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

ABSTRACT: 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.

SUBMITTER: Demeny H

PROVIDER: S-EPMC7674959 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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

Demeny Helga H Florea Bogdan B Tabaran Flaviu F Danciu Cecilia Gabriella CG Ognean Laurent L

Frontiers in veterinary science 20201105

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 hyp ...[more]

PMID: 33251270

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