neuronal ceroid lipofuscinosis in a tibetan terrier

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Page 1: Neuronal Ceroid Lipofuscinosis in a Tibetan Terrier

143:4, 2010 ESVP/ECVP Proceedings 2010 353

P13NEURONAL CEROID LIPOFUSCINOSIS IN A TIBETAN

TERRIERK. Agnieszka and F. Ehrensperger

Vetsuisse Faculty, Institute of Veterinary Pathology, SwitzerlandIntroduction: The neuronal ceroid lipofuscinoses (NCLs) area group of inherited (autosomal recessive) and progressive lysosomalstorage diseases characterized by the accumulation of autofluorescentinclusions (lipopigment) mainly in neurological tissue, the retina andmuscle, resulting in progressive neurodegeneration and prematuredeath.Materials and Methods: Ceroid lipofuscinosis was diagnosed ina 10-year-old Tibetan terrier on the basis of clinical (including genetesting) and histopathological findings. The animal was first pre-sented at 6 years of age with marked anxiety, nervousness, irritabilityand biting. Despite therapy with tranquilizers, the disease progressed.The dog was finally presented at 9e10 years of age with extreme anx-iety, amentia, aggression, loss of learned behaviour and nyctalopia.Results: Necropsy examination revealed moderate dilation of thelateral ventricles of the brain. The cerebrum and cerebellum showedslightly yellow discoloration of the parenchyma. Microscopically,within the cytoplasm of neurons and macrophages of the brain andspinal cord, there was marked accumulation of finely pale brown-grey material (HE). In some neurons there was eosinophilic and au-tofluorescent granular material. Special staining (PAS, LFB, ZN)confirmed lipopigment storage.Conclusions: The gross and microscopical findings, together withclinical data and the results of the gene test, confirmed the presenceof the canine form of late-onset NCL in this dog.

P14BONY FUSION AFTER REIMPLANTATION OF REMOVEDAUTOGENOUS CANCELLOUS BONE IN A DOG TREATED

SURGICALLY FOR DISC-ASSOCIATED WOBBLERSYNDROME BY A DISTRACTABLE VERTEBRAL TITANIUM

CAGEB. Wegge, S. De Decker, J.G.I. Caemaert, L. Van Ham

and K. ChiersGhent University, Belgium

Introduction: Disc-associated wobbler syndrome (DAWS) is themost common cause of cervical spondylomyelopathy in dogs and isgenerally treated by surgery. In this case the use of a distractabletitanium cage for surgical treatment was evaluated.Materials and Methods: A 10-year-old Dalmatian with DAWSwas submitted for surgical treatment. After total discectomy ofC5eC6 and C6eC7, a median corpectomy of C6 was performedwith a pneumatic drill. The removed cancellous bone was carefullycollected to act as an autogenous cancellous bone graft. Subsequently,the titanium implant was placed and affixed in the defect. Finally, itwas filled and covered with the collected cancellous bone. The dogimproved and survived for 22 months, but was humanely destroyedbecause of lumbosacral stenosis. The intervertebral tissue surroundingthe implant was removed and submitted for histopathological exam-ination to confirm bony fusion of the bone graft with the lateral edgesof the vertebral body.Results: Samples of the lateral and ventral sides showed mature can-cellous bone. The spinal cord section at the level of the implantshowed no signs of compression.Conclusions: This is the first report demonstrating bony fusion afterreimplantation of mechanically removed cancellous bone after a verte-bral corpectomy in a dog.

P15MORPHOLOGICAL STUDY OF THE OPTIC TECTUM OF

THE BEARDED DRAGONM.E. Mozos*, C.R. Jimenez*, M. Pumarola*, M. Ruiz*,

M. P�erez y and E. Blascoy*University of Cordoba and yAut�onomous University, Spain

Introduction: The optic tectum (OT) is one of the most complexstructures in the central nervous system (CNS) of lizards and receivesboth visual and auditory inputs. The aim of this study was to inves-tigate the OT of the bearded dragon (Pogona vitticeps) by histochem-ical and immunohistochemical techniques.Materials and Methods: Formalin-fixed and paraffin wax-embed-ded samples of the CNS of three healthy adults were used. Serial sam-ples of the mesencephalon were consecutively stained by HE, Nilssand Kluver-Barrera staining techniques, by silver impregnation andby the use of immunohistochemical markers for neuronal (anti-neu-ron-specific enolase [NSE], PGP 9.5 and neurofilament) and glial(GFAP, olig2, myelin proteolipid protein [PLP] and myelin basicprotein [MBP]) markers. The immunohistochemical study was car-ried out using the ABC method.Results: Correlation of the staining patterns demonstrated the highcomplexity in the distribution of neurons and nerve fibres. All anti-bodies showed cross-reactivity with the lizard CNS counterparts.NSE and PGP 9.5 reacted with numerous neurons and axons throughthe tectal layers. The neurofilament marker showed strong and diffusereaction with fibres. PLP and MBP antibodies reacted with myelin-ated fibres of different length and thickness.Conclusions: OT architecture in the bearded dragon appears sim-ilar to that of other lizards (brown anole [Anolis sagrei] and leopardgeeko [Eublepharis macularis]) and birds.

P16INCLUSION BODY DISEASE IN SNAKES: TISSUE

DISTRIBUTION AND MORPHOMETRIC ANALYSIS OFINCLUSIONS

M.E. Mozos, M.J. Ruiz, C.R. Jim�enez, J. P�erez, R. Zafraand A. P�erez-�Ecija

University of Cordoba, SpainIntroduction: Inclusion body disease (IBD) is a disorder of uncer-tain origin described in captive snakes (boids and vipers). Diagnosisis based on the observation of intracytoplasmic eosinophilic bodies(IBs), which can be present in virtually any cell type, but ante-mor-tem diagnosis is a challenge. The aim of this study was to analyze thetissue distribution and size of IBs in order to obtain data that facilitatecriteria for new diagnostic procedures.Materials and Methods: Formalin-fixed and paraffin wax-embed-ded samples from all organ systems of two boa constrictors presentingwith chronic and subacute clinical onset of IBD, respectively, werestudied. The number of IB-containing cells and the diameters ofthe IBs measured in 10 fields (�400) selected in five serial sectionsusing Image Pro Plus software were evaluated.Results: A variable number and size of IBs were found in the major-ity of tissues and organs in both animals; however, the Harderiangland (75% and 80% of affected cells per field, respectively), thevomeronasal organ (49% and 50%), kidney (26% and 39%) andliver (28% and 20%) were most affected. In relation to the mean di-ameter of IBs, the renal tubules showed the largest (1.8 mm), followedby the liver (1.68 mm), the Harderian gland (1.52 mm) and the vom-eronasal organ (1.2 mm).Conclusions: The Harderian gland and the vomeronasal organ arethe most suitable samples for diagnostic purposes.