sofie Bhatti, Ingeborgh Polis, Seppe Diels, and Luc Van Ham. 2010. “Clinical and Electrophysiological Characterization of Myokymia and Neuromyotonia in Jack Russell Terriers.” Journal of Veterinary Internal Medicine 24 (4): 882–889.
APA
Vanhaesebrouck, A., Van Soens, I., Poncelet, L., Duchateau, L., Bhatti, S., Polis, I., Diels, S., et al. (2010). Clinical and electrophysiological characterization of myokymia and neuromyotonia in Jack Russell terriers. JOURNAL OF VETERINARY INTERNAL MEDICINE, 24(4), 882–889.
Vancouver
1.
Vanhaesebrouck A, Van Soens I, Poncelet L, Duchateau L, Bhatti S, Polis I, et al. Clinical and electrophysiological characterization of myokymia and neuromyotonia in Jack Russell terriers. JOURNAL OF VETERINARY INTERNAL MEDICINE. 2010;24(4):882–9.
MLA
Vanhaesebrouck, An, Iris Van Soens, L Poncelet, et al. “Clinical and Electrophysiological Characterization of Myokymia and Neuromyotonia in Jack Russell Terriers.” JOURNAL OF VETERINARY INTERNAL MEDICINE 24.4 (2010): 882–889. Print.