A 3-Dimensional Printed Patient-Specific Surgical Guide to Facilitate Transsphenoidal Hypophysectomy in Dogs.

Objective: Hypophysectomy in dogs is a difficult surgery that requires specific learning and training. We aimed to evaluate the accuracy of a 3-dimensional printed patient-specific surgical guide to facilitate choosing the entry point in the basisphenoid bone before approaching the sella turcica during transsphenoidal hypophysectomy in dogs. Methods: Two canine cadavers and 8 dogs undergoing transsphenoidal hypophysectomy for Cushing's disease treatment, involving design and fabrication of a 3-dimensional printed guide. The ideal entry point in the basisphenoid bone outer cortical layer was determined in each dog pre-operatively; its anatomical location was described with a set of measurements then compared to post-operative computed tomography measures describing the location of the outer cortical window created in the basisphenoid bone. Results: Several guide designs were proposed, and a consensus reached based on surgeons' experience performing hypophysectomy. The device chosen could be applied to the size and shape of skulls encountered in this case series. The pre-planned measurements were comparable to post-operative measurement (there was also no statistical difference), with median of differences <0.1 mm, which we judged as clinically acceptable. Clinical Significance: Hypophysectomy in dogs is a challenging procedure that has a learning curve and needs to be performed by specialist neurosurgeons. We propose that a low-profile 3-dimensional printed surgical guide can aid the specialist neurosurgeon to locate the burring site of the outer cortical layer of the basisphenoid bone at a pre-defined location and with good accuracy. It does not alleviate the need to understand the anatomy of the region and to know how to create a slot within the basisphenoid bone, which remains essential to enter the sella turcica. This device could help specialist veterinary neurosurgeons wishing to be trained to perform hypophysectomy.

Canine Lafora Disease: An Unstable Repeat Expansion Disorder.

Canine Lafora disease is a recessively inherited, rapidly progressing neurodegenerative disease caused by the accumulation of abnormally constructed insoluble glycogen Lafora bodies in the brain and other tissues due to the loss of NHL repeat containing E3 ubiquitin protein ligase 1 (NHLRC1). Dogs have a dodecamer repeat sequence within the NHLRC1 gene, which is prone to unstable (dynamic) expansion and loss of function. Progressive signs of Lafora disease include hypnic jerks, reflex and spontaneous myoclonus, seizures, vision loss, ataxia and decreased cognitive function. We studied five dogs (one Chihuahua, two French Bulldogs, one Griffon Bruxellois, one mixed breed) with clinical signs associated with canine Lafora disease. Identification of polyglucosan bodies (Lafora bodies) in myocytes supported diagnosis in the French Bulldogs; muscle areas close to the myotendinous junction and the myofascial union segment had the highest yield of inclusions. Postmortem examination of one of the French Bulldogs revealed brain Lafora bodies. Genetic testing for the known canine NHLRC1 mutation confirmed the presence of a homozygous mutation associated with canine Lafora disease. Our results show that Lafora disease extends beyond previous known breeds to the French Bulldog, Griffon Bruxellois and even mixed-breed dogs, emphasizing the likely species-wide nature of this genetic problem. It also establishes these breeds as animal models for the devastating human disease. Genetic testing should be used when designing breeding strategies to determine the frequency of the NHLRC1 mutation in affected breeds. Lafora diseases should be suspected in any older dog presenting with myoclonus, hypnic jerks or photoconvulsions.

Magnetic resonance imaging of a giant frontal hemorrhagic mucocele with intracranial extension in a cat.

A 6-year-old domestic short-haired cat was presented with an acute onset of right cortical encephalopathy. Magnetic resonance imaging (MRI) performed 4 days after the onset of clinical signs revealed a lesion originating from the right frontal sinus with intracranial extension and compression of the right frontal lobe. The lesion was T1-weighted hypointense and T2-weighted and fluid-attenuated inversion recovery hyperintense. Signal voids within the lesion were observed on T2* images, consistent with hemorrhage. Peripheral ring enhancement was visible on postcontrast sequences. These features were consistent with a giant hemorrhagic mucocele. To the authors' knowledge, this is the first report of MRI characteristics of this lesion in a cat.

Juvenile-onset polyneuropathy in American Staffordshire Terriers.

BACKGROUND: The only hereditary neurologic disorder described so far in American Staffordshire Terriers is adult-onset cerebellar degeneration secondary to ceroid lipofuscinosis. We have seen several dogs with a newly recognized neurological disease characterized by locomotor weakness with or without respiratory signs and juvenile onset consistent with degenerative polyneuropathy of genetic origin. OBJECTIVES: To characterize a novel polyneuropathy in juvenile American Staffordshire Terriers. ANIMALS: Fourteen American Staffordshire Terriers presented with clinical signs consistent with juvenile-onset polyneuropathy at 5 veterinary hospitals between May 2005 and July 2017. METHODS: Case series. Dogs were included retrospectively after a diagnosis of degenerative polyneuropathy had been confirmed by nerve biopsy. Clinical, pathological, electrophysiological, histological data, and outcome were reviewed and a pedigree analysis performed. RESULTS: All dogs displayed clinical signs of neuromuscular disease with generalized motor and sensory involvement, associated with focal signs of laryngeal paralysis (10/14 dogs) and megaesophagus (1/14 dogs). Histopathological findings were consistent with degenerative polyneuropathy. Follow-up was available for 11 dogs, and 3 dogs were euthanized shortly after diagnosis. In these 11 dogs, the disease was slowly progressive and the animals maintained good quality of life with ability to walk. Pedigree analysis was mostly consistent with an autosomal recessive mode of inheritance. CONCLUSIONS AND CLINICAL IMPORTANCE: Juvenile polyneuropathy, associated with laryngeal paralysis, is a newly described entity in American Staffordshire Terriers, and results from degenerative neuropathy. When surgery for laryngeal paralysis is performed, lifespan may be similar to that of normal dogs even though affected dogs have locomotor disturbance.