Neuronal vacuolation and spinocerebellar degeneration in a Rottweiler puppy

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A four-month-old female Rottweiler was presented to the Cornell University Hospital for Animals for difficulty breathing, and progressive ataxia and paresis. She had been purchased from a breeder two and a half months prior to presentation. Since purchase, the dog was noted to be somewhat abnormal. She would run into things in the house, and pant often. The panting progressed to fits of respiratory distress. She had also begun showing signs of progressive ataxia and paresis. The patient was prescribed a non­steroidal anti-inflammatory drug, an antihistamine, and several antibiotics by the refe!Ting veterinarian, but continued to deteriorate despite treatment. She was refe1Ted to Cornell for further workup. On presentation to the Cornell University Hospital for Animals, the patient was mentally obtunded, tachypneic, markedly ataxic, and ambulatory paraparetic. She had stridorous breathing, likely secondary to laryngeal paralysis. Additionally, hypermetria, titubations, miotic pupils, positional vertical nystagmus, a changing strabismus, and decreased proprioception were noted on exam. The patient was neurolocalized to multifocal nervous system with suspected involvement of the brainstem, cerebellum, spinal cord and peripheral nerves (laryngeal paralysis). Further diagnostics including an MRI, CSF analysis, and bloodwork were unremarkable. The neurologic findings coupled with her signalment and normal ancillary tests were suggestive of a degenerative disease, with our top differential being a rare syndrome · called neuronal vacuolation and spinocerebellar degeneration. (NVSCD), Due to the grave prognosis and rapid progression of her clinical signs, euthanasia was elected. A necropsy was performed which confirmed the diagnosis of neuronal vacuolation and spinocerebellar degeneration. This report will describe the pertinent clinicopathologic findings associated with NVSCD and briefly review several other idiopathic degenerative diseases of young Rottweilers.

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neuronal vacuolation, spinocerebellar degeneration, laryngeal paralysis, Rottweilers, multifocal neurologic disorder


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