TY - JOUR
T1 - Focal epilepsy with fear-related behavior as primary presentation in Boerboel dogs
AU - Stassen, Quirine E M
AU - Grinwis, Guy C M
AU - van Rhijn, Nieke C
AU - Beukers, Martjin
AU - Verhoeven-Duif, Nanda M
AU - Leegwater, Peter A J
N1 - Publisher Copyright:
© 2018 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.
PY - 2019/3
Y1 - 2019/3
N2 - BACKGROUND: Focal seizures with fear as a primary ictal manifestation, their diagnostic challenges, and impact on quality of life are well described in human medicine. Reports focusing on ictal fear-like behavior in animals are scarce.OBJECTIVE: To describe the clinical and histopathological characteristics of a novel focal epilepsy in Boerboel dogs.ANIMALS: Five client-owned Boerboel littermates presented for evaluation of sudden episodes of severe fear-related behavior.METHODS: Clinical examination, complete blood cell count, routine blood biochemistry, and urinalysis were performed in all dogs. Magnetic resonance imaging (MRI) scans of the brain were performed in 3 affected Boerboels. In addition, in 2 affected Boerboels, metabolic screening, cerebrospinal fluid (CSF) analysis, and necropsy were performed.RESULTS: Onset of signs was 3 months of age in all affected Boerboels. All Boerboels howled loudly, had an extremely fearful facial expression and trembled during seizures. All affected Boerboels also had autonomic or motor signs. Results of laboratory investigations, diagnostic imaging, and metabolic screening were generally unremarkable. Histopathology showed moderate numbers of single large vacuoles in the perikaryon of neurons throughout the brain, specifically in the deeper cerebral cortical regions. Family history, pedigree analysis, and the homogenous phenotype were suggestive of autosomal recessive inheritance.CONCLUSIONS AND CLINICAL IMPORTANCE: The observed paroxysmal fear-related behavior represents a newly recognized hereditary focal epilepsy in dogs with distinctive clinical and histopathologic features. Veterinarians should be aware that sudden episodes of unusual behavior can represent focal epilepsy.
AB - BACKGROUND: Focal seizures with fear as a primary ictal manifestation, their diagnostic challenges, and impact on quality of life are well described in human medicine. Reports focusing on ictal fear-like behavior in animals are scarce.OBJECTIVE: To describe the clinical and histopathological characteristics of a novel focal epilepsy in Boerboel dogs.ANIMALS: Five client-owned Boerboel littermates presented for evaluation of sudden episodes of severe fear-related behavior.METHODS: Clinical examination, complete blood cell count, routine blood biochemistry, and urinalysis were performed in all dogs. Magnetic resonance imaging (MRI) scans of the brain were performed in 3 affected Boerboels. In addition, in 2 affected Boerboels, metabolic screening, cerebrospinal fluid (CSF) analysis, and necropsy were performed.RESULTS: Onset of signs was 3 months of age in all affected Boerboels. All Boerboels howled loudly, had an extremely fearful facial expression and trembled during seizures. All affected Boerboels also had autonomic or motor signs. Results of laboratory investigations, diagnostic imaging, and metabolic screening were generally unremarkable. Histopathology showed moderate numbers of single large vacuoles in the perikaryon of neurons throughout the brain, specifically in the deeper cerebral cortical regions. Family history, pedigree analysis, and the homogenous phenotype were suggestive of autosomal recessive inheritance.CONCLUSIONS AND CLINICAL IMPORTANCE: The observed paroxysmal fear-related behavior represents a newly recognized hereditary focal epilepsy in dogs with distinctive clinical and histopathologic features. Veterinarians should be aware that sudden episodes of unusual behavior can represent focal epilepsy.
KW - Animals
KW - Dog Diseases/diagnosis
KW - Dogs
KW - Epilepsies, Partial/diagnosis
KW - Fear/physiology
KW - Female
KW - Male
KW - Pedigree
U2 - 10.1111/jvim.15346
DO - 10.1111/jvim.15346
M3 - Article
C2 - 30580458
SN - 0891-6640
VL - 33
SP - 694
EP - 700
JO - Journal of Veterinary Internal Medicine
JF - Journal of Veterinary Internal Medicine
IS - 2
ER -