Inherited myopathy of great Danes.

A hereditary, non-inflammatory myopathy occurring in young great Danes with distinctive histological features in muscle biopsy specimens is reviewed. Onset of clinical signs is usually before one year of age and both sexes are affected. Clinical signs are characterised by exercise intolerance, muscle wasting, and an exercise-induced tremor. Although most affected dogs have a severe form of the disease, occasional dogs may have a less pronounced form and survive into adulthood with an acceptable quality of life. Litters containing affected puppies are born to clinically unaffected parents, and an autosomal recessive pattern of inheritance is likely. All recorded cases have had fawn or brindle coat coloration. Elevated serum creatinine kinase concentrations and spontaneous electrical activity in skeletal muscles are frequently found. While originally reported (Targett and others 1994) as a central core myopathy in this breed, the histochemical characteristics of the distinct cytoarchitectural structures differ from those of the well-characterised central core myopathy in human beings. In fact, these structures differ from any known myopathy in human beings and likely represents a unique non-inflammatory myopathy affecting dogs. Until this myopathy is characterised further, the name inherited myopathy in great Danes is suggested.

The effect of injection technique on the passive spread of astrocytes following transplantation into rat spinal cord white matter.

Bisbenzimide-labelled astrocytes were transplanted into the spinal white matter of the rat using three different injection techniques and the variability of the longitudinal distance over which they were found was compared 30 min later. Cells spread up to 5.02 mm and the greatest variability was seen when they were injected as a bolus (54%), compared with 26% when injected over 2 min. These results show the importance of establishing the extent of passive spread of cells and its variability when performing studies in cell migration.

Histological characterization of an ependymoma in the fourth ventricle of a cat.

A tumour occupying the fourth ventricle in a 3-year-old cat was removed surgically and characterized as a tanycytic ependymoma on the basis of histological features of low cellularity, inconspicuous perivascular pseudorosettes and fascicular architecture. Immunohistochemical analysis of sections revealed that the neoplastic cells were immunoreactive for glial fibrillary acidic protein (GFAP), vimentin and S-100. The histological and immunohistochemical findings were similar to those of human tanycytic ependymoma, a subclassification of ependymoma not previously described in domestic species.

Acute intervertebral disc extrusion in a cat: clinical and MRI findings.

A 5 year old, neutered male, domestic shorthaired cat had acute left hemiparesis and Horner's syndrome. Magnetic resonance imaging (MRI) revealed a loss of the normal signal from the nucleus pulposus of the intervertebral disc at C3/4, narrowing of the ventral subarachnoid space and slight dorsal displacement of the spinal cord and a focal hyperintense lesion affecting the left side of the spinal cord at the same level. The presumptive diagnosis was focal spinal cord oedema associated with intervertebral disc extrusion. A traumatic aetiology was suspected. The cat was treated conservatively and improved gradually over a period of 6 months.

Results of magnetic resonance imaging in dogs with vestibular disorders: 85 cases (1996-1999).

OBJECTIVE: To determine results of magnetic resonance (MR) imaging in dogs with vestibular disorders (VD) and correlate results of MR imaging with clinical findings. DESIGN: Retrospective study. ANIMALS: 85 dogs. PROCEDURE: Information on signalment, clinical signs, and presumptive lesion location was obtained from the medical records, and MR images were reviewed. RESULTS: 27 dogs had peripheral VD, 37 had central VD, and 21 had paradoxical VD. Of the 27 dogs with peripheral VD, 11 (41%) had MR imaging abnormalities involving the ipsilateral tympanic bulla compatible with otitis media (6 also had abnormalities involving the petrous portion of the ipsilateral temporal bone compatible with otitis interna), 7 (26%) had MR imaging abnormalities compatible with middle ear neoplasia, 2(7%) had an ipsilateral cerebellopontine angle lesion, and 7 (26%) did not have MR imaging abnormalities. All dogs with central and paradoxical VD had abnormalities evident on MR images. Of the 37 dogs with central VD, 13 (35%) had an extra-axial lesion, 6 (16%) had an intra-axial lesion, and 18 (49%) had multiple intra-axial lesions. In 23 (62%) dogs with central VD, lesions on MR images corresponded with location suspected on the basis of clinical signs. Of the 21 dogs with paradoxical VD, 12 (57%) had an extra-axial lesion, 5 (24%) had an intra-axial lesion, and 4 (19%) had multiple intra-axial lesions. Location of lesions on MR images agreed with location suspected on the basis of clinical signs in 19 (90%) dogs. CONCLUSIONS AND CLINICAL RELEVANCE: Results suggest that MR imaging may be helpful in the diagnosis and treatment of VD in dogs.

Neurological signs and results of magnetic resonance imaging in 40 cavalier King Charles spaniels with Chiari type 1-like malformations.

In human beings a Chiari type 1 malformation is a developmental condition characterised by cerebellar herniation and syringohydromyelia. Abnormalities compatible with such a malformation were identified by magnetic resonance imaging in 39 cavalier King Charles spaniels with neurological signs and in one neurologically normal cavalier King Charles spaniel that was examined postmortem. The dogs with these abnormalities had a wide variety of neurological signs, but there was no apparent correlation between the neurological signs and the severity of cerebellar herniation, syringohydromyelia or hydrocephalus.

Suspected mitochondrial myopathy in a Jack Russell terrier.

A Jack Russell terrier with a history of progressive exercise intolerance was examined at the age of four months and again 10 months later. Clinical examination revealed a stunted, thin dog with a stilted gait. The dog had raised lactate levels before and after feeding and a raised lactate/pyruvate ratio after feeding, indicating a metabolic abnormality. Histochemical evaluation of muscle biopsies revealed subsarcolemmal accumulation of oxidative activity when stained with nicotinamide adenine dinucleotide tetrazollum reductase and ragged red fibres when stained with modified Gomori trichrome; all fibre types were involved. Ultrastructural examination of the muscle confirmed the presence of subsarcolemmal accumulations of mitochondria. Histochemical staining for the activity of enzymes of the Krebs cycle, oxidative phosphorylation and other metabolic cytosolic enzymes failed to demonstrate an abnormality. In view of the clinical picture and the biochemical and histological findings, a tentative diagnosis of mitochondrial myopathy was made. The difficulties associated with diagnosing mitochondrial disorders are discussed.

The use of xenografting to evaluate the remyelinating potential of glial cell cultures.

Experiments in rodents have shown a potential role for glial cell transplantation as a means of influencing repair in the central nervous system of man. A crucial step in developing human therapy is to establish whether knowledge gained from studies in rodents is applicable to larger mammalian species. In order to explore this issue we examined the ability of cat glial cell cultures to remyelinate areas of ethidium-bromide-induced demyelination in the spinal cord of immunosuppressed rats and cats. Transplantation of density-gradient-isolated glial cells obtained from the forebrain of 7-day-old kittens resulted in enhanced oligodendrocyte remyelination in the rat but failed to enhance oligodendrocyte remyelination in the cat. The feasibility of enhancing oligodendrocyte remyelination in the cat lesion was demonstrated by transplanting a rat culture containing a high proportion of cells of the oligodendrocyte lineage. Tissue culture of the density-gradient-isolated cell preparations suggested that the failure of the kitten glial preparation to enhance oligodendrocyte remyelination in the cat was most probably due to its poor oligodendrocyte-generating capacity. However, our lack of understanding of the biology of feline glial cells precludes a full understanding of these experiments.

Magnetic resonance imaging of a medullary dermoid cyst with secondary hydrocephalus in a dog.

Magnetic resonance images were acquired of the brain of a 7-year-old male Golden Retriever with hydrocephalus secondary to a medullary lesion. Images were acquired prior to and 4 weeks following surgical treatment for the hydrocephalus, and the dog was euthanased following the second imaging session. The MR images demonstrated a medullary lesion with patchy but predominantly hyperintense signal with both T1- and T2-weighting, within which small areas of low signal were scattered. There was little edema associated with this lesion and no enhancement with gadolinium. Postmortem examination revealed the medullary mass to be a dermoid cyst. Several small nodular lesions were identified within the central nervous system on the magnetic resonance images whose origin was uncertain on postmortem examination.

Results of myelography in seven dogs with myelomalacia.

Myelomalacia is a hemorrhagic infarction of the spinal cord that can occur as a sequel to acute spinal cord injury. Myelomalacia may be focal or diffuse; the diffuse form is typically associated with cranial migration of neurologic signs ("ascending syndrome") and is often fatal. In a retrospective study of seven affected dogs, diffuse myelomalacia was associated with intervertebral disc extrusion in five dogs, focal myelomalacia was associated with fibrocartilagenous embolus in one dog, and had no apparent cause in one dog. The myelographic signs included a variable degree of contrast medium infiltration into the spinal cord in six dogs (86%) and/or spinal cord swelling in six dogs (86%). In one dog with focal myelomalacia, the only myelographic sign was spinal cord swelling.

Material in the middle ear of dogs having magnetic resonance imaging for investigation of neurologic signs.

The aim of this study was to determine the prevalence and potential significance of finding material in the middle ear of dogs having magnetic resonance (MR) imaging. Of 466 MR studies reviewed, an increased signal was identified in the tympanic bulla in 32 (7%) dogs. Cavalier King Charles spaniels, Cocker spaniels, Bulldogs, and Boxers were over-represented compared to the population of dogs having MR imaging. Five (16%) dogs had definite otitis media and one (3%) had a meningioma invading the middle ear. Of the remaining dogs, 13 (41%) had possible otitis media and 13 (41%) had neurologic conditions apparently unrelated to otitis media. The most common appearance of material in the middle ear was isointense in T1-weighted images and hyperintense in T2-weighted images. There was no apparent correlation between the signal characteristics of the material and the diagnosis. Enhanced signal after gadolinium administration was observed affecting the lining of the bulla in dogs with otitis media and in dogs with unrelated neurologic conditions. In dogs without clinical signs of otitis media, finding an increased signal in the middle ear during MR imaging may reflect subclinical otitis media or fluid accumulation unrelated to inflammation. Brachycephalic dogs may be predisposed to this condition.

Failure to achieve remyelination of demyelinated rat axons following transplantation of glial cells obtained from the adult human brain.

The ability of transplanted glial cells to myelinate axons in experimental animals offers the prospect that it may be possible to achieve remyelination in human demyelinating disease by the implantation of oligodendrocyte lineage cells. Autologous normal white matter could represent a potential source of cells whose use would avoid tissue rejection and overcome ethical and practical constraints associated with the use of fetal tissue. To determine the remyelinating potential of cells isolated from adult human CNS, a cell preparation prepared from adult human white matter which contained 56% oligodendrocytes, 3% preoligodendrocytes and 1% precursor cells was transplanted into non-repairing demyelinating lesions in immunosuppressed rats created by the injection of ethidium bromide into x-irradiated spinal cord white matter. The extent of remyelination was examined 3 and 5 weeks after transplantation. Although the transplanted oligodendrocytes survived in the area of demyelination, associated with demyelinated axons and produced myelin membranes, no myelin sheaths were produced and there was no evidence of cell migration or division. The failure of human oligodendrocytes to form myelin sheaths may reflect the poor remyelinating potential of post mitotic oligodendrocytes, and the failure of the small number of co-transplanted bipotential oligodendrocyte progenitor cells to differentiate and myelinate axons may be a consequence of lack of appropriate environmental factors within the rat lesion required for expansion and differentiation of these cells.