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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Newborn rats inoculated intraperitoneally with a hantavirus strain (SR-11) developed neurological signs such as ataxia and limb paralysis. The main histological lesions were scattered and multiple neuronal degeneration and necrosis with eosinophilic cytoplasmic inclusion bodies (CIB) in the brain, spinal cord and ganglia. Immunohistochemically, the viral antigen was detected in neurons, capillary endothelial and glial cells throughout the nervous tissue and CIB were identified as consisting of hantaviral antigen (nucleocapsid protein). Ultrastructurally, CIB in the neurons consisted of an accumulation of granular or filamentous materials, or both. They were seen near a well-developed Golgi apparatus and associated with well-developed rough endoplasmic reticulum and increased numbers of ribosomes. The present results suggested that this virus strain was highly infective in neurons of the newborn rats and that excess production of viral antigen which accumulated as CIB resulted in the neuronal changes.
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PMID:Pathological studies on central nervous tissues of rats infected with Rattus serotype hantavirus (SR-11 strain). 168 44

As reported previously, the peculiar intracytoplasmic eosinophilic inclusion bodies (IEIBs) extensively appeared in the autopsied brain tissue from a 49-year-old man having familial ataxia with cerebrospinal fluid abnormality, and histochemically showed abundant proteins, but few lipids and carbohydrates. Ultrastructurally, many membrane-bound vacuoles derived from the distended cisterns of rough-surfaced endoplasmic reticulum (RER) appeared in the neurons. They were filled with fine granular, less dense materials. The IEIBs, shown as a homogeneous dense core, were found in some of the vacuoles. Similar vacuoles also appeared in astrocytes, oligodendrocytes, vascular pericytes, ependymal and choroidal epithelial cells. It is suggested that the vacuoles result from the accumulation of metabolic products in the distended RER cisterns of the cells in the central nervous system, presumably representing a genetically determined functional abnormality of the RER in protein synthesis and/or transport.
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PMID:Further postmortem examination of a case of familial ataxia with cerebrospinal fluid abnormality: an electron microscopic study of the intracytoplasmic eosinophilic inclusion bodies in the central nervous system. 255 8

TOCP (Tri-orthocresyl phosphate), an organophosphorus compound, has been implicated in producing neuropathy in the male S. D. rats. Repeated subcutaneous doses of TOCP (600 mg/kg) for up to 6 weeks produced ataxia, most striking at 50 days after final injection, followed by gradual recovery. Ultrastructurally, the internal structure of affected nerve fibers was primarily composed of altered smooth endoplasmic reticulum, tubular membrane system, and mitochondria, although myelin sheath was found to be essentially normal. In the histopathological examination, axonal and myelin degeneration was disclosed in the gracile nucleus and in the gracile fasciculus of the cords as well as in the sciatic nerves. The localization and degree of these changes were considered to be "dying back", showing systemic neuropathy. In addition, muscular lesion showed small group atrophy, corresponding to Type I fiber atrophy.
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PMID:Studies on the delayed neurotoxicity of organophosphorus compounds--(III). 299 36

A 10-year-old boy with the hyperornithinemia, hyperammonemia and homocitrullinuria (HHH) syndrome is described. With dietary restriction of protein intake and supplementary administration of L-ornithine and L-arginine, the high concentration of ammonia decreased and the clinical signs of truncal ataxia and lethargy improved. A deficiency of ornithine transport into liver mitochondria was demonstrated biochemically, and glycogen granules and smooth surface endoplasmic reticulum were increased, but mitochondria showed normal construction ultrastructurally. Cranial computed tomography (CT) showed diffuse white matter low density and cerebellar vermis atrophy. The impairment of ornithine transport and energy production in the central nervous system may be related to the cranial CT findings and neurological signs.
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PMID:Clinical, biochemical and ultrastructural study on the pathogenesis of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. 340 56

1. Two duplicate groups of rainbow trout (Salmo gairdneri; mean weight 27 g) were given diets of differing selenium content (deficient 0.025 mg Se/kg; supplemented 1.022 mg Se/kg) for 30 weeks. 2. There were no significant differences between treatments in weight gain but packed cell volume, liver vitamin E and liver and plasma Se concentrations were all significantly lower in the Se-deficient trout. 3. Ataxia occurred in about 10% of the Se-deficient trout and histopathologies were evident in nerve cord (damage to axon sheath) and liver (loss of integrity in endoplasmic reticulum and mitochondria with appearance of increased vesiculation). 4. Glutathione peroxidase (EC 1.11.1.9) activity was significantly reduced in liver and plasma of Se-deficient fish but there was no indication, from differential assay, of any non-Se-dependent glutathione peroxidase activity. Glutathione transferase (EC 2.5.1.18) activity was significantly increased in Se-deficient trout.
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PMID:Some effects of selenium deficiency on glutathione peroxidase (EC 1.11.1.9) activity and tissue pathology in rainbow trout (Salmo gairdneri). 367 60

Intracerebral inoculation to guinea pigs of cerebrospinal fluid or brain suspensions from 6 patients with Creutzfeldt--Jakob disease was followed in 4 cases by the appearance, after a long incubation period, of a serially transmissible experimental disease. The disease was characterized by tremor, discrete ataxia and convulsions and led to a fatal outcome within several days. Microscopic lesions in the brain of guinea pigs with the experimental disease consisted in glial proliferation, spongiform change and vacuolation of neuron cytoplasm. Electron microscopy proved that the vacuoles were situated in the endoplasmic reticulum and in the cytoplasmic processes.
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PMID:Further data on experimental Creutzfeldt--Jakob disease (spongiform encephalopathy) in the guinea pig. 634 14

Central and peripheral nerve fibre damage has been produced in Long-Evans hooded male rats with tri-ortho-cresyl phosphate. Animals were dosed by gavage with intermittent or daily amounts of the organophosphate and examined after 2, 6, 12, 18 and 24 weeks. The distribution of central nervous system (spinal cord) damage and the differential vulnerability among various peripheral nerves supported a "dying-back' classification for the neuropathy. Giant axonal swellings, containing massive accumulations of smooth endoplasmic reticulum, hallmarked the neuropathy. In spite of severe neurological damage the animals displayed only moderate functional disturbances. These findings have shown that the rat is highly sensitive to the structural damage caused by organo-phosphates, although resistant to the ataxia.
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PMID:A rodent model of organophosphorus-induced delayed neuropathy: distribution of central (spinal cord) and peripheral nerve damage. 652 45

The temporal bones of a patient who suffered sudden deafness and ataxia after administration of both furosemide and ethacrynic acid, were prepared for light and electron microscopy. There was no loss of hair or supporting cells. However, some hair cells, in both the vestibular neuroepithelium and the organ of Corti, particularly in the basal turn, were more densely staining and more granular than normal. Membrane whorls also were common within mitochondria of such cells. The endoplasmic reticulum of some spiral ganglion cells was dilated. The major cytologic changes were found in the stria vascularis of the cochlea and dark cell areas of the vestibular system. There was marked dilatation of the intercellular fluid spaces, consistent with the biochemical observation that loop diuretics interfere first with enzyme systems responsible for fluid transport within the inner ear.
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PMID:Ultrastructural histopathology in a case of human ototoxicity due to loop diuretics. 697 8

Myelin deficiency (md) is a new mutant in the Wistar rat caused by an X-linked recessive lethal gene. One-half of the male offspring develop tremor and ataxia at 10-12 days of age and seizures at 16-21 days. Usually, the animals die 24-28 days postnatally unless survival is prolonged by anticonvulsants. Light microscopic examination of the C.N.S. shows a complete lack of myelin. The P.N.S. is normally myelinated, however. Frontal cortex, corpus callosum, optic nerves, cerebellum and spinal cord were studied routinely in affected animals aged 3-46 days. Abnormal males were identified three days after birth by the absence of myelinated axons from the ventral funiculus of the cervical cord. In mutants aged 3-16 days, axons had the usual ultrastructural features but were either entirely non-myelinated or, rarely, were invested by poorly organized, non-compacted, myelin-like loops of membranes, 2 to 4 in number. In mutants aged 17-20 days, axonal swellings appeared. These increased in number with longer survival times and contained large numbers of microtubules, neurofilaments, mitochondria and dense bodies. Normal C.N.S. myelin was not observed at any age. Two types of abnormal glial cell occur in md. The first, present in white matter at three days of age, is an abnormal oligodendrocyte. The cytoplasm contains dilatation of the rough-surfaced endoplasmic reticulum and the nuclear envelope is widened. A second cell-type, conspicuous by 10 days, has an electron-dense nucleus with prominently clumped chromatin and large cytoplasmic lipid droplets. This second cell type is believed to be a microgliacyte. The number of cytologically-normal oligodendrocytes decreases as mutants age while hypertrophied, filament-rich astrocytes occur in increasing numbers. The myelin defect in md C.N.S. is probably due to an abnormality of oligodendrocytes. Axonal alterations are probably secondary. Myelin deficiency resembles the murine mutant, Jimpy (jp), although ultrastructural changes in oligodendrocytes appear to be dis-similar and md, in contrast to jp, contains no normal-appearing C.N.S. myelin.
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PMID:Ultrastructure of the central nervous system in a myelin deficient rat. 713 Oct 49

Cerebellar lesions develop in a heritable disorder characterized by recurrent episodic seizures in newborn and young calves and by ataxia in survivors of several bouts of convulsions. Earliest changes were altered patterns of phosphatase reaction products in Purkinje cells. Purkinje cells axons in the outer half of the granular layer developed fusiform or spheroidal argyrophilic swellings. Early lesions were restricted to the lingula and uvula, but cases with more persistent clinical disease involved other parts of the vermis. In ataxic calves these lesions were also in the cerebellar hemispheres. The axonal swellings showed proliferation of tubulovesicular endoplasmic reticulum, neurofibrils and mitochondria. In other swellings axoplasm was degenerate, and in some it resembled Purkinje cell cytoplasm. In some clinically normal adult transmitters of the disorder, light microscopy showed some similar swellings, but most calves that recovered had no lesions.
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PMID:Morphogenesis of cerebellar lesions in bovine familial convulsions and ataxia. 746 75


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