[Lafora disease in a Beagle - diagnosis and therapy]. / Lafora-Erkrankung bei einem Beagle Diagnose und Therapie.
Tierarztl Prax Ausg K Kleintiere Heimtiere
; 48(5): 361-364, 2020 Oct.
Article
em De
| MEDLINE
| ID: mdl-33086413
ABSTRACT
Lafora disease is an autosomal recessive lysosomal storage disorder leading to an accumulation of toxic glycogen bodies into the cells of the central nervous system and other tissues. In the progressive form of myoclonic epilepsy, clinical signs typically start around 7â
years of age. Causal therapy is impossible, however, in the early stages the symptoms may at least be alleviated by modern antiepileptic drugs. In the case reported here, an approximately 7-year-old Beagle presented with daytime-dependent fasciculations, focal and generalized myoclonus ranging up to a brief tonic-clonic seizure. The signs could be triggered and augmented by stress, sounds and light. Histologic examination was performed on biopsy samples of skin, liver, muscle and nervous tissue to test for the clinical diagnosis of Lafora disease. Sarcoplasmic PAS-positive pla®ue deposits typical of Lafora bodies were detected in the muscle biopsies but not in any of the other specimens. Initial treatment with phenobarbital and imepitoin was unsuccessful. However, treatment with levetiracetam significantly alleviated the clinical signs. At time of writing this publication, 2â
years following the diagnosis, the now 9-year-old dog shows occasional, stress-related increase in fokal myoclonic seizures. Episodes of collapse or tonic-clonic seizures did not occur to any further extent.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doença de Lafora
/
Doenças do Cão
/
Anticonvulsivantes
Tipo de estudo:
Diagnostic_studies
Limite:
Animals
Idioma:
De
Revista:
Tierarztl Prax Ausg K Kleintiere Heimtiere
Assunto da revista:
MEDICINA VETERINARIA
Ano de publicação:
2020
Tipo de documento:
Article