Nemaline rods in canine myopathies: 4 case reports and literature review.

The diagnosis of nemaline rod myopathy (NM) is based on the presence of numerous pathognomonic rods within a fresh frozen muscle biopsy specimen. Three forms of congenital NM have been described in humans, and rods have been found to occur in various other conditions. A similar myopathy was described in 1986 in a family of cats. In this report, we describe a case of congenital NM in a 10-month-old Border Collie, an adult-onset NM in an 11-year-old Schipperke, and 2 acquired myopathies with nemaline rods in adult dogs associated with hypothyroidism and Cushing's syndrome. Common clinical features included exercise intolerance, abnormal electromyography, and the presence of nemaline rods in fresh, frozen, and glutaraldehyde-fixed biopsies from proximal appendicular limb muscles. Staining of cryostat sections of muscle biopsy specimens by the modified Gomori trichrome technique disclosed numerous rod bodies that were localized to type 1 fibers by the histochemical adenosine triphosphatase reaction. Accumulation of rods also was demonstrated by electron microscopy in 2 of the cases with localized enlargement and streaming of Z lines. Documentation of NM in a young Border Collie and the adult-onset form in the Schipperke alerts clinicians to the existence of this disorder in these breeds.

Assessment of dorsal nerve root and spinal cord dorsal horn function in clinically normal dogs by determination of cord dorsum potentials.

OBJECTIVE: To establish normal predictive values for cord dorsum potential (CDP) onset latency after thoracic and pelvic limb sensory or mixed nerve stimulation in adult dogs. ANIMALS: 26 clinically normal adult dogs. PROCEDURE: Sensory nerve action potentials (SNAP) were recorded proximally from tibial and lateral superficial radial nerves after distal stimulation. The CDP were recorded from the L4-L5 interarcuate ligament for the tibial nerve and from the C7-T1 interarcuate ligament for the radial nerve. Linear regression analyses were performed for CDP onset latency, and mean +/- SD was calculated for CDP onset to peak latency differences and sensory nerve conduction velocities (SNCV). RESULTS: For the tibial nerve, expected CDP onset latency (CDPOL) = -1.194 + 0.014 X pelvic limb length (mm; R2 = 0.912); CDPOL = -2.156 + 0.011 X pelvic limb/spinal length (mm; R2 = 0.911); and CDPOL = 0.941 + 2.197 X tibial nerve SNAP latency (milliseconds; R2 = 0.903). For the radial nerve, CDPOL = -0.9 + 0.014 x thoracic limb length (mm; R2 = 0.873); and CDPOL = 1.454 + 1.874 X radial nerve SNAP latency (milliseconds; R2 = 0.903). Mean +/- SD for CDP onset to peak latency difference for tibial and radial nerves was 3.1+/-0.3 and 3.0+/-0.4 milliseconds, respectively. CONCLUSIONS: Strong linear associations exist between CDPOL and a number of easily measured peripheral independent variables in dogs. There is also a narrow range of normal values for CDP onset to peak latency differences that is independent of limb length. CLINICAL RELEVANCE: CDP evaluation can be used to accurately assess functional severity and distribution of abnormalities in proximal sensory nerves, dorsal nerve roots, and spinal cord dorsal horns in dogs with suspected neuropathy, radiculopathy, or myelopathy involving the brachial or lumbosacral intumescences.

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.

GM1-gangliosidosis in a cross-bred dog confirmed by detection of GM1-ganglioside using electrospray ionisation-tandem mass spectrometry.

The post-mortem diagnosis of lysosomal storage diseases can be confounded by the unavailability of suitable material. Here we report the diagnosis of GM1-gangliosidosis in a cross-bred dog, from which only formalin-fixed brain was available, by a combination of electron microscopy and the detection of elevated levels of GM1-ganglioside within the tissue using the novel technique of electrospray ionisation tandem mass spectrometry. Electron microscopic examination of ultrathin sections of resin-embedded tissue revealed cytoplasmic inclusions (membranous cytoplasmic and zebra bodies) in brain stem and cerebellar neurons that were characteristic of a gangliosidosis. Glycolipids were extracted from the fixed tissue and analysed by tandem mass spectrometry. Two major ions were detected, which corresponded to GM1 (d18:1-C18:0) and Gm1 (d20:1-C18:0). Their identity was confirmed by comparison of their fragmentation patterns with those of authentic standards. The concentration of GM1 was approximately sixfold higher on a wet weight basis than in the brain of a normal control dog, confirming the diagnosis of GM1-gangliosidosis.