title: Myokymia and neuromyotonia in veterinary medicine: a comparison with peripheral nerve hyperexcitability syndrome in humans.
abstract: Involuntary muscle hyperactivity can result from muscle or peripheral nerve hyperexcitability or central nervous system dysfunction. In humans, diseases causing hyperexcitability of peripheral nerves are grouped together under the term 'peripheral nerve hyperexcitability' (PNH). Hyperexcitability of the peripheral motor nerve can result into five different phenotypic main variants, i.e. fasciculations, myokymia, neuromyotonia, cramps and tetany, each with their own clinical and electromyographic characteristics. This review focuses on the most commonly described expressions of PNH in veterinary medicine, i.e. myokymia and neuromyotonia, in particular in young Jack Russell terriers. Data from 58 veterinary cases with generalized myokymia and neuromyotonia were analyzed, including unpublished treatment and follow-up data on eight Jack Russell terriers from a previous study and seven additional Jack Russell terriers. A dysfunction of the potassium channel or its associated proteins has been found in many human syndromes characterized by PNH, in particular in generalized myokymia and neuromyotonia, and is suspected to occur in veterinary medicine. Potential pathomechanisms of potassium channel dysfunction leading to signs of PNH are broad and include genetic mutations, antibody-mediated attack or ion channel maldistribution due to axonal degeneration or demyelination. A more accurate classification of the different PNH syndromes will facilitate a more rapid diagnosis and guide further research into natural occurring PNH in animals.
authors.affiliation: Department of Veterinary Medicine, The Queen's Veterinary School Hospital, University of Cambridge, Cambridge CB3 0ES, UK. av354@cam.ac.uk
authors.forename: An E
authors.initials: AE
authors.lastname: Vanhaesebrouck
authors.forename: Sofie F M
authors.initials: SF
authors.lastname: Bhatti
authors.forename: Robin J M
authors.initials: RJ
authors.lastname: Franklin
authors.forename: Luc
authors.initials: L
authors.lastname: Van Ham
mesh_terms: D000818:Animals; D006801:Humans; D020386:Isaacs Syndrome / Q000662:veterinary*; D009120:Muscle Cramp / Q000473:pathology; D020385:Myokymia / Q000662:veterinary*; D010523:Peripheral Nervous System Diseases / Q000473:pathology*; D013746:Tetany / Q000473:pathology
keywords: Dog; Hereditary ataxia; Jack Russell terrier; Muscle hyperactivity; Potassium channel