Cerebellar Degeneration in a Mature Staffordshire Terrier

Abstract

A 5-year-old Staffordshire terrier exhibited slowly progressive signs of cerebellar disease, including nystagmus and dysmetria. After a 30-month course, the dog was euthanized. Grossly, the cerebellum was small and comprised only 5% of the brain weight. Histopathological examination of the brain documented diffuse degeneration. Purkinje cells were most depleted, but granular cells and the molecular layer of cerebellum were also depleted. The history and necropsy examination were evidence of late-onset primary cerebellar degeneration.

Computer‐Assisted Magnetic Resonance Imaging Brain Morphometry in American Staffordshire Terriers with Cerebellar Cortical Degeneration

“…Cerebellar cortical degeneration in dogs has been reported in at least 27 different pure breeds and 1 mixed‐breed dog 1–40 . The onset of clinical signs differs among breeds but clinical signs usually start at <6 months of age 1,8,16–37,39 .…”

“…The onset of clinical signs differs among breeds but clinical signs usually start at <6 months of age 1,8,16–37,39 . Familial late‐onset cerebellar abiotrophies have been described in Gordon Setters, 2–4 Old English Sheepdogs, 5 Brittany Spaniels, 6,7 a Schnauzer‐Beagle dog, 9 Pit Bull Terriers, 15 Espagneul Bretons, 38 a Bernese Mountain dog, 40 and American Staffordshire Terriers 10–15 . Initially, clinical signs are very subtle, eg, an occasional sway of the body when making a sudden movement 13 .…”

“…Gross pathologic examination typically reveals diffuse atrophy of the cerebellum 4,8,12,13,19,22,31,33,39,40 . Histopathologic findings such as marked loss of Purkinje neurons, depletion of granular cell bodies, and shrinkage of the granular and molecular cell layer have been reported alone or in combination 2–40 . In breeds with familial late‐onset cerebellar abiotrophies, the mode of inheritance has been shown to be autosomal recessive in Old English Sheepdogs 5 and Gordon Setters 2,3 and it is suspected to be the same in American Staffordshire Terriers 13 .…”

Late‐onset cerebellar abiotrophy in a Labrador Retriever

“…4,5 Signs begin with generalised vestibular ataxia progressing to intention tremor, truncal sway, nystagmus, inconsistent menace responses and intermittent head tilt. American Staffordshire Terriers with late-onset CA develop clinical signs at 18-19 months of age.…”

“…1 Normal at birth, affected animals over weeks to months progressively develop dysmetria because of the lack of inhibitory Purkinje neurones input to the cerebellar and vestibular nuclei. 3 The late-onset form is uncommon and has been reported in American Staffordshire Terriers, 4,5 Old English Sheepdogs, 6 Gordon Setters, 7 Brittany Spaniels 8 and a Boxer. Early onset has been described in Portugese Podencos, Beagles and Rhodesian Ridgebacks and the intermediate onset form has been described in Labrador Retrievers, Border Collies, Rough-coated Collies, and English Bulldogs.…”