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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 29 (1974), S. 325-336 
    ISSN: 1432-0533
    Keywords: Comparative Neuropathology ; Hypertrophic Neuropathy ; Relapsing Neuropathy ; Onion Bulbs
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Postmortem examination of a twelve-year-old dog with a history of relapsing paresis revealed widespread nerve enlargement. The hypertrophic nerves contained numerous onion buld formations and areas of mucoid degeneration. The clinical and pathologic features in this case were compared to those reported in inherited and acquired forms of hypertrophic neuropathy in man.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 46 (1979), S. 17-24 
    ISSN: 1432-0533
    Keywords: Comparative neuropathology ; Horse ; Chronic polyradiculoneuritis ; Cauda equina neuritis ; Idiopathic polyradiculity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Four cases of neuritis of the cauda equina (NCE) were studied by light and electron microscopy. Examination of sacral intradural rootlets revealed inflammatory cell infiltrates and an array of myelinated fiber changes which included myelin stripping by invading mononuclear cells and macrophages, as well as splitting and vesiculation of myelin lamellae without obvious participation by leukocytes. More distally in the extradural roots, there was marked granulomatous inflammation, and demyelinative changes were overshadowed by widespread evidence of irreversible axon damage. In all cases, unusual crystalline inclusions appeared in the endoneurium and sheaths of the rootlets and fascicles. The changes in NCE were compared and contrasted with those observed in acute and chronic inflammatory demyelinative neuropathies.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 53 (1981), S. 137-143 
    ISSN: 1432-0533
    Keywords: Inherited hypertrophic neuropathy ; Demyelinating neuropathy ; Dog
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A recessively inherited hypertrophic neuropathy was discovered in Tibetan Mastiff dogs. Affected pups developed a generalized weakness with hyporeflexia shortly after weaning. Diagnostic findings in 10–18-week-old pups included: moderate to severe reduction in nerve conduction velocities, infrequent denervation potentials, and inconstant elevation in CSF protein. Light- and electron-microscopic studies of peripheral nerves and roots revealed widespread demyelination and primitive onion bulb formation with relatively little degeneration of axons. Myelin changes often were most striking in the cytoplasmic regions of the sheaths and consisted of separations at the major dense lines, anomalous incisure patterns, and marked filamentous accumulations in the inner spirals and adaxonal cytoplasm. The results of these initial studies suggest an inborn defect in the Schwann cell's ability to form or maintain a stable myelin sheath.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 67 (1985), S. 247-253 
    ISSN: 1432-0533
    Keywords: GM2 Gangliosidosis ; Dog ; Meganeurites
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A storage disease in a 2-year-old Japanese Spaniel resembled a GM2 gangliosidosis previously identified in a now extinct line of German Shorthaired Pointers. Despite a later appearance of signs in the Japanese Spaniel, the distribution, staining, and ultrastructure of the stored material were similar in the two breeds. Golgi studies of cerebral cortical neurons revealed the formation of spiny and aspiny enlargements at the axon hillock region (meganeurites) and the growth of secondary neurites from this region. As in the German Shorthaired Pointer model, there was massive storage of GM2 ganglioside as well as a seemingly paradoxical increase in total β-hexosaminidase activity measured in vitro.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 63 (1984), S. 51-56 
    ISSN: 1432-0533
    Keywords: Peripheral nerves ; Dog diseases ; Transplantation ; Schwann cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Segments of peripheral nerve from dogs with canine inherited hypertrophic neuropathy (CIHN) were transplanted to the transected sciatic nerve of immuno incompetent mice. Regenerating mouse axons penetrated the grafts and were myelimated by dog Schwann cells. In grafts collected 3 or more months after transplantation, filamentous or granular material, identical to that occurring in nerves of affected dogs, accumulated in myelinating Schwann cells. Demyelinated fibers were only rarely found in grafted segments of affected nerve. Neither filamentous accumulations nor demyelination were observed in grafts of control canine nerve. These results indicate that CIHN is associated with a defect in Schwann cell function, and the abnormal accumulations of filaments suggest that the defect may be in the cytoskeleton. The rarity of demyelination in grafts suggests that some factor in addition to the Schwann cell defect is required to precipitate myelin destruction.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 76 (1988), S. 46-54 
    ISSN: 1432-0533
    Keywords: Polyradiculoneuritis ; Protozoa ; Schwann cell destruction ; Axon degeneration ; Dog
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Four pups in a litter of eight Labrador Retrievers suddenly developed hind limb weakness. In three, paralysis ascended rapidly resulting in quadriplegia, cervical weakness, dysphagia and death. Postmortem examination revealed a severe polyradiculoneuritis in which roots, ganglia, and spinal and cranial nerves were heavily infiltrated by lymphocytes, plasma cells and macrophages and contained abundant protozoan pseudocysts. On sections of the brain and spinal cord protozoa were less frequent and appeared independent of the glial nodules which marked focal areas of necrosis. The organisms innitially were thought to beToxoplasma gondii, but this supposition was not supported by serological, immunocytochemical, or electron microscopic findings. Ultrastructurally the organisms resembled an unidentified sporozoan parasite, which has been reported in the CNS of dogs in Scandinavia. The inflamed spinal roots contained many degenerated and demyelinated axons. Electron microscopic studies indicated that the tachyzoite-like organisms, through their invasive and proliferative activities, brough about many of the degenerative changes in the Schwann cells and axons of the spinal roots and nerves.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-0533
    Keywords: Niemann-Pick disease type C ; Neuroaxonal dystrophy ; Lamellar inclusions ; Sphingomyelin ; Feline model
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 9-week old domestic short-hair kitten with progressive neurological dysfunction had histopathological lesions consistent with a lysosomal storage disease. Light microscopy of the brain, spinal cord, and ganglia revealed distention and vacuolation of many neuronal populations, and extensive neuroaxonal dystrophy. Large numbers of foamy macrophages were observed in the liver, spleen, lymph nodes, and lung. Hepatocytes appeared pale and swollen. Ultrastructural examination of all affected tissues and organs revealed heterogeneous membranous inclusions. Lipid analysis of liver revealed an excess of cholesterol, glucosylceramide, lactosylceramide and phospholipids including sphingomyelin. There was some increase in the levels of brain GM2 and GM3 gangliosides. Sphingomyelinase activity in liver was partially deficient or low normal. Skin fibroblasts were cultured from two affected cats from the colony established with littermates of the subject of this report. The cultured skin fibroblasts had partially decreased sphingomyelinase activity and a greatly decreased ability to esterify exogenous cholesterol. Clinical, morphological, and biochemical findings suggest that this cat had sphingolipidosis similar to human Niemann-Pick disease type C, a disease not previously described in the cat. The feline form of this storage disease may provide a useful model for studies on the human disease.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Equine motor neuron disease ; Eosinophilic inclusions ; Bunina bodies ; Comparative neuropathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Equine motor neuron disease (EMND) is a sporadic, progressive neurodegenerative disorder that has been identified recently in horses of different breeds in North America. The cause is unknown. Pathologic changes which occur in spinal and certain brain stem motor neurons include chromatolysis, swelling, neurofilamentous accumulation, and development of eosinophilic cytoplasmic inclusions. Punctate eosinophilic inclusions, the type usually encountered in degenerating neurons, resembled Bunina bodies at the light microscopic level, but differed in their ultrastructural composition. These and less common but larger juxtanuclear inclusions appeared to be aggregated vesicular residues of membranous organelle degradation. The third kind of eosinophilic inclusion was a marginated derivative of endoplasmic reticulum and consisted of large membrane-bound accumulations of finely granular material. It was concluded that, although the distribution and nature of the lesions in EMND appeared similar to those of human motor neuron disease, none of the equine eosinophilic inclusions duplicated the ultrastructure of Bunina bodies.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 28 (1974), S. 191-204 
    ISSN: 1432-0533
    Keywords: Comparative Neuropathology ; Canine Polyneuritis ; Demyelination ; Onion Bulb Formation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 6-year-old dog was afflicted with a paresis that developed insidiously and abated during a temporary remission. Clinical findings included: tetraparesis, diminished or absent reflexes, muscle wasting, albuminocytologic dissociation, muscle denervation potentials, and delayed peripheral nerve conduction velocities. The pathologic changes were those of a longstanding demyelinating polyradiculoneuritis. Segmental myelin breakdown, which occurred in the presence of invading cells, and apparently in their absence, was accompanied by some Wallerian degeneration. Reparative changes were noted in numerous small onion bulb formations. Myelin was restored to demyelinated axon segments in the cores of the onion bulbs, while axon sprouts appeared in the peripheral Schwann cell lamellae. This polyradiculoneuritis was unlike spontaneous canine neuritides previously reported, and was compared with experimentally induced peripheral nerve disorders and spontaneous poly-neuritides in man.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 39 (1977), S. 43-51 
    ISSN: 1432-0533
    Keywords: Comparative neurology ; Canine neuronal ceroid-lipofuscinosis ; Kufs' disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Intraneuronal accumulation of cytosomes with the fluorescent and tinctorial properties of ceroid-lipofuscin occurred in the C.N.S. of a fully matured dog with signs of slowly progressive cerebellar disease. Ultrastructural study revealed various membrane-bound inclusions in addition to typical lipofuscin granules. The clinicopathologic findings in this case were contrasted with those occurring in English Setters with juvenile neuronal ceroid-lipofuscinosis, and compared with those reported in Kufs' disease in man.
    Type of Medium: Electronic Resource
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