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EEG Patterns Orienting to Lafora Disease Diagnosis—A Case Report in Two Beagles

Lafora Disease (LD) is a rare, fatal, late-onset, progressive form of myoclonic epilepsy, occurring in humans and dogs. Clinical manifestations of LD usually include seizures, spontaneous and reflex myoclonus with contractions of the neck and limb muscles. We studied the electroencephalogram (EEG) p...

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Autores principales: Demeny, Helga, Florea, Bogdan, Tabaran, Flaviu, Danciu, Cecilia Gabriella, Ognean, Laurent
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7674959/
https://www.ncbi.nlm.nih.gov/pubmed/33251270
http://dx.doi.org/10.3389/fvets.2020.589430
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author Demeny, Helga
Florea, Bogdan
Tabaran, Flaviu
Danciu, Cecilia Gabriella
Ognean, Laurent
author_facet Demeny, Helga
Florea, Bogdan
Tabaran, Flaviu
Danciu, Cecilia Gabriella
Ognean, Laurent
author_sort Demeny, Helga
collection PubMed
description Lafora Disease (LD) is a rare, fatal, late-onset, progressive form of myoclonic epilepsy, occurring in humans and dogs. Clinical manifestations of LD usually include seizures, spontaneous and reflex myoclonus with contractions of the neck and limb muscles. We studied the electroencephalogram (EEG) patterns of two beagles in whom LD was subsequently confirmed by genetic testing. In both cases, the EEG recordings, accompanied by electromyography (EMG), have shown similar uncommon patterns. The hypovoltaged background rhythm was interrupted by waxing “crescendo” polyspikes-slow wave complexes appearing 80–250 ms after the start of intermittent photic stimulation, followed by myoclonic jerks after 80–150 ms. This study highlights the value of EEG in establishing a presumptive diagnosis of LD in dogs.
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spelling pubmed-76749592020-11-27 EEG Patterns Orienting to Lafora Disease Diagnosis—A Case Report in Two Beagles Demeny, Helga Florea, Bogdan Tabaran, Flaviu Danciu, Cecilia Gabriella Ognean, Laurent Front Vet Sci Veterinary Science Lafora Disease (LD) is a rare, fatal, late-onset, progressive form of myoclonic epilepsy, occurring in humans and dogs. Clinical manifestations of LD usually include seizures, spontaneous and reflex myoclonus with contractions of the neck and limb muscles. We studied the electroencephalogram (EEG) patterns of two beagles in whom LD was subsequently confirmed by genetic testing. In both cases, the EEG recordings, accompanied by electromyography (EMG), have shown similar uncommon patterns. The hypovoltaged background rhythm was interrupted by waxing “crescendo” polyspikes-slow wave complexes appearing 80–250 ms after the start of intermittent photic stimulation, followed by myoclonic jerks after 80–150 ms. This study highlights the value of EEG in establishing a presumptive diagnosis of LD in dogs. Frontiers Media S.A. 2020-11-05 /pmc/articles/PMC7674959/ /pubmed/33251270 http://dx.doi.org/10.3389/fvets.2020.589430 Text en Copyright © 2020 Demeny, Florea, Tabaran, Danciu and Ognean. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Veterinary Science
Demeny, Helga
Florea, Bogdan
Tabaran, Flaviu
Danciu, Cecilia Gabriella
Ognean, Laurent
EEG Patterns Orienting to Lafora Disease Diagnosis—A Case Report in Two Beagles
title EEG Patterns Orienting to Lafora Disease Diagnosis—A Case Report in Two Beagles
title_full EEG Patterns Orienting to Lafora Disease Diagnosis—A Case Report in Two Beagles
title_fullStr EEG Patterns Orienting to Lafora Disease Diagnosis—A Case Report in Two Beagles
title_full_unstemmed EEG Patterns Orienting to Lafora Disease Diagnosis—A Case Report in Two Beagles
title_short EEG Patterns Orienting to Lafora Disease Diagnosis—A Case Report in Two Beagles
title_sort eeg patterns orienting to lafora disease diagnosis—a case report in two beagles
topic Veterinary Science
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7674959/
https://www.ncbi.nlm.nih.gov/pubmed/33251270
http://dx.doi.org/10.3389/fvets.2020.589430
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