46 results on '"Thibaud JL"'
Search Results
2. Unusual ocular lesions in a cat with a thiamine deficiency
- Author
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Chahory, S, Thibaud, JL, Colle, Marie-Anne, WYERS, Monique, Clerc, Bernard, Blot, Stéphane, ProdInra, Migration, and Inconnu
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[SDV] Life Sciences [q-bio] ,[SDV]Life Sciences [q-bio] ,ComputingMilieux_MISCELLANEOUS - Abstract
International audience
- Published
- 2006
3. A new familial nodo-paranodopathy in American Staffordshire Terriers
- Author
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Rosati, M, Vandenberghe, H, Escriou, C, Porcarelli, L, Recio Caride, A, Añor, S, Gandini, G, Corlazzoli, D, Thibaud, JL, Blot, S, and Matiasek, K
- Published
- 2019
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4. Mesoangioblast stem cells ameliorate muscle function in dystrophic dogs
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Giuseppe D'Antona, Orietta Pansarasa, Roberto Bottinelli, Laura Perani, Giulio Cossu, Maurilio Sampaolesi, Claudio Bordignon, Chiara Rinaldi, Nicolas Granger, Inès Barthélémy, Jean-Laurent Thibaud, Beatriz G. Gálvez, Yvan Torrente, M. Gabriella Cusella De Angelis, Sara Mantero, Maria Guttinger, Paolo Mognol, Stéphane Blot, Anna Innocenzi, Rossana Tonlorenzi, Sampaolesi, M, Blot, S, D’Antona, G, Granger, N, Tonlorenzi, R, Innocenzi, A, Mognol, P, Thibaud, Jl, Galvez, B, Barthélémy, I, Perani, L, Mantero, S, Guttinger, M, Pansarasa, O, Rinaldi, C, CUSELLA DE ANGELIS, Mg, Torrente, Y, Bordignon, Claudio, Bottinelli, R, and Cossu, G.
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Male ,Pathology ,Duchenne muscular dystrophy ,medicine.medical_treatment ,Expression ,Dystrophin ,stem cell terapy ,Myocyte ,MESOANGIOBLASTS ,STEM CELLS ,MUSCULAR DYSTROPHY ,Muscular dystrophy ,Creatine Kinase ,Skeletal-Muscle ,Multidisciplinary ,Duchenne Muscular-Dystrophy ,biology ,Stem-cell therapy ,Anatomy ,Combined Modality Therapy ,Multipotent ,Adult Stem Cells ,medicine.anatomical_structure ,Stem cell ,Delivery ,golden retriever dog model ,medicine.medical_specialty ,Recombinant Fusion Proteins ,Population ,Transplantation, Heterologous ,Gene-Therapy ,T-Cells ,Transplantation, Autologous ,Dogs ,medicine ,Animals ,Humans ,Transplantation ,Muscle Cells ,Mesoangioblast ,Skeletal muscle ,Genetic Therapy ,Muscular Dystrophy, Animal ,medicine.disease ,Muscular Dystrophy, Duchenne ,biology.protein ,Repair ,Stem Cell Transplantation - Abstract
Duchenne muscular dystrophy remains an untreatable genetic disease that severely limits motility and life expectancy in affected children. The only animal model specifically reproducing the alterations in the dystrophin gene and the full spectrum of human pathology is the golden retriever dog model. Affected animals present a single mutation in intron 6, resulting in complete absence of the dystrophin protein, and early and severe muscle degeneration with nearly complete loss of motility and walking ability. Death usually occurs at about 1 year of age as a result of failure of respiratory muscles. Here we report that intra-arterial delivery of wild-type canine mesoangioblasts (vessel-associated stem cells) results in an extensive recovery of dystrophin expression, normal muscle morphology and function ( confirmed by measurement of contraction force on single fibres). The outcome is a remarkable clinical amelioration and preservation of active motility. These data qualify mesoangioblasts as candidates for future stem cell therapy for Duchenne patients. ispartof: Nature vol:444 issue:7119 pages:574-579 ispartof: location:England status: published
- Published
- 2006
5. Suspected spontaneous early hemorrhagic transformation of multiple ischemic strokes secondary to primary splenic torsion in a German Shepherd dog.
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Van Caenegem N, Troupel T, Mortier J, Thibaud JL, and Blot S
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- Female, Dogs, Animals, Humans, Magnetic Resonance Imaging veterinary, Hemorrhage etiology, Hemorrhage veterinary, Ischemic Stroke veterinary, Splenic Diseases complications, Splenic Diseases surgery, Splenic Diseases veterinary, Stroke veterinary, Dog Diseases diagnostic imaging, Dog Diseases etiology
- Abstract
A 3-year-old female German Shepherd dog was presented with generalized tonic-clonic epileptic seizures, right-sided central vestibular syndrome, and right trigeminal nerve dysfunction. Acute lacunar ischemic strokes within both thalami, right side of the mesencephalon, left side of the myelencephalon, both sides of the cervical spinal cord, and acute hemorrhagic strokes within the rostral part of the right cerebellar hemisphere and right rostral colliculus were identified on magnetic resonance imaging. Additional evaluation identified multiple renal infarcts and complete splenic torsion, with entrapment of the left pancreatic lobe. Medical management, splenectomy, partial pancreatectomy, and intensive physical rehabilitation led to clinical improvement. The histology of the spleen was consistent with hemorrhagic infarction. Three months after onset, neurological examination identified only mild vestibular sequelae. The final diagnosis was multiple ischemic strokes secondary to primary splenic torsion. Spontaneous early hemorrhagic transformation, a well-known condition in human medicine, also was found in this case., (© 2022 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals LLC on behalf of American College of Veterinary Internal Medicine.)
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- 2022
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6. Epidemiological, clinical, and electrophysiological findings in dogs and cats with traumatic brachial plexus injury: A retrospective study of 226 cases.
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Troupel T, Van Caenegem N, Jeandel A, Thibaud JL, Nicolle A, and Blot S
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- Animals, Cats, Dogs, Retrospective Studies, Sensitivity and Specificity, Brachial Plexus, Cat Diseases, Dog Diseases diagnosis, Dog Diseases epidemiology
- Abstract
Background: The imaging and electrodiagnostic (EDX) characteristics of traumatic brachial plexus injury (TBPI) are incompletely reported., Objectives: To describe the epidemiological, clinical, and EDX characteristics of TBPIs in a series of cases in dogs and cats; to determine the association between clinical data, EDX findings, and clinical outcomes; and to assess the sensitivity and specificity of EDX studies to classify nerve lesions., Animals: One hundred and seventy-five dogs and 51 cats with TBPI and EDX exploration of radial nerve, ulnar nerve, or both nerves., Methods: Retrospective case series. All medical records were searched for dogs and cats presenting with TBPIs that underwent EDX exploration. Epidemiological, clinical, EDX, and follow-up data were extracted. Association between clinical data, EDX findings, and clinical outcomes was explored., Results: Forty-six percent of affected animals were injured before 2 years of age and 57% of dogs weighed more than 20 kg. The radial compound muscle action potential (CMAP) amplitude for dogs and cats that had clinical improvement was higher than in animals without improvement (4.3 mV [0-23.6] vs 0 mV [0-2.4], respectively, P = .02). A discriminating radial CMAP amplitude threshold value of 5 mV had a specificity of 93% (95% CI [80-100]) to predict recovery., Conclusions and Clinical Importance: Electrodiagnostic studies, particularly measurement of radial CMAP amplitude, are valuable diagnostic tests to refine the prognosis of these animals., (© 2021 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals LLC. on behalf of the American College of Veterinary Internal Medicine.)
- Published
- 2021
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7. Definitive-intent uniform megavoltage fractioned radiotherapy protocol for presumed canine intracranial gliomas: retrospective analysis of survival and prognostic factors in 38 cases (2013-2019).
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Debreuque M, De Fornel P, David I, Delisle F, Ducerveau MN, Devauchelle P, and Thibaud JL
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- Animals, Brain Neoplasms mortality, Brain Neoplasms radiotherapy, Dogs, Dose Fractionation, Radiation, Female, Glioma mortality, Glioma radiotherapy, Male, Prognosis, Quality of Life, Radiotherapy, High-Energy veterinary, Retrospective Studies, Survival Analysis, Treatment Outcome, Brain Neoplasms veterinary, Dog Diseases radiotherapy, Glioma veterinary
- Abstract
Background: Radiotherapy (RT) is currently considered the treatment of choice for presumed canine intracranial gliomas. However, variable therapeutic responses are described, due to heterogeneous populations and different radiation methods or protocols. Only one study dedicated to intracranial suspected glioma highlighted prognostic criteria. Determination or confirmation of specific clinical and imaging prognostic factors may guide the therapeutic management of these tumours. The objectives were to provide data on long-term clinical outcome (including quality of life, QoL) and to determine specific prognostic factors associated with survival time. We report a single-institution retrospective study, including all dogs with suspected symptomatic primary solitary intracranial glioma, treated with a complete uniform fractionated megavoltage radiation protocol of 15x3Gy over 5 weeks, between January 2013 and February 2019. Thirty-eight client-owned dogs were included. Medical records were retrospectively evaluated for median overall survival time (MST), clinical and imaging responses. Prognostic factors on survival were researched in terms of signalment, clinical presentation, tumour imaging characteristics and response following RT. Finally, the RT's impact on the dogs' clinical signs and Qol were evaluated by the owners., Results: The disease-specific MST was 698 days (95% CI: 598-1135). Survival at 1 and 2 years were respectively 74.2 ± 7.4% and 49.0 ± 9.8%. Initial clinical signs were related to survival, as well as tumour characteristics such as cystic-pattern, mass effect and Tumour/Brain volume ratio. No significant adverse effect or radiotoxicity was observed., Conclusions: RT appears as a safe and effective treatment for canine intracranial gliomas, allowing long-term tumour control, improvement of life's quality and management of associated clinical signs. The initial clinical signs and MRI characteristics (Tumour/Brain volume ratio, cyst-like lesion and mass effect) may help predict the prognosis.
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- 2020
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8. Case Report: Intramedullary Intervertebral Disk Extrusion in a Cat: Clinical, Computed Tomographic, High-Field Magnetic Resonance Imaging, and Outcome Findings.
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Debreuque M, Valin I, Prata D, De Fornel P, and Thibaud JL
- Abstract
Background: Intramedullary disk extrusions has rarely been described in veterinary medicine, more especially in cats, with only two cases are reported in the veterinary literature. Diagnosis may be difficult, even though clinical presentation and imaging studies, such as MRI or CT, can present specific features. Treatment and prognosis are not clearly described. Case presentation: A 10-year-old domestic shorthair female cat was evaluated for a 12 h-history of peracute-onset of paraparesis with flaccid tail and urinary and fecal incontinence. The patellar reflexes were normal, the pelvic flexor reflexes were decreased (more markedly on the right limb) and the perianal reflex was absent. The tail was flaccid, without nociception. Abdominal palpation revealed a small urinary bladder, easily expressed. Manipulation of the lumbar vertebral column elicited marked pain. Neurological examination was consistent with a L7-caudal segments lesion. A lumbosacral MRI and CT evaluations were performed and revealed a focal intramedullary hemorrhagic lesion, with an associated vertical linear tract communicating with the L5-L6 intervertebral disk space, and a suspected intramedullary focus of mineralization. These imaging findings were highly suggestive of an L5-L6 intramedullary disk extrusion. A dorsal L5-L6 laminectomy confirmed the presence of intramedullary degenerative nucleus pulposus fragments, which were surgically removed. Rapid and progressive neurological improvement was observed post-surgery. At the 1-year follow-up, right plantigrade stance and mild paraparesis were still noticed, but jumps and voluntary tail movements were observed. Occasional urinary and fecal incontinence episodes remained. Conclusions: This is the first feline case report of an intramedullary disk herniation with long-term follow-up available. Clinical description, CT and High-Field MRI findings, surgical procedure and histological results are reported, and help describing the characteristics of this rare non-compressive category of peracute intervertebral disk extrusion. Surgical management may be considered in feline cases of intramedullary disk herniation and may be associated with a good outcome., (Copyright © 2020 Debreuque, Valin, Prata, De Fornel and Thibaud.)
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- 2020
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9. Magnetic resonance imaging of a giant frontal hemorrhagic mucocele with intracranial extension in a cat.
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Vandenberghe H, Thibaud JL, Moissonnier P, and Blot S
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- Animals, Cats, Hemorrhage diagnostic imaging, Male, Mucocele diagnostic imaging, Cat Diseases diagnostic imaging, Hemorrhage veterinary, Magnetic Resonance Imaging veterinary, Mucocele veterinary
- Abstract
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., (© 2018 American College of Veterinary Radiology.)
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- 2020
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10. X-linked muscular dystrophy in a Labrador Retriever strain: phenotypic and molecular characterisation.
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Barthélémy I, Calmels N, Weiss RB, Tiret L, Vulin A, Wein N, Peccate C, Drougard C, Beroud C, Deburgrave N, Thibaud JL, Escriou C, Punzón I, Garcia L, Kaplan JC, Flanigan KM, Leturcq F, and Blot S
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- Animals, Dogs, Genes, Modifier, Male, Muscle, Skeletal metabolism, Muscle, Skeletal pathology, Muscular Dystrophy, Duchenne pathology, Mutation, Disease Models, Animal, Dystrophin genetics, Muscular Dystrophy, Duchenne genetics, Phenotype
- Abstract
Background: Canine models of Duchenne muscular dystrophy (DMD) are a valuable tool to evaluate potential therapies because they faithfully reproduce the human disease. Several cases of dystrophinopathies have been described in canines, but the Golden Retriever muscular dystrophy (GRMD) model remains the most used in preclinical studies. Here, we report a new spontaneous dystrophinopathy in a Labrador Retriever strain, named Labrador Retriever muscular dystrophy (LRMD)., Methods: A colony of LRMD dogs was established from spontaneous cases. Fourteen LRMD dogs were followed-up and compared to the GRMD standard using several functional tests. The disease causing mutation was studied by several molecular techniques and identified using RNA-sequencing., Results: The main clinical features of the GRMD disease were found in LRMD dogs; the functional tests provided data roughly overlapping with those measured in GRMD dogs, with similar inter-individual heterogeneity. The LRMD causal mutation was shown to be a 2.2-Mb inversion disrupting the DMD gene within intron 20 and involving the TMEM47 gene. In skeletal muscle, the Dp71 isoform was ectopically expressed, probably as a consequence of the mutation. We found no evidence of polymorphism in either of the two described modifier genes LTBP4 and Jagged1. No differences were found in Pitpna mRNA expression levels that would explain the inter-individual variability., Conclusions: This study provides a full comparative description of a new spontaneous canine model of dystrophinopathy, found to be phenotypically equivalent to the GRMD model. We report a novel large DNA mutation within the DMD gene and provide evidence that LRMD is a relevant model to pinpoint additional DMD modifier genes.
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- 2020
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11. In vivo stem cell tracking using scintigraphy in a canine model of DMD.
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Barthélémy I, Thibaud JL, de Fornel P, Cassano M, Punzón I, Mauduit D, Vilquin JT, Devauchelle P, Sampaolesi M, and Blot S
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- Animals, Cell Differentiation physiology, Cell Tracking methods, Disease Models, Animal, Dogs, Dystrophin metabolism, Female, Male, Muscle, Skeletal metabolism, Muscle, Skeletal pathology, Muscular Dystrophy, Animal metabolism, Muscular Dystrophy, Duchenne metabolism, Radionuclide Imaging methods, Stem Cells metabolism, Tissue Distribution physiology, Cell Movement physiology, Muscular Dystrophy, Animal pathology, Muscular Dystrophy, Duchenne pathology, Stem Cells pathology
- Abstract
One of the main challenges in cell therapy for muscle diseases is to efficiently target the muscle. To address this issue and achieve better understanding of in vivo cell fate, we evaluated the relevance of a non-invasive cell tracking method in the Golden Retriever Muscular Dystrophy (GRMD) model, a well-recognised model of Duchenne Muscular Dystrophy (DMD). Mesoangioblasts were directly labelled with
111 In-oxine, and injected through one of the femoral arteries. The scintigraphy images obtained provided the first quantitative mapping of the immediate biodistribution of mesoangioblasts in a large animal model of DMD. The results revealed that cells were trapped by the first capillary filters: the injected limb and the lung. During the days following injection, radioactivity was redistributed to the liver. In vitro studies, performed with the same cells prepared for injecting the animal, revealed prominent cell death and111 In release. In vivo, cell death resulted in111 In release into the vasculature that was taken up by the liver, resulting in a non-specific and non-cell-bound radioactive signal. Indirect labelling methods would be an attractive alternative to track cells on the mid- and long-term.- Published
- 2020
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12. Symptomatic lateral ventricular cystic lesion in a young cat.
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Debreuque M, Ducerveau MN, Valin I, de Fornel P, Manassero M, and Thibaud JL
- Abstract
Case Summary: A 1.5-year-old male neutered Persian cat was referred for acute deterioration of chronic left head tilt and ataxia. A lateral intraventricular cystic lesion, closely associated with the left choroid plexus, was identified on MRI. The intralesional signal intensity and cytological analysis of the fluid revealed a liquid similar to cerebrospinal fluid. After trepanation, an endoscopic-assisted fenestration and aspiration of the cyst were performed to temporally relieve the high intracranial pressure while waiting for surgical cystoperitoneal shunt placement. Three weeks after surgery, clinical relapse and recurrence of the lesion were noted on the pre-cystoperitoneal shunting MRI. During anaesthesia, the cat arrested. Cardiac resuscitation was successfully performed and cystoperitoneal shunting was postponed. Global brain ischaemia was then suspected, based on major forebrain clinical signs and MRI abnormalities. During a 6-month recovery period, a further three fine-needle CT-guided aspirations of the lesion were required, owing to clinical recurrence and increased cyst size. Cystoperitoneal shunting was eventually performed, allowing persistent reduction of the lesion and long-term improvement of the cat's neurological status., Relevance and Novel Information: This is the first report of a symptomatic lateral intraventricular cystic lesion in a cat. A left lateral intraventricular choroid plexus cyst was suspected based on the MRI features. Our case suggests that endoscopic fenestration and CT-guided aspiration are not adequate treatments for long-term management. Cystoperitoneal shunting may be a safe procedure, allowing significant and stable reduction of the cystic lesion, associated with improvement in the cat's neurological status by preventing high intracranial pressure., Competing Interests: Conflict of interest: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2020.)
- Published
- 2020
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13. In Vivo Myoblasts Tracking Using the Sodium Iodide Symporter Gene Expression in Dogs.
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Punzón I, Mauduit D, Holvoet B, Thibaud JL, de Fornel P, Deroose CM, Blanchard-Gutton N, Vilquin JT, Sampaolesi M, Barthélémy I, and Blot S
- Abstract
Stem cell-based therapies are a promising approach for the treatment of degenerative muscular diseases; however, clinical trials have shown inconclusive and even disappointing results so far. Noninvasive cell monitoring by medicine imaging could improve the understanding of the survival and biodistribution of cells following injection. In this study, we assessed the canine sodium iodide symporter (cNIS) reporter gene as an imaging tool to track by single-photon emission computed tomography (SPECT/CT) transduced canine myoblasts after intramuscular (IM) administrations in dogs. cNIS-expressing cells kept their myogenic capacities and showed strong
99 m Tc-pertechnetate (99 m TcO4 - ) uptake efficiency both in vitro and in vivo . cNIS expression allowed visualization of cells by SPECT/CT along time: 4 h, 48 h, 7 days, and 30 days after IM injection; biopsies collected 30 days post administration showed myofiber's membranes expressing cNIS. This study demonstrates that NIS can be used as a reporter to track cells in vivo in the skeletal muscle of large animals. Our results set a proof of concept of the benefits NIS-tracking tool may bring to the already challenging cell-based therapies arena in myopathies and pave the way to a more efficient translation to the clinical setting from more accurate pre-clinical results., (© 2020 The Authors.)- Published
- 2020
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14. Canine neuropathies: powerful spontaneous models for human hereditary sensory neuropathies.
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Correard S, Plassais J, Lagoutte L, Botherel N, Thibaud JL, Hédan B, Richard L, Lia AS, Delague V, Mège C, Mathis S, Guaguère E, Paradis M, Vallat JM, Quignon P, and André C
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- Animals, Disease Models, Animal, Dogs, Female, Genetic Predisposition to Disease genetics, Humans, Inbreeding, Male, Hereditary Sensory and Autonomic Neuropathies genetics, Hereditary Sensory and Autonomic Neuropathies veterinary
- Abstract
In humans, hereditary sensory neuropathies (HSN), also known as hereditary sensory and autonomic neuropathies (HSAN), constitute a clinically and genetically heterogeneous group of disorders characterized by progressive sensory loss, often accompanied by chronic skin ulcerations and nail dystrophic changes. To date, although around 20 genes have already been discovered, they do not explain the genetic causes of all patients. In dogs, similar neuropathies are also diagnosed, several breeds being predisposed to specific forms of the disease. Indeed, the breed specificity of most canine genetic diseases is due to the small numbers of founders and high levels of inbreeding. Recent knowledge and tools developed to study the canine genome efficiently allows deciphering the genetic bases of such diseases. To date, a dozen breeds are recognized to develop specific HSN. For the Border collie and hunting dog breeds, the genes involved have recently been discovered. Other affected breeds thus constitute potential genetic models, with new genes to be found in dogs that can be considered as candidate genes for human HSAN/HSN. Here, we review the different forms of human and canine HSAN/HSN and we present a novel form in Fox terrier cases, highlighting the advantages of the dog model for such rare human diseases.
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- 2019
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15. Juvenile-onset polyneuropathy in American Staffordshire Terriers.
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Vandenberghe H, Escriou C, Rosati M, Porcarelli L, Recio Caride A, Añor S, Gandini G, Corlazzoli D, Thibaud JL, Matiasek K, and Blot S
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- Animals, Biopsy veterinary, Dog Diseases genetics, Dogs, Electromyography veterinary, Female, Male, Muscle, Skeletal pathology, Neural Conduction, Pedigree, Peripheral Nerves pathology, Polyneuropathies genetics, Polyneuropathies pathology, Retrospective Studies, Dog Diseases pathology, Polyneuropathies veterinary
- Abstract
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., (© 2018 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.)
- Published
- 2018
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16. Bone haemophilic pseudotumour of the ulna: A rare complication of haemophilia in a dog.
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Decambron A, Manassero M, Thibaud JL, Reyes-Gomez E, and Viateau V
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- Animals, Bone Diseases diagnosis, Bone Diseases surgery, Dogs, Hemophilia A complications, Hemophilia A surgery, Magnetic Resonance Imaging, Male, Tomography, X-Ray Computed, Bone Diseases veterinary, Hemophilia A veterinary, Ulna
- Abstract
Objectives: This case report describes for the first time a bone haemophilic pseudotumour in a dog., Case Description: A seven-month-old German Shepherd male dog was presented with the complaint of a forelimb weight-bearing lameness with major swelling that expanded dramatically after fine needle aspiration. Radiographs showed a large, well-defined ulnar diaphyseal cystic-like osteolytic lesion. Based on prolonged activated partial thromboplastin time (aPTT) and low factor VIII activity, haemophilia A was diagnosed. Bone scintigraphy, computed tomography, magnetic resonance imaging, and histological findings definitely ruled out malignant neoplasia or inflammation and strongly supported a bone haemophilic pseudotumour over an aneurysmal bone cyst. Segmental ulnar resection and replacement by a polymethylmethacrylate spacer combined with perioperative bleeding management resulted in a successful outcome., Discussion: This case provided evidence that a bone haemophilic pseudotumour may be the sole presenting clinical sign of haemophilia A in dogs. Early diagnosis, based on history and magnetic resonance imaging findings, is imperative for prompt treatment leading to successful outcome. It is challenging as fine needle aspiration or biopsy is contraindicated. As described in humans, surgical excision of the lesion combined with management of severe postoperative bleeding was associated with successful outcome in the present case., Clinical Significance: A bone haemophilic pseudotumour should be considered in the differential diagnosis of expanding mass associated with osteolysis, especially in young male dogs. Perioperative monitoring of the bleeding disorder and subsequent FVIII replacement therapy was of paramount importance in the present case.
- Published
- 2017
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17. Anatomical and mesoscopic characterization of the dystrophic diaphragm: An in vivo nuclear magnetic resonance imaging study in the Golden retriever muscular dystrophy dog.
- Author
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Thibaud JL, Matot B, Barthélémy I, Fromes Y, Blot S, and Carlier PG
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- Animals, Dogs, Dystrophin deficiency, Magnetic Resonance Imaging, Diaphragm diagnostic imaging, Muscular Dystrophy, Animal diagnostic imaging
- Abstract
Because respiratory failure remains a major issue in Duchenne Muscular Dystrophy patients, respiratory muscles are a key target of systemic therapies. In the Golden Retriever Muscular Dystrophy (GRMD) dogs, the disease shows strong clinical and histological similarities with the human pathology, making it a valuable model for preclinical therapeutic trials. We report here the first nuclear magnetic resonance (NMR) imaging anatomical study of the diaphragm in GRMD dogs and healthy controls. Both T1- and T2-weighted images of the diaphragm of seven healthy and thirteen GRMD dogs, from 3 to 36 months of age, were acquired on a 3 tesla NMR scanner. Abnormalities of texture and shape were revealed and consisted of increases in signal intensity on T2-weighted images and in signal heterogeneity on both T1- and T2-weighted images of the dystrophic diaphragm. These abnormalities were associated with a significant thickening of the muscle and we identified a clear 8-mm-threshold distinguishing clinically preserved GRMD dogs from those more severely affected. In this study, we demonstrated the feasibility of NMR imaging of the diaphragm and depicted several anatomical and mesoscopic anomalies in the dystrophic diaphragm. NMR imaging of the diaphragm shows a promise as an outcome measure in preclinical trials using GRMD dogs., (Copyright © 2017 Elsevier B.V. All rights reserved.)
- Published
- 2017
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18. Facial and vestibular neuropathy of unknown origin in 16 dogs.
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Jeandel A, Thibaud JL, and Blot S
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- Animals, Bell Palsy diagnosis, Bell Palsy diagnostic imaging, Bell Palsy pathology, Bell Palsy veterinary, Dog Diseases diagnostic imaging, Dog Diseases pathology, Dogs, Female, Follow-Up Studies, Magnetic Resonance Imaging veterinary, Male, Retrospective Studies, Vestibular Neuronitis diagnosis, Vestibular Neuronitis diagnostic imaging, Vestibular Neuronitis pathology, Dog Diseases diagnosis, Vestibular Neuronitis veterinary
- Abstract
Objectives: The aim of this study was to describe the signalment, clinical presentation, diagnostic findings and long-term follow-up in dogs with concomitant facial and vestibular neuropathy of unknown origin., Methods: Appropriate cases were located through medical record searches. Inclusion criteria comprised dogs that had: clinical signs of facial paralysis with concomitant peripheral vestibular syndrome, thyroid function tests, no abnormalities on magnetic resonance imaging of the brain and tympanic bullae, and cerebrospinal fluid analysis., Results: Sixteen dogs met the inclusion criteria. Facial paralysis had acute onset (<24 hours) in all dogs, thyroid function was within normal limits. There was albuminocytologic dissociation in cerebrospinal fluid of 69% of the dogs. There was complete resolution of clinical signs in 31% of the dogs but 38% showed long-term vestibular deficits, 46% developed hemifacial contracture, 15% had permanent facial paralysis and 15% relapsed., Clinical Significance: Facial and vestibular neuropathy of unknown origin shares similarities with idiopathic facial paralysis. The prognosis for return of normal facial and vestibular function is guarded and there may be relapse after recovery., (© 2016 British Small Animal Veterinary Association.)
- Published
- 2016
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19. Forelimb treatment in a large cohort of dystrophic dogs supports delivery of a recombinant AAV for exon skipping in Duchenne patients.
- Author
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Le Guiner C, Montus M, Servais L, Cherel Y, Francois V, Thibaud JL, Wary C, Matot B, Larcher T, Guigand L, Dutilleul M, Domenger C, Allais M, Beuvin M, Moraux A, Le Duff J, Devaux M, Jaulin N, Guilbaud M, Latournerie V, Veron P, Boutin S, Leborgne C, Desgue D, Deschamps JY, Moullec S, Fromes Y, Vulin A, Smith RH, Laroudie N, Barnay-Toutain F, Rivière C, Bucher S, Le TH, Delaunay N, Gasmi M, Kotin RM, Bonne G, Adjali O, Masurier C, Hogrel JY, Carlier P, Moullier P, and Voit T
- Subjects
- Animals, Cohort Studies, Disease Models, Animal, Dogs, Dose-Response Relationship, Drug, Exons, Genetic Therapy, Genetic Vectors administration & dosage, Humans, Infusions, Intravenous, Muscular Dystrophy, Duchenne genetics, Muscular Dystrophy, Duchenne physiopathology, RNA, Small Nuclear metabolism, Dependovirus genetics, Dystrophin genetics, Forelimb physiopathology, Muscular Dystrophy, Duchenne therapy, RNA, Small Nuclear genetics
- Abstract
Duchenne muscular dystrophy (DMD) is a severe muscle-wasting disorder caused by mutations in the dystrophin gene, without curative treatment yet available. Our study provides, for the first time, the overall safety profile and therapeutic dose of a recombinant adeno-associated virus vector, serotype 8 (rAAV8) carrying a modified U7snRNA sequence promoting exon skipping to restore a functional in-frame dystrophin transcript, and injected by locoregional transvenous perfusion of the forelimb. Eighteen Golden Retriever Muscular Dystrophy (GRMD) dogs were exposed to increasing doses of GMP-manufactured vector. Treatment was well tolerated in all, and no acute nor delayed adverse effect, including systemic and immune toxicity was detected. There was a dose relationship for the amount of exon skipping with up to 80% of myofibers expressing dystrophin at the highest dose. Similarly, histological, nuclear magnetic resonance pathological indices and strength improvement responded in a dose-dependent manner. The systematic comparison of effects using different independent methods, allowed to define a minimum threshold of dystrophin expressing fibers (>33% for structural measures and >40% for strength) under which there was no clear-cut therapeutic effect. Altogether, these results support the concept of a phase 1/2 trial of locoregional delivery into upper limbs of nonambulatory DMD patients.
- Published
- 2014
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20. Corrigendum: Mesoangioblast stem cells ameliorate muscle function in dystrophic dogs.
- Author
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Sampaolesi M, Blot S, D'Antona G, Granger N, Tonlorenzi R, Innocenzi A, Mognol P, Thibaud JL, Galvez BG, Barthélémy I, Perani L, Mantero S, Guttinger M, Pansarasa O, Rinaldi C, De Angelis MG, Torrente Y, Bordignon C, Bottinelli R, and Cossu G
- Published
- 2013
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21. Muscle function recovery in golden retriever muscular dystrophy after AAV1-U7 exon skipping.
- Author
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Vulin A, Barthélémy I, Goyenvalle A, Thibaud JL, Beley C, Griffith G, Benchaouir R, le Hir M, Unterfinger Y, Lorain S, Dreyfus P, Voit T, Carlier P, Blot S, and Garcia L
- Subjects
- Animals, Base Sequence, Calcium metabolism, Dogs, Exons, Forelimb physiopathology, Genetic Therapy, Genetic Vectors administration & dosage, Injections, Intramuscular, Molecular Sequence Data, Muscle Contraction, Muscle Fibers, Skeletal metabolism, Muscle Fibers, Skeletal pathology, Muscle Strength, Muscle, Skeletal metabolism, Muscle, Skeletal pathology, Muscle, Skeletal physiopathology, Muscular Dystrophy, Animal physiopathology, Transcription, Genetic, Utrophin genetics, Utrophin metabolism, Alternative Splicing, Dependovirus genetics, Dystrophin genetics, Muscular Dystrophy, Animal therapy, Oligoribonucleotides, Antisense genetics, RNA, Small Nuclear genetics
- Abstract
Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder resulting from lesions of the gene encoding dystrophin. These usually consist of large genomic deletions, the extents of which are not correlated with the severity of the phenotype. Out-of-frame deletions give rise to dystrophin deficiency and severe DMD phenotypes, while internal deletions that produce in-frame mRNAs encoding truncated proteins can lead to a milder myopathy known as Becker muscular dystrophy (BMD). Widespread restoration of dystrophin expression via adeno-associated virus (AAV)-mediated exon skipping has been successfully demonstrated in the mdx mouse model and in cardiac muscle after percutaneous transendocardial delivery in the golden retriever muscular dystrophy dog (GRMD) model. Here, a set of optimized U7snRNAs carrying antisense sequences designed to rescue dystrophin were delivered into GRMD skeletal muscles by AAV1 gene transfer using intramuscular injection or forelimb perfusion. We show sustained correction of the dystrophic phenotype in extended muscle areas and partial recovery of muscle strength. Muscle architecture was improved and fibers displayed the hallmarks of mature and functional units. A 5-year follow-up ruled out immune rejection drawbacks but showed a progressive decline in the number of corrected muscle fibers, likely due to the persistence of a mild dystrophic process such as occurs in BMD phenotypes. Although AAV-mediated exon skipping was shown safe and efficient to rescue a truncated dystrophin, it appears that recurrent treatments would be required to maintain therapeutic benefit ahead of the progression of the disease.
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- 2012
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22. Electrophysiological features in dogs with peripheral nerve sheath tumors: 51 cases (1993-2010).
- Author
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le Chevoir M, Thibaud JL, Labruyère J, Uriarte A, De Fornel-Thibaud P, Moissonnier P, Delisle F, and Blot S
- Subjects
- Action Potentials physiology, Animals, Dogs, Electromyography veterinary, Female, Magnetic Resonance Imaging, Male, Muscle Neoplasms physiopathology, Muscle, Skeletal innervation, Neoplasm Invasiveness, Nerve Sheath Neoplasms physiopathology, Neural Conduction physiology, Retrospective Studies, Tomography, X-Ray Computed, Dog Diseases physiopathology, Muscle Neoplasms veterinary, Muscle, Skeletal physiopathology, Nerve Sheath Neoplasms veterinary
- Abstract
Objective: To determine the electrophysiological changes in dogs with peripheral nerve sheath tumors (PNSTs), evaluate the prevalence of these changes, assess the correlation between spontaneous activity in epaxial muscles and proximal invasion by the tumor, and evaluate whether knowledge of electrophysiological changes could be helpful in the imaging diagnosis via CT or MRI., Design: Retrospective case series., Animals: 51 dogs with a histologic (n = 18) or a suspected (33) diagnosis of PNST., Procedures: Clinical, postmortem, and histologic reports and details of electrodiagnostic procedures and CT or MRI reports were studied. Twenty-four CT and 6 MRI reports for dogs with PNSTs were reviewed by a single observer blinded to the diagnosis., Results: Only 2 of the 51 dogs had no electrophysiological changes. The most commonly affected muscles were those innervated by the radial, ulnar, median, tibial-sciatic, and peroneal nerves. Abnormal spontaneous epaxial muscle activity was significantly more frequent in the group with foraminal or spinal invasion by the tumors. Knowledge of the electrophysiological changes increased diagnostic accuracy of CT., Conclusions and Clinical Relevance: Results suggested that electrophysiological studies may be sensitive for the detection of PNST and helpful in the imaging diagnosis. Epaxial electromyographic abnormalities appeared to be predictive for intervertebral or vertebral canal invasion by PNSTs in dogs.
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- 2012
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23. Comprehensive longitudinal characterization of canine muscular dystrophy by serial NMR imaging of GRMD dogs.
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Thibaud JL, Azzabou N, Barthelemy I, Fleury S, Cabrol L, Blot S, and Carlier PG
- Subjects
- Animals, Dogs, Image Processing, Computer-Assisted, Longitudinal Studies, Magnetic Resonance Spectroscopy methods, Muscle, Skeletal pathology, Muscular Dystrophy, Animal diagnosis
- Abstract
The Golden Retriever Muscular Dystrophy (GRMD) dog is the closest animal counterpart of Duchenne muscular dystrophy in humans and has, for this reason, increasingly been used in preclinical therapeutic trials for this disease. The aim of this study was to describe the abnormalities in canine dystrophic muscle non-invasively, quantitatively, thoroughly and serially by means of NMR imaging. Thoracic and pelvic limbs of five healthy and five GRMD dogs were imaged in a 3T NMR scanner at 2, 4, 6 and 9months of age. Standard and fat-saturated T(1)-, T(2)- and proton-density-weighted images were acquired. A measurement of T(1) and a two-hour kinetic study of muscle enhancement after gadolinium-chelate injection were also performed. Ten out of the 15 indices evaluated differed between healthy and GRMD dogs. The maximal relative enhancement after gadolinium injection and the proton-density-weighted/T(2)-weighted signal ratio were the most discriminating indices. Inter-muscle heterogeneity was found to vary significantly for most of the indices. The body of data that has been acquired here will help in designing and interpreting preclinical trials using dystrophin-deficient dogs., (Copyright © 2012 Elsevier B.V. All rights reserved.)
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- 2012
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24. Splitting of Pi and other ³¹P NMR anomalies of skeletal muscle metabolites in canine muscular dystrophy.
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Wary C, Naulet T, Thibaud JL, Monnet A, Blot S, and Carlier PG
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- Animals, Dogs, Magnetic Phenomena, Muscular Dystrophy, Animal pathology, Phosphorus Isotopes, Rest, Magnetic Resonance Spectroscopy, Metabolome, Muscle, Skeletal metabolism, Muscle, Skeletal pathology, Muscular Dystrophy, Animal metabolism, Phosphates metabolism
- Abstract
Many anomalies exist in the resting (31) P muscle spectra of boys with Duchenne muscular dystrophy (DMD) but few have been reported in Golden Retriever muscular dystrophy (GRMD), the closest existing animal model for DMD. Because GRMD is recommended for preclinical evaluation of therapies and quantitative outcome measures are needed, we investigated anomalies of (31) P NMRS in tibial cranial and biceps femoris muscles from 14 GRMD compared to 9 control (CONT) dogs. Alterations observed in DMD children - low phosphocreatine and high phospho-monoesters and -diesters - were all found in GRMD but increased pH was not. More surprisingly, inorganic phosphate (Pi) appeared to present a prominent splitting with an enhanced Pi(b) resonance at 0.3 ppm downfield of Pi(a) . Assuming that both resonances are Pi, the pH for Pi(a) in GRMD corresponded to a physiological intracellular pH(a) (6.97 ± 0.05), while pH(b) approached the extracellular range (7.27 ± 0.10) and correlated with pH(a) in GRMD (R(2) = 0.65). Both Pi(a) and Pi(b) were elevated compared to CONT and Pi(a) increased with age for GRMD (R(2) = 0.48, p < 0.001). Magnetisation transfer experiments between γATP and Pi were conducted to better characterise Pi pools. Equal T1 relaxation times for Pi(b) and Pi(a) did not support a mitochondrial origin of Pi(b) . We suggest that Pi(b) could originate from degenerating hypercontracted cells that have a leaky membrane and inadequate cell homeostasis and pH regulation. Pi(b) showed minimal chemical exchange in all dogs, while the exchange rate of Pi(a) was reduced in GRMD and might extraneously reflect low glycolytic activity in DMD. Taken together, the ensemble of (31) P NMRS alterations identifies muscle dysfunction and could provide useful biomarkers of therapeutic efficacy. Furthermore, among these, two might relate more specifically to dystrophic processes and merit further investigation: one is the existence of the enhanced alkaline Pi(b) pool; the other, mechanisms by which membrane disruption might increase phosphodiesters in dystrophy., (Copyright © 2012 John Wiley & Sons, Ltd.)
- Published
- 2012
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25. Age-related thoracic radiographic changes in golden and labrador retriever muscular dystrophy.
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Bedu AS, Labruyère JJ, Thibaud JL, Barthélémy I, Leperlier D, Saunders JH, and Blot S
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- Aging, Animals, Bronchopneumonia diagnostic imaging, Bronchopneumonia etiology, Bronchopneumonia veterinary, Diaphragm diagnostic imaging, Dogs, Esophagus diagnostic imaging, Funnel Chest diagnostic imaging, Funnel Chest etiology, Funnel Chest veterinary, Lung diagnostic imaging, Dog Diseases diagnostic imaging, Muscular Dystrophy, Animal diagnostic imaging, Radiography, Thoracic veterinary
- Abstract
Golden retriever and Labrador retriever muscular dystrophy are inherited progressive degenerative myopathies that are used as models of Duchenne muscular dystrophy in man. Thoracic lesions were reported to be the most consistent radiographic finding in golden retriever dogs in a study where radiographs were performed at a single-time point. Muscular dystrophy worsens clinically over time and longitudinal studies in dogs are lacking. Thus our goal was to describe the thoracic abnormalities of golden retriever and Labrador retriever dogs, to determine the timing of first expression and their evolution with time. To this purpose, we retrospectively reviewed 390 monthly radiographic studies of 38 golden retrievers and six Labrador retrievers with muscular dystrophy. The same thoracic lesions were found in both golden and Labrador retrievers. They included, in decreasing frequency, flattened and/or scalloped diaphragmatic shape (43/44), pulmonary hyperinflation (34/44), hiatal hernia (34/44), cranial pectus excavatum (23/44), bronchopneumonia (22/44), and megaesophagus (14/44). The last three lesions were not reported in a previous radiographic study in golden retriever dogs. In all but two dogs the thoracic changes were detected between 4 and 10 months and were persistent or worsened over time. Clinically, muscular dystrophy should be included in the differential diagnosis of dogs with a combination of these thoracic radiographic findings., (© 2012 Veterinary Radiology & Ultrasound.)
- Published
- 2012
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26. Bradykinin restores left ventricular function, sarcomeric protein phosphorylation, and e/nNOS levels in dogs with Duchenne muscular dystrophy cardiomyopathy.
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Su JB, Cazorla O, Blot S, Blanchard-Gutton N, Ait Mou Y, Barthélémy I, Sambin L, Sampedrano CC, Gouni V, Unterfinger Y, Aguilar P, Thibaud JL, Bizé A, Pouchelon JL, Dabiré H, Ghaleh B, Berdeaux A, Chetboul V, Lacampagne A, and Hittinger L
- Subjects
- Animals, Dogs, Myocardial Contraction drug effects, Nitric Oxide Synthase Type I analysis, Nitric Oxide Synthase Type III analysis, Phosphorylation, Bradykinin pharmacology, Muscular Dystrophy, Duchenne physiopathology, Nitric Oxide Synthase Type I physiology, Nitric Oxide Synthase Type III physiology, Proteins metabolism, Sarcomeres metabolism, Ventricular Function, Left drug effects
- Abstract
Aims: Cardiomyopathy is a lethal result of Duchenne muscular dystrophy (DMD), but its characteristics remain elusive. The golden retriever muscular dystrophy (GRMD) dogs produce DMD pathology and mirror DMD patient's symptoms, including cardiomyopathy. We previously showed that bradykinin slows the development of pacing-induced heart failure. Therefore, the goals of this research were to characterize dystrophin-deficiency cardiomyopathy and to examine cardiac effects of bradykinin in GRMD dogs., Methods and Results: At baseline, adult GRMD dogs had reduced fractional shortening (28 ± 2 vs. 38 ± 2% in control dogs, P < 0.001) and left ventricular (LV) subendocardial dysfunction leading to impaired endo-epicardial gradient of radial systolic velocity (1.3 ± 0.1 vs. 3.8 ± 0.2 cm/s in control dogs, P < 0.001) measured by echocardiography. These changes were normalized by bradykinin infusion (1 µg/min, 4 weeks). In isolated permeabilized LV subendocardial cells of GRMD dogs, tension-calcium relationships were shifted downward and force-generating capacity and transmural gradient of myofilament length-dependent activation were impaired compared with control dogs. Concomitantly, phosphorylation of sarcomeric regulatory proteins and levels of endothelial and neuronal nitric oxide synthase (e/nNOS) in LV myocardium were significantly altered in GRMD dogs. All these abnormalities were normalized in bradykinin-treated GRMD dogs., Conclusions: Cardiomyopathy in GRMD dogs is characterized by profound LV subendocardial dysfunction, abnormal sarcomeric protein phosphorylation, and impaired e/nNOS, which can be normalized by bradykinin treatment. These data provide new insights into the pathophysiological mechanisms accounting for DMD cardiomyopathy and open new therapeutic perspectives.
- Published
- 2012
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27. Effects of an immunosuppressive treatment in the GRMD dog model of Duchenne muscular dystrophy.
- Author
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Barthélémy I, Uriarte A, Drougard C, Unterfinger Y, Thibaud JL, and Blot S
- Subjects
- Accelerometry, Animals, Biomechanical Phenomena drug effects, Creatine Kinase blood, Cyclosporine pharmacology, Cyclosporine therapeutic use, Disease Models, Animal, Dogs, Follow-Up Studies, Gait drug effects, Humans, Immunosuppressive Agents pharmacology, Motor Activity drug effects, Muscular Dystrophy, Animal blood, Muscular Dystrophy, Animal complications, Muscular Dystrophy, Animal physiopathology, Muscular Dystrophy, Duchenne blood, Muscular Dystrophy, Duchenne complications, Muscular Dystrophy, Duchenne physiopathology, Principal Component Analysis, Tetany blood, Tetany complications, Tetany physiopathology, Immunosuppressive Agents therapeutic use, Muscular Dystrophy, Animal drug therapy, Muscular Dystrophy, Duchenne drug therapy
- Abstract
The GRMD (Golden retriever muscular dystrophy) dog has been widely used in pre-clinical trials targeting DMD (Duchenne muscular dystrophy), using in many cases a concurrent immune-suppressive treatment. The aim of this study is to assess if such a treatment could have an effect on the disease course of these animals. Seven GRMD dogs were treated with an association of cyclosporine A (immunosuppressive dosage) and prednisolone (2 mg/kg/d) during 7 months, from 2 to 9 months of age. A multi-parametric evaluation was performed during this period which allowed us to demonstrate that this treatment had several significant effects on the disease progression. The gait quality as assessed by 3D-accelerometry was dramatically improved. This was consistent with the evolution of other parameters towards a significant improvement, such as the clinical motor score, the post-tetanic relaxation and the serum CK levels. In contrast the isometric force measurement as well as the histological evaluation argued in favor of a more severe disease progression. In view of the disease modifying effects which have been observed in this study it should be concluded that immunosuppressive treatments should be used with caution when carrying out pre-clinical studies in this canine model of DMD. They also highlight the importance of using a large range of multi-parametric evaluation tools to reliably draw any conclusion from trials involving dystrophin-deficient dogs, which reproduce the complexity of the human disease.
- Published
- 2012
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28. Centronuclear myopathy in Labrador retrievers: a recent founder mutation in the PTPLA gene has rapidly disseminated worldwide.
- Author
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Maurer M, Mary J, Guillaud L, Fender M, Pelé M, Bilzer T, Olby N, Penderis J, Shelton GD, Panthier JJ, Thibaud JL, Barthélémy I, Aubin-Houzelstein G, Blot S, Hitte C, and Tiret L
- Subjects
- Alleles, Animals, Dogs, Genes, Recessive, Myopathies, Structural, Congenital genetics, Phenotype, Dog Diseases genetics, Founder Effect, Mutation, Myopathies, Structural, Congenital veterinary, Protein Tyrosine Phosphatases genetics
- Abstract
Centronuclear myopathies (CNM) are inherited congenital disorders characterized by an excessive number of internalized nuclei. In humans, CNM results from ~70 mutations in three major genes from the myotubularin, dynamin and amphiphysin families. Analysis of animal models with altered expression of these genes revealed common defects in all forms of CNM, paving the way for unified pathogenic and therapeutic mechanisms. Despite these efforts, some CNM cases remain genetically unresolved. We previously identified an autosomal recessive form of CNM in French Labrador retrievers from an experimental pedigree, and showed that a loss-of-function mutation in the protein tyrosine phosphatase-like A (PTPLA) gene segregated with CNM. Around the world, client-owned Labrador retrievers with a similar clinical presentation and histopathological changes in muscle biopsies have been described. We hypothesized that these Labradors share the same PTPLA(cnm) mutation. Genotyping of an international panel of 7,426 Labradors led to the identification of PTPLA(cnm) carriers in 13 countries. Haplotype analysis demonstrated that the PTPLA(cnm) allele resulted from a single and recent mutational event that may have rapidly disseminated through the extensive use of popular sires. PTPLA-deficient Labradors will help define the integrated role of PTPLA in the existing CNM gene network. They will be valuable complementary large animal models to test innovative therapies in CNM.
- Published
- 2012
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29. Vascular endothelial dysfunction in Duchenne muscular dystrophy is restored by bradykinin through upregulation of eNOS and nNOS.
- Author
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Dabiré H, Barthélémy I, Blanchard-Gutton N, Sambin L, Sampedrano CC, Gouni V, Unterfinger Y, Aguilar P, Thibaud JL, Ghaleh B, Bizé A, Pouchelon JL, Blot S, Berdeaux A, Hittinger L, Chetboul V, and Su JB
- Subjects
- Animals, Blood Pressure, Carotid Arteries enzymology, Cyclic GMP metabolism, Disease Models, Animal, Dogs, Muscular Dystrophy, Duchenne enzymology, Nitric Oxide physiology, Ventricular Dysfunction, Left, Bradykinin physiology, Endothelium, Vascular physiopathology, Muscular Dystrophy, Duchenne physiopathology, Nitric Oxide Synthase Type I metabolism, Nitric Oxide Synthase Type III metabolism
- Abstract
Little is known about the vascular function and expression of endothelial and neuronal nitric oxide synthases (eNOS and nNOS) in Duchenne muscular dystrophy (DMD). Bradykinin is involved in the regulation of eNOS expression induced by angiotensin-converting enzyme inhibitors. We characterized the vascular function and eNOS and nNOS expression in a canine model of DMD and evaluated the effects of chronic bradykinin treatment. Vascular function was examined in conscious golden retriever muscular dystrophy (GRMD) dogs with left ventricular dysfunction (measured by echocardiography) and in isolated coronary arteries. eNOS and nNOS proteins in carotid arteries were measured by western blot and cyclic guanosine monophosphate (cGMP) content was analyzed by radioimmunoassay. Compared with controls, GRMD dogs had an impaired vasodilator response to acetylcholine. In isolated coronary artery, acetylcholine-elicited relaxation was nearly absent in placebo-treated GRMD dogs. This was explained by reduced nNOS and eNOS proteins and cGMP content in arterial tissues. Chronic bradykinin infusion (1 μg/min, 4 weeks) restored in vivo and in vitro vascular response to acetylcholine to the level of control dogs. This effect was NO-mediated through upregulation of eNOS and nNOS expression. In conclusion, this study is the first to demonstrate that DMD is associated with NO-mediated vascular endothelial dysfunction linked to an altered expression of eNOS and nNOS, which can be overcome by bradykinin.
- Published
- 2012
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30. Surgical treatment and radiation therapy of frontal lobe meningiomas in 7 dogs.
- Author
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Uriarte A, Moissonnier P, Thibaud JL, Reyes-Gomez E, Devauchelle P, and Blot S
- Subjects
- Animals, Dogs, Female, Male, Meningeal Neoplasms radiotherapy, Meningeal Neoplasms surgery, Meningioma radiotherapy, Meningioma surgery, Postoperative Complications veterinary, Prognosis, Seizures etiology, Seizures radiotherapy, Seizures surgery, Seizures veterinary, Survival Analysis, Treatment Outcome, Dog Diseases radiotherapy, Dog Diseases surgery, Frontal Lobe radiation effects, Frontal Lobe surgery, Meningeal Neoplasms veterinary, Meningioma veterinary
- Abstract
The cases of 7 adult dogs with generalized seizures managed by surgical excision and radiation therapy for frontal lobe meningiomas were reviewed. The neurological examination was unremarkable in 6 of the 7 dogs. Five dogs were operated on using a bilateral transfrontal sinus approach and 2 using a unilateral sinotemporal approach to the frontal lobe. One dog was euthanized 14 d after surgery; radiation therapy was initiated 3 wk after surgery in the remaining 6 dogs. Long-term follow-up consisted of neurological examination and magnetic resonance imaging (MRI) and/or computed tomography (CT) scan after radiation therapy. The mean survival time for dogs that had surgery and radiation therapy was 18 mo after surgery. Frontal lobe meningiomas have been associated with poor prognosis. However, the surgical approaches used in these cases, combined with radiation therapy, allow a survival rate for frontal lobe meningiomas similar to that for meningiomas located over the cerebral convexities.
- Published
- 2011
31. Longitudinal ambulatory measurements of gait abnormality in dystrophin-deficient dogs.
- Author
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Barthélémy I, Barrey E, Aguilar P, Uriarte A, Le Chevoir M, Thibaud JL, Voit T, Blot S, and Hogrel JY
- Subjects
- Age Factors, Aging, Analysis of Variance, Animals, Disease Progression, Dog Diseases genetics, Dog Diseases metabolism, Dogs, Dystrophin genetics, Lameness, Animal genetics, Lameness, Animal metabolism, Longitudinal Studies, Male, Muscular Dystrophy, Animal genetics, Muscular Dystrophy, Animal metabolism, Principal Component Analysis, Actigraphy instrumentation, Dog Diseases physiopathology, Dystrophin deficiency, Gait genetics, Lameness, Animal physiopathology, Muscular Dystrophy, Animal physiopathology
- Abstract
Background: This study aimed to measure the gait abnormalities in GRMD (Golden retriever muscular dystrophy) dogs during growth and disease progression using an ambulatory gait analyzer (3D-accelerometers) as a possible tool to assess the effects of a therapeutic intervention., Methods: Six healthy and twelve GRMD dogs were evaluated twice monthly, from the age of two to nine months. The evolution of each gait variable previously shown to be modified in control and dystrophin-deficient adults was assessed using two-ways variance analysis (age, clinical status) with repeated measurements. A principal component analysis (PCA) was applied to perfect multivariate data interpretation., Results: Speed, stride length, total power and force significantly already decreased (p<0.01) at the age of 2 months. The other gait variables (stride frequency, relative power distributions along the three axes) became modified at later stages. Using the PCA analysis, a global gait index taking into account the main gait variables was calculated, and was also consistent to detect the early changes in the GRMD gait patterns, as well as the progressive degradation of gait quality., Conclusion: The gait variables measured by the accelerometers were sensitive to early detect and follow the gait disorders and mirrored the heterogeneity of clinical presentations, giving sense to monitor gait in GRMD dogs during progression of the disease and pre-clinical therapeutic trials.
- Published
- 2011
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32. Minimally invasive video-assisted cervical ventral slot in dogs. A cadaveric study and report of 10 clinical cases.
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Leperlier D, Manassero M, Blot S, Thibaud JL, and Viateau V
- Subjects
- Animals, Cadaver, Dogs, Intervertebral Disc Displacement surgery, Orthopedic Procedures instrumentation, Orthopedic Procedures methods, Cervical Vertebrae pathology, Dog Diseases surgery, Intervertebral Disc Displacement veterinary, Orthopedic Procedures veterinary, Video-Assisted Surgery veterinary
- Abstract
Objectives: To investigate the feasibility of a minimally invasive video-assisted (MIVA) cervical ventral slot (VS) in dogs without the use of fluoroscopy, and to report our initial clinical experiences in dogs., Methods: Two surgical approaches to an intervertebral disk space (IVDS) were performed in eight intact canine cadavers to determine the feasibility of MIVA-VS using the Destandau Endospine™ Devicea (DED) without fluoroscopic guidance. In a subsequent clinical study, 10 client-owned dogs admitted for a Hansen type 1 disk extrusion underwent a MIVA-VS. Recorded data in both studies included: incision lengths, correct targeting of the IVDS, technical problems encountered during the procedure, and potential damage to major anatomical structures. In the 10 clinical cases, duration of the procedure and clinical outcome at five and 12 days, and after a minimum of three months were also recorded., Results: Correct exposure of the targeted IVDS was achieved in all cases. There was no major iatrogenic damage. Mean skin incision length was 39 mm and mean surgery time was 52 minutes. The technique provided increased illumination and magnification of the surgical field. Recovery was uneventful in all cases., Clinical Relevance: The present study provided evidence that MIVA-VS using the DED was feasible and a relatively fast and safe procedure for the treatment of cervical disk herniation. Advantages of the technique seemed to include shorter incisions, less dissection and improved visibility.
- Published
- 2011
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33. Botulism in 2 urban dogs.
- Author
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Uriarte A, Thibaud JL, and Blot S
- Subjects
- Action Potentials, Animals, Botulism diagnosis, Dogs, Evoked Potentials, Female, Neural Conduction physiology, Botulism veterinary, Dog Diseases diagnosis, Electromyography veterinary
- Abstract
Two dogs from the same owner were referred for ascending weakness and paresis of 2 to 3 days duration. Electromyography and electroneurography determined that there were normal F-waves, decreased compound action potential, and decreased activity on repetitive nerve stimulation. These findings were valuable in diagnosing botulism in the dogs.
- Published
- 2010
34. A canine Arylsulfatase G (ARSG) mutation leading to a sulfatase deficiency is associated with neuronal ceroid lipofuscinosis.
- Author
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Abitbol M, Thibaud JL, Olby NJ, Hitte C, Puech JP, Maurer M, Pilot-Storck F, Hédan B, Dréano S, Brahimi S, Delattre D, André C, Gray F, Delisle F, Caillaud C, Bernex F, Panthier JJ, Aubin-Houzelstein G, Blot S, and Tiret L
- Subjects
- ATP-Binding Cassette Transporters genetics, Age Factors, Animals, Arylsulfatases deficiency, Catalytic Domain genetics, Cell Line, Cerebellar Cortex metabolism, Cerebellar Cortex pathology, Cerebellar Cortex ultrastructure, Chromosome Mapping, Chromosomes, Mammalian genetics, Dog Diseases enzymology, Dogs, Female, Gene Expression Profiling, Gene Frequency, Genotype, Haplotypes, Humans, Male, Microscopy, Electron, Transmission, Molecular Sequence Data, Pedigree, Polymorphism, Single Nucleotide, Reverse Transcriptase Polymerase Chain Reaction, Arylsulfatases genetics, Dog Diseases genetics, Mutation, Missense, Neuronal Ceroid-Lipofuscinoses veterinary
- Abstract
Neuronal ceroid lipofuscinoses (NCLs) represent the most common group of inherited progressive encephalopathies in children. They are characterized by progressive loss of vision, mental and motor deterioration, epileptic seizures, and premature death. Rare adult forms of NCL with late onset are known as Kufs' disease. Loci underlying these adult forms remain unknown due to the small number of patients and genetic heterogeneity. Here we confirm that a late-onset form of NCL recessively segregates in US and French pedigrees of American Staffordshire Terrier (AST) dogs. Through combined association, linkage, and haplotype analyses, we mapped the disease locus to a single region of canine chromosome 9. We eventually identified a worldwide breed-specific variant in exon 2 of the Arylsulfatase G (ARSG) gene, which causes a p.R99H substitution in the vicinity of the catalytic domain of the enzyme. In transfected cells or leukocytes from affected dogs, the missense change leads to a 75% decrease in sulfatase activity, providing a functional confirmation that the variant might be the NCL-causing mutation. Our results uncover a protein involved in neuronal homeostasis, identify a family of candidate genes to be screened in patients with Kufs' disease, and suggest that a deficiency in sulfatase is part of the NCL pathogenesis.
- Published
- 2010
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35. Chronic traumatic brain injury in a dog.
- Author
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Laurent S, Thibaud JL, Hordeaux J, Reyes-Gomez E, Delisle F, Blot S, and Colle MA
- Subjects
- Animals, Atrophy pathology, Atrophy veterinary, Brain Injury, Chronic pathology, Dogs, Female, Necrosis pathology, Necrosis veterinary, Brain pathology, Brain Injury, Chronic veterinary, Dog Diseases pathology
- Abstract
Chronic traumatic brain injury is rare in man and has not been previously documented in dogs. This report describes a 2-year-old female American Staffordshire bull terrier that was referred with forelimb and hindlimb ataxia, decreased vigilance and disorientation following repeated aggression and physical abuse by its owner. A diffuse cortical lesion was suspected. Cerebrospinal fluid analysis revealed neutrophilic pleocytosis and computed tomography showed marked widening of the cerebral sulci with mild bilateral ventriculomegaly. The dog was humanely destroyed in view of the poor prognosis. Necropsy examination revealed narrowing of the cerebral cortical gyri and consequent widening of the sulci without distortion or displacement of the neural parenchyma. These features were consistent with bilateral diffuse cortical atrophy. Microscopically, there were chronic subarachnoid haemorrhages and the cortical subpial layer displayed spongiosis, capillary hyperplasia, astrocytosis, microgliosis and frequent neuronal necrosis occurring in a characteristic laminar pattern. This histopathological pattern of damage was significantly different from that previously described in people suffering from repeated traumatic brain injuries over a long period of time., ((c) 2009 Elsevier Ltd. All rights reserved.)
- Published
- 2010
- Full Text
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36. Gait analysis using accelerometry in dystrophin-deficient dogs.
- Author
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Barthélémy I, Barrey E, Thibaud JL, Uriarte A, Voit T, Blot S, and Hogrel JY
- Subjects
- Analysis of Variance, Animals, Dog Diseases metabolism, Dogs, Principal Component Analysis methods, Reproducibility of Results, Dog Diseases physiopathology, Dystrophin deficiency, Gait physiology, Muscular Dystrophy, Animal metabolism, Muscular Dystrophy, Animal physiopathology
- Abstract
Dogs affected with Golden Retriever Muscular Dystrophy (GRMD) exhibit striking clinical similarities with patients suffering from Duchenne muscular dystrophy (DMD), particularly gait impairments. The purpose of this study was to describe the use and reliability of accelerometry in gait assessment of dogs with muscular dystrophy. Eight healthy and 11 GRMD adult dogs underwent three gait assessment sessions, using accelerometry. Three-axial recordings of accelerations were performed, and gait variables calculated. Total power, force and regularity of accelerations, stride length and speed, normalized by height at withers, stride frequency, and cranio-caudal power were significantly decreased, whereas medio-lateral power was significantly increased in GRMD dogs. Moreover, these variables were repeatable within and between sessions. Accelerometry provides reliable variables which highlight specific gait patterns of GRMD dogs, describing objectively and quantitatively their slow, short-stepped, and swaying gait. As it is easy to set-up, quick to perform and inexpensive, accelerometry represents a useful tool, to assess locomotion during pre-clinical trials.
- Published
- 2009
- Full Text
- View/download PDF
37. Spinal cryptococcoma in an immunocompetent cat.
- Author
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Belluco S, Thibaud JL, Guillot J, Krockenberger MB, Wyers M, Blot S, and Colle MA
- Subjects
- Animals, Cat Diseases physiopathology, Cats, Cryptococcosis physiopathology, Cryptococcus neoformans, Immunohistochemistry, Male, Spinal Cord Diseases pathology, Thoracic Vertebrae, Cat Diseases microbiology, Cat Diseases pathology, Cryptococcosis pathology, Cryptococcosis veterinary, Spinal Cord Diseases microbiology, Spinal Cord Diseases veterinary
- Abstract
This report describes an unusual case of primary cryptococcoma in the proximal thoracic spinal cord of an 11-year-old immunocompetent cat from a farm on which there were large numbers of pigeons. This animal was referred for examination with progressive paralysis and shown to be free from feline immunodeficiency virus, feline leukaemia virus, feline coronavirus and Toxoplasma gondii. It died 2 months later. At necropsy, the only lesion detected was a malacic area, 4cm in length, in the spinal cord. Histopathological examination of the spinal cord revealed severe granulomatous inflammation associated with large numbers of encapsulated yeast cells. In addition to the granulomatous host response, necrosis, digestion chambers, Gitter cells, spheroids and lymphocytic perivascular cuffs were features of the malacic areas. Immunohistochemistry confirmed the presence of Cryptococcus neoformans var. grubii yeast cells.
- Published
- 2008
- Full Text
- View/download PDF
38. Progressive myelopathy due to a spontaneous intramedullary hematoma in a dog: pre- and postoperative clinical and magnetic resonance imaging follow-up.
- Author
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Thibaud JL, Hidalgo A, Benchekroun G, Fanchon L, Crespeau F, Delisle F, and Blot S
- Subjects
- Animals, Dogs, Follow-Up Studies, Hematoma complications, Hematoma diagnosis, Hematoma surgery, Magnetic Resonance Imaging methods, Male, Spinal Cord Diseases diagnosis, Spinal Cord Diseases etiology, Spinal Cord Diseases surgery, Treatment Outcome, Dog Diseases diagnosis, Dog Diseases surgery, Hematoma veterinary, Magnetic Resonance Imaging veterinary, Spinal Cord Diseases veterinary
- Abstract
A 4-year-old, male Jack Russell terrier was presented for a 6-month history of progressive right hemiparesis with episodic cervical hyperesthesia. The neurological examination showed a right-sided, upper motoneuron syndrome and partial Horner's syndrome. Two magnetic resonance imaging (MRI) examinations were performed 3 months apart and revealed a persistent cervical intramedullary hematoma. A dorsal myelotomy was performed. A subacute hematoma was confirmed histologically without underlying lesions. Eighteen months later, the dog's clinical signs were minimal. Two MRI examinations were performed 2 weeks and 5 months after surgery and revealed regressing signal abnormalities at the surgical site, consistent with a surgical scar.
- Published
- 2008
- Full Text
- View/download PDF
39. Successful treatment of cervical spinal epidural empyema secondary to grass awn migration in a cat.
- Author
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Granger N, Hidalgo A, Leperlier D, Gnirs K, Thibaud JL, Delisle F, and Blot S
- Subjects
- Animals, Cat Diseases diagnostic imaging, Cat Diseases pathology, Cats, Diagnosis, Differential, Empyema etiology, Epidural Abscess etiology, Female, Foreign-Body Migration complications, Foreign-Body Migration diagnosis, Foreign-Body Migration therapy, Pasteurella Infections etiology, Pasteurella multocida isolation & purification, Tomography, X-Ray Computed veterinary, Cat Diseases diagnosis, Cat Diseases surgery, Cervical Vertebrae pathology, Empyema veterinary, Epidural Abscess veterinary, Foreign-Body Migration veterinary, Pasteurella Infections veterinary
- Abstract
Spinal epidural empyema (SEE) represents a severe pyogenic infection of the epidural space. Clinical signs of the disease are non-specific--increased body temperature, intense neck pain, neurological signs of a transverse myelopathy--and can lead to severe and permanent neurological deficits. This report describes the diagnosis and successful surgical treatment of cervical SEE secondary to grass awn migration in a cat. Although it is uncommon, this disease should be suspected in cats with progressive myelopathy. Early diagnosis and emergency surgery combined with antibiotic therapy are required to allow a complete recovery.
- Published
- 2007
- Full Text
- View/download PDF
40. Characterization of dystrophic muscle in golden retriever muscular dystrophy dogs by nuclear magnetic resonance imaging.
- Author
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Thibaud JL, Monnet A, Bertoldi D, Barthélémy I, Blot S, and Carlier PG
- Subjects
- Animals, Disease Models, Animal, Dogs, Male, Time Factors, Magnetic Resonance Spectroscopy, Muscle, Skeletal pathology, Muscular Dystrophy, Animal diagnosis
- Abstract
The Golden Retriever Muscular Dystrophy dog lacks dystrophin. Disease progression in this model shares many similarities with the Duchenne muscular dystrophy, both from anatomico pathological and clinical standpoints. The model is increasingly used in pre-clinical trials but needs to be further investigated, particularly with reference to the evaluation of therapies. The aim of this study was to identify quantitative indices that would help characterize the dystrophic dog non-invasively using NMR imaging. Two-month-old dystrophic dogs and healthy control animals were scanned at 4T. Standard T2- and T1-weighted images, fat-saturated T1-weighted images pre- and post-gadolinium chelate injection were acquired and kinetics of muscle enhancement were studied over a 2-h period. Several indices were found to be abnormally high in dystrophic dogs: the T2-weighted/T1-weighted signal ratio, T2-weighted image heterogeneity and maximal signal enhancement post-gadolinium. These may be proposed to evaluate muscle structural alterations non-invasively in this disease.
- Published
- 2007
- Full Text
- View/download PDF
41. Mesoangioblast stem cells ameliorate muscle function in dystrophic dogs.
- Author
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Sampaolesi M, Blot S, D'Antona G, Granger N, Tonlorenzi R, Innocenzi A, Mognol P, Thibaud JL, Galvez BG, Barthélémy I, Perani L, Mantero S, Guttinger M, Pansarasa O, Rinaldi C, Cusella De Angelis MG, Torrente Y, Bordignon C, Bottinelli R, and Cossu G
- Subjects
- Adult Stem Cells immunology, Animals, Combined Modality Therapy, Creatine Kinase blood, Dogs, Dystrophin biosynthesis, Dystrophin genetics, Dystrophin immunology, Genetic Therapy, Humans, Male, Muscle Cells, Recombinant Fusion Proteins biosynthesis, Recombinant Fusion Proteins genetics, Transplantation, Autologous, Transplantation, Heterologous, Adult Stem Cells transplantation, Muscular Dystrophy, Animal therapy, Muscular Dystrophy, Duchenne therapy, Stem Cell Transplantation
- Abstract
Duchenne muscular dystrophy remains an untreatable genetic disease that severely limits motility and life expectancy in affected children. The only animal model specifically reproducing the alterations in the dystrophin gene and the full spectrum of human pathology is the golden retriever dog model. Affected animals present a single mutation in intron 6, resulting in complete absence of the dystrophin protein, and early and severe muscle degeneration with nearly complete loss of motility and walking ability. Death usually occurs at about 1 year of age as a result of failure of respiratory muscles. Here we report that intra-arterial delivery of wild-type canine mesoangioblasts (vessel-associated stem cells) results in an extensive recovery of dystrophin expression, normal muscle morphology and function (confirmed by measurement of contraction force on single fibres). The outcome is a remarkable clinical amelioration and preservation of active motility. These data qualify mesoangioblasts as candidates for future stem cell therapy for Duchenne patients.
- Published
- 2006
- Full Text
- View/download PDF
42. Tissue Doppler imaging for detection of radial and longitudinal myocardial dysfunction in a family of cats affected by dystrophin-deficient hypertrophic muscular dystrophy.
- Author
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Chetboul V, Blot S, Sampedrano CC, Thibaud JL, Granger N, Tissier R, Bruneval P, Gaschen F, Gouni V, Nicolle AP, and Pouchelon JL
- Subjects
- Animals, Cardiomyopathies complications, Cardiomyopathies diagnosis, Cat Diseases diagnostic imaging, Cat Diseases physiopathology, Cats, Echocardiography, Doppler, Color veterinary, Female, Male, Muscular Dystrophy, Animal complications, Muscular Dystrophy, Animal diagnostic imaging, Muscular Dystrophy, Animal physiopathology, Predictive Value of Tests, Ventricular Dysfunction, Left veterinary, Cardiomyopathies veterinary, Cat Diseases diagnosis, Dystrophin deficiency, Muscular Dystrophy, Animal diagnosis
- Abstract
Diagnosis of feline hypertrophic cardiomyopathy currently is based on the presence of myocardial hypertrophy detected using conventional echocardiography. The accuracy of tissue Doppler imaging (TDI) for earlier detection of the disease has never been described. The objective of this sudy was to quantify left ventricular free wall (LVFW) velocities in cats with hypertrophic muscular dystrophy (HFMD) during preclinical cardiomyopathy using TDI. The study animals included 22 healthy controls and 7 cats belonging to a family of cats with HFMD (2 affected adult males, 2 heterozygous adult females, one 2.5-month-old affected male kitten, and 2 phenotypically normal female kittens from the same litter). All cats were examined via conventional echocardiography and 2-dimensional color TDI. No LVFW hypertrophy was detected in the 2 carriers or in the affected kitten when using conventional echocardiography and histologic examination, respectively. The LVFW also was normal for 1 affected male and at the upper limit of normal for the 2nd male. Conversely, LVFW dysfunction was detected in all affected and carrier cats with HFMD when using TDI. TDI consistently detects LVFW dysfunction in cats with HFMD despite the absence of myocardial hypertrophy. Therefore, TDI appears more sensitive than conventional echocardiography in detecting regional myocardial abnormalities.
- Published
- 2006
- Full Text
- View/download PDF
43. First isolation of Mycobacterium microti (Llama-type) from a dog.
- Author
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Deforges L, Boulouis HJ, Thibaud JL, Boulouha L, Sougakoff W, Blot S, Hewinson G, Truffot-Pernot C, and Haddad N
- Subjects
- Acute Disease, Animals, Biopsy, Fine-Needle veterinary, Dog Diseases transmission, Dogs, Fatal Outcome, Humans, Male, Mycobacterium classification, Peritonitis microbiology, Zoonoses, Dog Diseases microbiology, Mycobacterium isolation & purification, Peritonitis veterinary
- Abstract
We report the first isolation of Mycobacterium microti from a dog with lesions of acute peritonitis. The isolate was demonstrated to be M. microti of Llama-Type by spoligotyping. Epidemiological implications of the isolation of this possibly zoonotic agent from a dog are discussed.
- Published
- 2004
- Full Text
- View/download PDF
44. Tissue Doppler assessment of diastolic and systolic alterations of radial and longitudinal left ventricular motions in Golden Retrievers during the preclinical phase of cardiomyopathy associated with muscular dystrophy.
- Author
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Chetboul V, Carlos C, Blot S, Thibaud JL, Escriou C, Tissier R, Retortillo JL, and Pouchelon JL
- Subjects
- Analysis of Variance, Animals, Cardiomyopathies complications, Cardiomyopathies diagnosis, Dogs, Echocardiography veterinary, Ultrasonography, Doppler, Duplex methods, Cardiomyopathies veterinary, Dog Diseases diagnosis, Muscular Dystrophy, Animal complications, Myocardial Contraction physiology, Ultrasonography, Doppler, Duplex veterinary, Ventricular Function, Left physiology
- Abstract
Objective: To quantify radial and longitudinal left ventricular free wall (LVFW) velocities in dogs during the preclinical phase of Golden Retriever muscular dystrophy (GRMD)-associated cardiomyopathy by use of tissue Doppler imaging (TDI)., Animals: 9 dogs with GRMD and 6 healthy control dogs., Procedure: All dogs (< 3 years old) were examined via conventional echocardiography and 2-dimensional color TDI. Myocardial velocities in the LVFW were recorded from right parasternal ventricular short-axis (radial motion) and left apical 4-chamber (longitudinal motion) views. Cardiac assessments via TDI included maximal systolic and early and late diastolic LVFW velocities in the endocardial and epicardial layers (for radial motion) and in the basal and apical segments (for longitudinal motion) (for longitudinal motion),, Results: -No notable ventricular dilatation or alteration of inotropism was detected in dogs with GRMD via conventional echocardiography. Compared with healthy dogs, endocardial velocities were significantly decreased in dogs with GRMD, resulting in marked decreases in radial myocardial velocity gradients during systole and early and late diastole. Similarly, basal and apical velocities were significantly decreased in systole and the former also in early diastole, resulting in significant decreases in the 2 corresponding longitudinal myocardial velocity gradients. The radial epicardial and longitudinal late diastolic velocities were comparable in the 2 groups., Conclusion and Clinical Relevance: Results indicated that GRMD-associated cardiomyopathy in dogs is associated with early marked dysfunction of both radial and longitudinal LVFW motions. These combined regional myocardial abnormalities might be useful criteria for detection of dilated cardiomyopathy at the preclinical stage of the disease in dogs.
- Published
- 2004
- Full Text
- View/download PDF
45. Cerebellar cortical degeneration in adult American Staffordshire Terriers.
- Author
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Olby N, Blot S, Thibaud JL, Phillips J, O'Brien DP, Burr J, Berg J, Brown T, and Breen M
- Subjects
- Animals, Cerebellar Ataxia epidemiology, Cerebellar Ataxia pathology, Cerebellum pathology, Dog Diseases diagnostic imaging, Dog Diseases etiology, Dog Diseases genetics, Dogs, Europe epidemiology, Female, Male, Pedigree, Purkinje Cells pathology, Radiography, United States epidemiology, Cerebellar Ataxia veterinary, Dog Diseases epidemiology, Dog Diseases pathology
- Abstract
Adult-onset cerebellar cortical degeneration recently has been reported in American Staffordshire Terriers. We describe the clinical and histopathologic features of this disease and examine its mode of inheritance in 63 affected dogs. The age at which neurologic deficits 1st were recognized varied from 18 months to 9 years, with the majority of dogs presented to veterinarians between 4 and 6 years of age. Time from onset of clinical signs to euthanasia varied from 6 months to 6.5 years, with the majority of affected dogs surviving from 2 to 4 years. Initial neurologic findings included stumbling, truncal sway, and ataxia exacerbated by lifting the head up and negotiating stairs. Signs progressed to obvious ataxia characterized by dysmetria, nystagmus, coarse intention tremor, variable loss of menace reaction, marked truncal sway, and falling with transient opisthotonus. With continued progression, dogs became unable to walk without falling repeatedly. Cerebellar atrophy was visible on magnetic resonance images and on gross pathology. Histopathologic findings included marked loss of Purkinje neurons with thinning of the molecular and granular layers and increased cellularity of the cerebellar nuclei. The closest common ancestor of the dogs was born in the 1950s and inheritance was most consistent with an autosomal recessive mode of transmission with a prevalence estimated at 1 in 400 dogs. This inherited disease is comparable to the group of diseases known as spinocerebellar ataxias in humans. Many spinocerebellar ataxias in humans are caused by nucleotide repeats, and this genetic aberration merits investigation as a potential cause of the disease in American Staffordshire Terriers.
- Published
- 2004
- Full Text
- View/download PDF
46. Theileria annulata in CD5(+) macrophages and B1 B cells.
- Author
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Moreau MF, Thibaud JL, Miled LB, Chaussepied M, Baumgartner M, Davis WC, Minoprio P, and Langsley G
- Subjects
- Animals, Cell Line, Flow Cytometry, Humans, Macrophages immunology, Reverse Transcriptase Polymerase Chain Reaction, B-Lymphocyte Subsets parasitology, CD5 Antigens analysis, Macrophages parasitology, Theileria annulata pathogenicity, Theileriasis parasitology
- Abstract
Theileria parasites infect and transform bovine leukocytes. We have analyzed laboratory-established Theileria sp.-infected leukocyte lines and observed that transformed macrophages express CD5. Low-level expression of CD5 by macrophages was further confirmed on three independent Theileria annulata clinical isolates from Tunisia. Interestingly, the fourth CD5(+) clinical isolate (MB2) was morphologically different, expressed surface immunoglobulin M (IgM) and BoLA class II, and had rearranged Ig light-chain genes. To demonstrate that MB2 did indeed contain CD5(+) B cells, individual clonal lines were obtained by limiting dilution, and CD5 expression and Ig gene rearrangement were confirmed. This suggests that in natural infections T. annulata can invade and transform CD5(+) B cells.
- Published
- 1999
- Full Text
- View/download PDF
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