Zerebelläre kortikale Abiotrophie beim American Staffordshire Terrier: Klinische und pathologische Beschreibung von drei Fällen.

Abstract

Three American Staffordshire Terriers were presented with gait abnormalities and loss of balance at the age of 4.5 (female) and 6 years (2 males). The onset varied between 3 and 5 years of age and the clinical signs were slowly progressive. The neurological examination revealed symmetrical generalized cerebellar ataxia with hypermetria, stiffness, and loss of balance with no evidence of paresis. The menace reflex was decreased in one dog and absent in another. A positional nystagmus was found in two dogs. The dogs were euthanized and a histopathological examination of each brain was performed. Pathological changes were confined to the cerebellum. The main finding was loss of Purkinje cells, as well as depletion of granular cell bodies and shrinkage of the granular and molecular cell layer. These findings are consistent with cerebellar cortical abiotrophy. A genetic basis is supposed, but the mode of inheritance is not determined yet. In contrast to some spinocerebellar ataxias in humans, the cause of Purkinje cell degeneration in cerebellar cortical abiotrophy of dogs is not known.

Neuronale Zeroidlipofuszinose bei einem adulten American Staffordshire Terrier

“…Clinically, affected ASTs show heterogeneous symptoms (1, 15). Wide-based stance, generalized ataxia with hypermetria and stiffness are frequently seen in affected animals whereas some dogs additionally show cranial nerve deficits like head tilt, nystagmus as well as a reduced or absent menace response (15,34). In contrast to other dog breeds affected by NCL, including Australian She pherd, Border Collie and Tibetan Terrier (20,22,25) visual impairment is not a feature in AST dogs with NCL.…”

“…Instead, staggering and difficulties to balance sudden movements are also described (30,33). Like in this case, proprioception, nociception as well as peripheral reflexes may be unremarkable (15,30). These differences in the clinical presentation are supposed to reflect variable expressivity of the genetic defect and environmental or genetic interactions that may play a certain role in the manifestation of the disease (1).…”

“…This case report presents a case of late-onset NCL associated with cerebellar cortical degeneration in an American Staffordshire Terrier (AST). The first histopathological descriptions of ASTs with cerebellar ataxia included degeneration and loss of mainly Purkinje cells but also rarefication of the granular layer (15,34) and the disease was found to be associated with storage of a fluorescent lipopigment in affected neurons (33). The data of a study with more than 60 affected dogs suggest an autosomal recessive mode of inheritance with a frequency of the deleterious gene in pedigrees of AST in the USA of 39% (30).…”

“…In addition, congenital defects, neoplasia and traumatic lesions of the brain have to be considered as differential diagnoses. Cerebellar hypoplasia is a congenital defect and thus affected animals will show first clinical signs shortly after birth or at time of ambulation (15). Neoplasia will cause progressive cerebellar deficits, but those are supposed to occur often one-sided and larger lesions may compress adjacent cerebrum and brain stem with associated clinical signs (15).…”

La céroïde-lipofuscinose de l'American Staffordshire Terriers

“…Chez l'AST, une ataxie cérébelleuse, également appelée abiotrophie cérébelleuse, a été décrite dès 2003, en Europe et aux USA (Hanzlicek et al 2003 ;Olby et al 2004 Il n'a jamais été mis en évidence de modification comportementale, mise à part une certaine indifférence à l'environne-ment, en fin d'évolution de la maladie. La majorité des chiens atteints a présenté les premiers symptômes de l'ataxie entre les âges de trois à cinq ans (Olby et al 2004, Abitbol et al 2010.…”