Peer-reviewed veterinary case report
Neurological signs and progression of L-2-HGA in Staffordshire bull
By Shea, A et al.·Published in The Veterinary record·2016·Neurology/Neurosurgery Service, United Kingdom·View original on PubMed →
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Original publication title: Clinical features and disease progression of L-2-hydroxyglutaric aciduria in 27 Staffordshire bull terriers.
- Species:
- dog
Plain-English summary
A group of 27 Staffordshire bull terriers showed signs of a rare neurological condition called L-2-hydroxyglutaric aciduria (L-2-HGA), which typically began around 12 months of age. Owners reported symptoms like stiffness in all four legs, gait problems, body tremors, and behavioral changes such as staring into space. While the disease is progressive, many dogs can still live a normal lifespan with proper management. About 70% of the dogs were alive several years after showing symptoms, although some did require euthanasia due to the severity of their condition.
People also search for: Staffordshire bull terrier neurological problems · L-2-hydroxyglutaric aciduria symptoms · dog seizures treatment
Abstract
To describe the development of clinical signs (CS) and outcome of L-2-hydroxyglutaric aciduria (L-2-HGA), owners of 119 Staffordshire bull terriers positive for the known L-2-hydroxyglutarate dehydrogenase autosomal-recessive mutations were requested to complete a questionnaire regarding their pet's CS. Questionnaires were returned for 27 dogs, all with neurological abnormalities-not all questions were answered in all cases. The mean age of CS onset was 12 months (range 2.5-60). Gait dysfunction was reported in 26/26 dogs, with stiffness of all four limbs the most common (24/26) and earliest recognised abnormality. Kyphosis (19/26), body and/or head tremors (19/26) and hypermetria (15/26) were frequent. Behavioural changes were present in 24/27 dogs; most commonly staring into space (21/24), signs of dementia (17/24) and loss of training (15/24). Eighteen dogs demonstrated paroxysmal seizure-like/dyskinetic episodes. Nineteen (70 per cent) dogs were alive at a mean survival time of 76.6 months (12-170) after onset of CS. L-2-HGA was the cause of euthanasia in six dogs. Euthanasia occurred at a mean survival time of 44 months (8.5-93) after onset of CS, with 2/8 dogs euthanased within 12 months. L-2-HGA is considered a progressive neurological disease; however, CS can be successfully managed with affected dogs potentially living a normal lifespan.
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Search related cases →Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/27729589/