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1.
AIM: To investigate an axonopathy of Merino sheep that caused progressive hindlimb ataxia and slight to moderate paresis, with the purpose of understanding its pathogenesis. METHODS: Tissues were fixed in buffered paraformaldehyde or paraformaldehyde and glutaraldehyde, processed into wax and epoxy resin, respectively, and examined by light and electron microscopy. Fresh frozen spinal cord and trigeminal nerve roots were subjected to homogenisation, centrifugation and two-dimensional electrophoresis. Selected protein spots were identified using matrix-assisted laser desorption ionisation (MALDI) mass spectrometry. RESULTS. By light microscopy, there were large pale foamy spheroidal axonal swellings affecting peripheral as well as central axons. By electron microscopy, these were shown to contain many membrane-bound vesicles. The main abnormalities in expressed proteins involved cytoskeletal elements and myosin heavy chain, the latter interpreted as associated with the molecular motor myosin Va. CONCLUSIONS: The disorder is the same as that described in Merinos in Australia as segmental axonopathy, and believed to have an inherited aetiology. The lesions and protein changes indicate abnormalities of the cytoskeleton, its relationship with the myelin sheath, and myosin Va molecular motor. The consequence appears to be abnormal axonal transport and inability to maintain the integrity of axons and their myelin sheaths.  相似文献   

2.
Aim: To investigate the nature and cause of a progressive ataxia in three 20-month-old Huntaway dogs that were litter mates.

Methods: Affected dogs were examined before they were humanely killed and submitted to necropsy. Selected formalin-fixed tissues were examined by light and electron microscopy.

Results: The lesions were those of axon and myelin degeneration within sensory, proprioceptive and motor tracts of the spinal cord and to a lesser degree some peripheral nerves.

Conclusion: A progressive myelopathy and neuropathy, tentatively described as a central-peripheral distal axonopathy, was present in all 3 dogs.The cause was not determined but was likely to be either genetic or nutritional.

Clinical relevance: In the early stages of this disease, careful examination maybe necessary to distinguish the signs of ataxia from orthopaedic disease such as hip dysplasia. Affected animals are unlikely to be of use as working dogs.  相似文献   

3.
The recurrent laryngeal nerves were examined by electron microscopy in five control, four subclinical and four clinical laryngeal hemiplegic horses. In addition, the peroneal nerve was examined in two horses in the latter group. The distally distributed loss of large myelinated fibres in the left recurrent laryngeal nerve seen by light microscopy was confirmed. In addition, active axonal pathology was found to be more evident than indicated by light microscopic investigations. The onion bulb formations observed indicated the repetitive nature of the damaging influence to nerve fibres. Although the pathological changes were most obvious in the distal left recurrent laryngeal nerve, alterations similar in type and distribution were present in other areas of the left and right nerves, and in the distal hindlimb nerves. The observation of fibres with inappropriately thick myelin sheaths relative to their axonal calibre, was confirmed statistically by determining the regressions of axis cylinder perimeter against the number of myelin lamellae. In conclusion, the peripheral nerve pathology of equine laryngeal hemiplegia was demonstrated to be a distally distributed loss of myelinated fibres, with considerable active axonal damage, in conjunction with axonal atrophy. These features suggest that this disease may be classified as a distal axonopathy.  相似文献   

4.
AIM: To investigate the nature and cause of a progressive ataxia in three 20-month-old Huntaway dogs that were litter mates. METHODS: Affected dogs were examined before they were humanely killed and submitted to necropsy. Selected formalin-fixed tissues were examined by light and electron microscopy. RESULTS: The lesions were those of axon and myelin degeneration within sensory, proprioceptive and motor tracts of the spinal cord and to a lesser degree some peripheral nerves. CONCLUSION: A progressive myelopathy and neuropathy, tentatively described as a central-peripheral distal axonopathy, was present in all 3 dogs. The cause was not determined but was likely to be either genetic or nutritional. CLINICAL RELEVANCE: In the early stages of this disease, careful examination may be necessary to distinguish the signs of ataxia from orthopaedic disease such as hip dysplasia. Affected animals are unlikely to be of use as working dogs.  相似文献   

5.
The long-term effect of betamethasone on the myelination of commissural and associational fibres was investigated in fetal sheep. We studied the corpus callosum and subcortical white matter by electron microscopy. Axons were subdivided into classes according to their axonal diameter: class I: < or =0.65 microm; class II: 0.66-0.84 microm; class III: > or =0.85 microm. Under control conditions, the different functions of the white matter tracts examined were reflected by three morphological criteria: (1) there was a diverse percentage of axonal classes in the investigated areas. In corpus callosum the axons of class II predominate (47.1%). In the subcortical white matter, class I axons with small diameter are in majority (40.8%). (2) In the subcortical white matter more axons are present, with especially large diameter and hence of axonal class III. (3) The axons of subcortical white matter have thicker myelin sheaths than those of the corpus callosum. Betamethasone administration caused a significant decrease of class II axons in the corpus callosum (36.9%). In corpus callosum, axons of all classes present thicker myelin sheaths. Betamethasone administration resulted in a change in the formation of the myelin sheath in the commissural fibres of the corpus callosum but not in the associational fibres of the subcortical white matter. This could be the morphological correlate to behavioral and cognitive changes known to occur in humans after prenatal glucocorticoid treatment.  相似文献   

6.
A male 14-year-old Arab horse was pathologically diagnosed as equine motor neuron disease (EMND), which was kept as a breeding horse on a farm in Tokachi district of Hokkaido in Japan. On examination of the peripheral nerves, the most characteristic feature was Wallerian-type degeneration revealed by myelinoclasis associated with myelin ovoids which were sometimes infiltrated by macrophages. The other abnormalities were axonal swellings which were surrounded by thin myelin sheaths. Ultrastructurally, the axonal swelling was due to an accumulation of neurofilaments, and was accompanied by a thin and degenerating myelin sheaths. In teased nerve fiber preparations, the most conspicuous change was myelinoclasis represented by segmentation into myelin ovoids or balls. Occasionally, segmental demyelination and axonal degeneration characterized by multifocal axonal swelling were observed.  相似文献   

7.
Nine horses with clinical signs of Australian Stringhalt were killed and tissues collected for a detailed pathological study. Lesions were limited to peripheral nerves and muscles. The most severely affected nerves were the superficial and deep peroneal, distal tibial, plantar digital, volar and recurrent laryngeal nerve with changes characterised by a selective loss of large diameter myelinated fibres with various degrees of demyelination, fibrosis, Schwann cell proliferation and onion-bulb formation. A routine evaluation of the brain and spinal cord by light microscopy failed to reveal any consistent abnormalities. Morphometric analysis of deep peroneal and recurrent laryngeal nerves confirmed the reduced number of large diameter myelinated axons. Teased fibre preparations of these nerves did not show any abnormalities in internodal distance. The most severe muscle lesions were in the long and lateral digital extensors, cranial tibial, dorsal cricoarytenoid, gracilis and lateral deep digital flexor with extensive atrophy of fibres and diffuse fibrosis. Histochemical evaluation of the long digital extensor from 3 affected horses showed an abnormally wide distribution in fibre size and a reduction in type II fibres compared with controls. These lesions are consistent with a distal axonopathy leading to neurogenic muscle atrophy. The distribution of neuromuscular lesions in Australian Stringhalt may be explained by the susceptibility of longer, larger myelinated nerve fibres to injury, but the cause for this distal axonopathy remains unknown.  相似文献   

8.
Serial peripheral nerve biopsies from two golden retriever littermates with chronic neurologic disease were taken for morphologic and morphometric evaluation. Teased nerve preparations were difficult to interpret due to the lightness of myelin staining. Light and electron microscopic findings were characterized by the following: reduced number of myelinated axons, presence of myelinated sheaths inappropriately thin for the caliber of the fiber, poor myelin compaction, increased numbers of Schwann cell nuclei, increased concentration of neurofilaments in myelinated axons, many Schwann cells with voluminous cytoplasm, and increased perineurial collagen. Onion bulb formation was not seen. In contrast to control data, a poor correlation was seen between numbers of myelin lamellae (ML) and axonal circumference (AC). The frequency distribution of ML ranged from 5 to 55 lamellae in affected animals (mean, 28 lamellae) compared to 20 to 140 lamellae in controls (mean, 66 lamellae). The ML/AC ratio was significantly reduced (P less than 0.001) in nerves of affected dogs. Morphometric results indicated that fibers of all calibers were hypomyelinated.  相似文献   

9.
The total number, distribution and size of the optic axons of the buffalo (Bos bubalis) were studied in five optic nerves using light and transmission electron microscopes. Semithin sections were cut at 0.40 μm with an ultramicrotome and stained with 0.3% toluidine blue for light microscopic analysis. The ultrathin sections were cut at 70 nm, mounted on copper mesh grids (No. 200) and stained with saturated solution of uranyl acetate dihydrate and examined with the transmission electron microscope. The optic axons were assessed in 10 regions of roughly equal area. The total number of optic fibers was estimated on semithin and ultrathin sections. The measurements of the axonal diameters were made on electron micrographs taken from various regions across the optic nerve. The mean value of the total number of optic axons calculated was 1 397 099 ± 10 995 and 1 584 255 by light and electron microscopes, respectively. There was very little difference in the density of the optic fibers between the 10 regions of the optic nerve. The optic nerve area ranged from 18.2 to 22.4 mm2 (mean, 21.3 mm2). The diameters of the optic axons ranged from 0.5 to 12.3 μm (mean, 3.3 μm).  相似文献   

10.
The clinical and pathological features of 19 neonatal Holstein-Friesian calves affected with moderate to severe neurological disease are presented. Most calves were recumbent from birth, and many developed variable neurological signs including hyperaesthesia or depression, limb extension, head tremor, nystagmus, apparent blindness, and opisthotonos when stimulated. Consistent lesions of moderate to severe, diffuse, axonal swelling and loss, with Wallerian-type degeneration and myelin depletion in the spinal cord and brainstem, and occasionally in the midbrain and peripheral nerve roots, were observed. The lesions indicated a pre-natal insult affecting mainly motor areas of the foetal neuraxis, however the aetiology of the disorder remains undetermined. It is suggested that the calves may have been affected by a hitherto unrecognised disease entity for which we propose the term, degenerative axonopathy.  相似文献   

11.
Electrophysiologic investigations of motor and sensory nerve as well as ventral nerve root function were performed on 12 dogs with suspected acute canine polyradiculoneuropathy (ACP) at different stages and with different severity of disease. The most reliable electrophysiologic indicators of ACP were electromyographic changes (occurring in 100% of affected dogs), significantly decreased compound muscle action potential amplitudes (in 75, 90, and 100% of affected dogs at all sites along the sciatic/tibial, radial, and ulnar nerves, respectively), increased minimum F-wave latencies (67%), increased F ratios (92%), and decreased F-wave amplitudes (67%). These findings suggest that ACP represents a peripheral motor axonopathy, with demyelination and axonal involvement also occurring in ventral nerve roots. Evidence of peripheral demyelination was present in some dogs although it was overshadowed by the prominent axonopathy. ACP more closely resembles the acute axonal or intermediate forms of Guillain-Barré syndrome in people.  相似文献   

12.
Numerous cases of ataxia, hind limb paresis, and paralysis have occurred in cheetah (Acinonyx jubatus) cubs over the past 10 yr within the European Endangered Species Program population, including 12 in mainland Europe, two in the British Isles, one in Namibia, and one in Dubai. The condition is the most important medical factor limiting European cheetah population growth. Eight cubs at the Salzburg Zoo, Austria, were affected. They demonstrated upper motor neuron lesions when alive and bilateral, symmetrical myelin degeneration of the spinal cord on necropsy. Ballooning of myelin sheaths surrounded mostly preserved axons, and no spheroids, characteristic of acute axonal degeneration, were found. Myelin loss markedly exceeded axonal degeneration. The syndrome's etiology is unclear, although viral, bacterial, parasitic, genetic, nutritional-metabolic, toxic, and physical causes have been considered.  相似文献   

13.
The spinal nerve roots and dorsal ganglia of 104- to 135-week-old rats with spontaneous radiculoneuropathy were examined by light and electron microscopy. Demyelination was common in myelinated fibers of various diameters of both ventral and dorsal roots. The most striking alteration was wide distention of myelin sheaths, which extended throughout the entire internode. The spaces formed between separated lamellae frequently were invaded by macrophages. Subsequent vesicular degeneration of myelin seemed to be mediated by invading macrophages. These processes caused complete myelin destruction, but most axons showed no degenerative changes except for obvious reduction in diameter. Occasionally, there were clumping and partial degradation of neurofilaments and ruptured axolemma in the severely demyelinated axons. A few fibers also were undergoing wallerian-type degeneration, perhaps secondary to the severe demyelinative changes. Remyelinating fibers in various phases of repair were coexistent with markedly demyelinated ones. Demyelinative changes described above also developed within some of these remyelinated internodes. There were no remarkable changes in neurons of the dorsal root ganglia, though accumulation of lipofuscin was common. Our findings suggest that the changes in the nerve roots are essentially a primary segmental demyelination in aging rats with radiculoneuropathy.  相似文献   

14.
Peripheral hypomyelinization was found in 2 Golden Retriever littermates that had pelvic limb ataxia, depressed postural reactions, and depressed segmental reflexes. Diagnostic findings included infrequent denervation potentials, reduced or absent evoked potentials, and markedly diminished motor nerve conduction velocities. Light and electron microscopy of peripheral nerves revealed fewer than normal myelinated axons, myelinated sheaths inappropriately thin for the caliber of the fiber, poor myelin compaction, greater than normal numbers of Schwann cell nuclei, many Schwann cells with voluminous cytoplasm, and greater than normal amount of perineural collagen. Findings were compatible with a peripheral hypomyelinization process; a defect in Schwann cell function was suspected.  相似文献   

15.
Vibrissae (whiskers) play a key role in underwater orientation in foraging phocids through vibrotactile sensation processing. Our aim was to evaluate the structure of northern elephant seal (NES) vibrissae by means of light (LM) and transmission electron microscopy (TEM), in order to elucidate their function. Vibrissal follicles were processed using standardized laboratory methods and LM/TEM techniques. Individual follicular axonal numbers were counted and axonal diameter measured and averaged. NES have mystacial, rhinal, supraorbital and labial vibrissae. The vibrissal follicles are histologically subdivided into a ring, upper and lower cavernous sinuses (LCS). Each vibrissa is innervated by the deep vibrissal nerve. The average number of axons per large mystacial vibrissa is 1804 (±123), rhinal 985 (±241), supraorbital 1,064 (±204) and 374 (±65) in labial vibrissa. The entire vibrissal system carries an estimated 148 573 axons, and mystacial vibrissae alone have 125 323 axons. Axonal conduction velocity for each vibrissal type is 55.26 m/s for labial, 56.58 m/s for rhinal and 35.88 m/s for mystacial vibrissae. TEM and LM revealed a plethora of mechanoreceptors within the vibrissal follicles: Merkel cell‐neurite complexes, lanceolate and pilo‐Ruffini end organs. A vast number of sensory axons projecting from the entire vibrissal system indicate that the vibrissal sensory area takes up a large proportion of phocids’ somatosensory cortex. In conclusion, NES has highly sensitive and finely tuned vibrotactile vibrissal sense organs.  相似文献   

16.
AIM: To investigate the nature of a neurological disease in Wiltshire sheep.

METHODS: Three affected lambs were examined, humanely killed and necropsied. Selected neurological tissues were examined by light and electron microscopy.

RESULTS: Primary neurological lesions were confined to the cerebellum and were characterised by loss of Purkinje cells and the presence of large hypertrophied dendrites of surviving Purkinje cells. These contained stacks of smooth endoplasmic reticulum. There was hyperplasia and cell swelling of Bergmann glia. Mild Wallerian-type degeneration affected white matter in the cerebellum and spinal cord.

CONCLUSION: The cerebellar lesions were of a degenerative and reactive rather than hypoplastic nature. These, and the history, suggest a genetic cause with putative inheritance as an autosomal recessive trait. Accordingly, the disorder is described as a cerebellar abiotrophy.  相似文献   

17.
Aim: To investigate the nature of a progressive ataxia in a New Zealand Huntaway dog.

Methods: The affected dog was examined clinically before being humanely killed and necropsied. Selected tissues were submitted to light and electron microscopy and to biochemical analyses.

Results: The histological lesions were interpreted as indicative of one of the forms of mucopolysaccharidosis type-III (dMPS-III), a lysosomal storage disease. Biochemically there was a deficiency of heparan sulphamidase. All the heparan sulphate chains had non-reducing-end glucosamine-N-sulphate residues.

Conclusion: The disease is dMPS-IIIA (Sanfilippo syndrome). An autosomal recessive mode of inheritance can be provisionally assumed from the nature of this disease in other species.  相似文献   

18.
Summary

In a female family line of Holstein‐Friesian cattle a series of trembling calves was born within a period of ten years. All trembling animals were male; female calves appeared normal At necropsy of one calf severe degenerative lesions of spinal cord and brain white matter were observed Both myelin and axons were lost and some macrophages occurred within digestion chambers. Obviously degenerated neurones were not found The lesion was interpreted as a single recessive sex‐linked hereditary trait.  相似文献   

19.
This inedited morphometric study has been developed from healthy canine spinal cord neuron cytoplasm and nucleus, and white matter axonal myelin sheath, from cervical, thoracic and lumbar regions. For the morphometric study, the parameters were area, perimeter, maximum and minimum diameters and roundness for neurons and myelin thickness for axon. For each parameter, 300 neurons were analysed. The results revealed that lumbar neurons had the highest mean values for the analysed parameters, indicating the presence of large neurons in this region, with large axons as a result of myelin thickness, which is proportional to axon calibre. We conclude that these morphometric results can contribute for the establishment of normal patterns, for canine spinal cord cervical, thoracic and lumbar segments.  相似文献   

20.
Myelination, the ensheathing of neuronal axons by myelin, is important for the proper function of both central and peripheral nervous systems. Various studies have investigated the quantitative parameters of myelination in certain species. Pigs are among the species of which their use as laboratory animals in neuroscience research increased the past few decades. However, there is limited data regarding the myelination process in the pig. Moreover, the maxillary nerve is crucial for Pseudorabies Virus (PrV) neuropathogenesis. In this context, a quantitative analysis of various myelination parameters of the maxillary nerve was performed, during the first 5 weeks of porcine post‐natal development, the time period, which exhibits the highest interest for PrV neuropathogenesis. The evaluation was conducted in four groups of uninfected pigs, at the time of birth (group 0w), at the age of 1 week (group 1w), 3 weeks (group 3w) and 5 weeks (group 5w), using toluidine blue staining, immunofluorescence and electron microscopy. Axon and fibre diameter, perimeter and surface, myelin sheath thickness and g‐ratio were measured on histological sections transverse to the longitudinal axis of the maxillary nerve. The thickness of myelin sheath was 0.76 μm for group 0w, 0.94 μm for group 1w, 0.98 μm for group 3w and 1.03 μm for group 5w. The g‐ratio was 0.529, 0.540, 0.542 and 0.531 for the respective animal groups. The results of this study contribute to the understanding of the myelination process in the pig will be used for the study of PrV effects on the myelination development of newborn piglets' maxillary nerve and may shed new light to their vulnerability to the virus.  相似文献   

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