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Peer-reviewed veterinary case report

Signs and progression of Lafora disease in Beagles

By Flegel, Thomas et al.·Published in Journal of veterinary internal medicine·2021·Small Animal Department, Germany·View original on PubMed

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Original publication title: A retrospective case series of clinical signs in 28 Beagles with Lafora disease.

Species:
dog

Plain-English summary

A group of 28 Beagles diagnosed with Lafora disease, a genetic condition, showed various symptoms starting around 8 years old. The dogs initially experienced myoclonic episodes (sudden muscle jerks) and some later developed tonic-clonic seizures (convulsions). As the disease progressed, many exhibited coordination issues, vision and hearing problems, and behavioral changes like increased sensitivity to light and noise. Despite these challenges, many dogs lived into their late teens, indicating that while Lafora disease significantly impacts their quality of life, they can still have a relatively normal lifespan.

People also search for: Beagle Lafora disease symptoms · myoclonic seizures in dogs · treatment for Beagle seizures · dog coordination problems · aging Beagle behavior changes

Abstract

BACKGROUND: Clinical signs and their progression in Beagles with Lafora disease are poorly described. OBJECTIVES: To describe clinical signs in Beagles with Lafora disease. ANIMALS: Twenty-eight Beagles with Lafora disease confirmed by genetic testing or histopathology. METHODS: Retrospective multicenter case series. Data regarding signalment, clinical signs, diagnostic tests and treatment were retrieved from hospital data files. A questionnaire was sent to owners asking about neurological deficits, changes in cognitive functions, behavioral changes, response to treatment and survival time. RESULTS: Onset of clinical signs was 8.3 years (mean; range, 6.3-13.3). All dogs had myoclonic episodes as an initial clinical sign with tonic-clonic seizures in n = 11/28 (39%) and n = 12/28 (43%) later developing tonic-clonic seizures. Deficits of coordination (n = 21/25; 84%), impaired vision (n = 15/26; 58%), and impaired hearing (n = 13/26; 50%) developed later. Mental decline was observed as loss of house training (urination; n = 8/25; 32%), difficulties performing learned tasks (n = 9/25; 36%), and difficulties learning new tasks (n = 7/23; 30%). Common behavioral changes were: increased photosensitivity (n = 20/26; 77%), staring into space (n = 16/25; 64%), reduced stress resistance (n = 15/26; 58%), increased noise sensitivity (n = 14/26; 54%), and separation anxiety (n = 11/25; 44%). Twenty-one dogs were alive (median age 11.9 years; range, 9.8-18.6), and 7 dogs were dead (mean age 12.1 years; SD: 1.3; range, 10.5-12.6) at time of writing. CONCLUSIONS AND CLINICAL IMPORTANCE: Lafora disease in Beagles causes significant behavioral changes, and mental decline as well as neurological deficits in addition to myoclonic episodes and generalized tonic-clonic seizures. Nevertheless, a relatively normal life span can be expected.

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Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/34486182/