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FAMILIAL CEREBELLAR ATAXIA WITH HYDROCEPHALUS IN BULL MASTIFFS
Author(s) -
Johnson Ronald P.,
Neer T. Mark,
Partington Beth P.,
Cho DooYoun,
Partington Curtis R.
Publication year - 2001
Publication title -
veterinary radiology and ultrasound
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.541
H-Index - 60
eISSN - 1740-8261
pISSN - 1058-8183
DOI - 10.1111/j.1740-8261.2001.tb00934.x
Subject(s) - cerebellum , medicine , ataxia , dentate nucleus , fastigial nucleus , hydrocephalus , cerebellar ataxia , anatomy , cerebellar degeneration , pathology , neuroscience , vacuolization , biology , endocrinology , radiology , psychiatry
Familial cerebellar ataxia with concurrent hydrocephalus has previously been described in a family of bull mastiff pups, and recently has been identified in a litter from Louisiana. The 4 affected pups had ataxia, hypermetria, conscious proprioceptive deficits, behavioral abnormalities, and a visual deficit. In magnetic resonance imaging of the brain of two of the pups, there were symmetric hydrocephalus and two focal areas of increased signal intensity within the central nuclei of the cerebellum. Histopathologically there was vacuolization and mild astrogliosis within the deep cerebellar nuclei (dentate, interpositus, fastigial), caudal colliculi, and lateral vestibular nuclei. Although the postmortem results were not exactly the same as in the previously published report, the clinical features and histopathologic findings strongly support the diagnosis. This disorder is most likely inherited in an autosomal recessive manner.