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

Tremors, mental changes, opsoclonus, muscle weakness, gait ataxia, incoordination, and slurred speech developed in several employees in a Virginia chemical plant during the summer of 1974. Epidemiologic and clinical studies suggested that the chlorinated insecticide chlordecone (Kepone) was responsible. Severity of symptoms seemed directly related to dose and duration of exposure. Five sural nerve and six muscle biopsy specimens were examined by light microscopy and electronmicroscopy. The sural nerves were also evaluated by computerized morphometry, which showed considerable decrease in the number of unmyelinated fibers and lesser abnormalities of myelinated fibers. Compared with the nerves of the control subjects, those of patients may have had an increase in Reich and Elzholz bodies, and a modest increase in endoneurial collagen. There were occasional "collagen pockets," stacks of Schwann cell cytoplasmic membranes, redundant Schwann cell cytoplasmic folds, and fewer unmyelinated axons. The skeletal muscles contained increased amounts of lipofuscin and lipidlike droplets in subsarcolemmal areas and within intermyofibrillary spaces; the significance of this is unknown. Fiber size variability, type I predominance, and type grouping were present in three cases. All results strongly suggest that chlordecone is a neurotoxic agent predominantly affecting Schwann cells and unmyelinated fibers of peripheral nerves.
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PMID:Chlordecone intoxication in man. II. Ultrastructure of peripheral nerves and skeletal muscle. 7 56

The clinical and pathological characteristics of a chondroma rodens affecting the internal occipital protruberance, cerebellar meninges and the cerebellum of a dog, are described. The principle clinical findings were Horner's syndrome and ataxia. The histopathologic differentiation of this tumour from other fibro-osseous growths, is discussed.
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PMID:Chondroma rodens in a dog. 8 5

Chronic bipolar electrodes were implanted in cortical, limbic, diencephalic and mesencephalic regions of the rat. Following recovery from surgery the rats were maintained for 14--26 days on a liquid diet in which 35--42% of total calories were provided by ethanol. Following ethanol withdrawal, electrographic and behavioral monitoring was continued for 8--10 h. The withdrawal of ethanol resulted in the time-dependent appearance of a variety of withdrawal signs including tail arching, ataxia, rigidity, tremor and spontaneous and audiogenic convulsions. These behavioral signs were accompanied by the development of epileptiform abnormalities across wide-spread brain regions. Analysis of preconvulsive spike activity revealed a greater spike frequency in limbic, mesencephalic and non-specific diencephalic regions, as compared to those in cortex and specific diencephalon. Seizure discharge during the tonic-clonic phase of the primary audiogenic convulsion was initiated in the mesencephalon or amygdala, but spread rather extensively to the remainder of the brain. In those instances, however, where multiple convulsions occurred following the audiogenic convulsions, there was a marked decline in spread of seizure discharge to the cortex. These results were interpreted to support the notion that some degree of neuroanatomical specificity exists in the genesis of epileptiform abnormalities during ethanol withdrawal. A comparison of these results with those studying the neural mechanisms underlying other forms of generalized epilepsy was made. It is hypothesized that central pacemaking regions such as medial thalamus or reticular formation may serve to organize isolated epileptiform activity into coherent patterns of paroxysmal activity throughout the brain during the ethanol withdrawal syndrome.
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PMID:Ethanol dependence in the rat: role of non-specific and limbic regions in the withdrawal reaction. 8 50

Feeding of 260 ppm of leptophos to mallard ducklings caused delayed neurotoxicity similar to that reported for hens. Thus leptophos caused ataxia, with subsequent paralysis, loss of appetite, and slow-down in the growth rate of the treated birds. Spinal lesions were identical in morphology and distribution to those seen in hens following leptophos administration. The severity of histologic changes correlated both with the clinical condition and the duration of intoxication.
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PMID:Delayed neurotoxicity induced by organophosphorus compounds in the wild mallard duckling: effect of leptophos. 8 97

A neurological disorder developed after prolonged exposure to nitrous oxide in 15 patients, all but 1 of whom were dentists. 13 patients had abused nitrous oxide to some extent for periods ranging from 3 months to several years, but 2 patients were exposed to nitrous oxide only professionally, by working in poorly ventilated surgeries. Symptoms included early sensory complaints, Lhermitte sign, loss of balance, leg weakness, gait ataxia, impotence, and sphincter disturbances. Neurological examination showed sensorimotor polyneuropathy, often combined with signs of involvement of the posterior and lateral columns of the spinal cord. Electrodiagnostic tests pointed to an axonal polyneuropathy, but other laboratory results were normal, including examination of the spinal fluid. The neurological picture is similar to that of subacute combined degeneration of the spinal cord, and it is possible that nitrous oxide interferes with the action of vitamin B12 in the nervous system.
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PMID:Myeloneuropathy after prolonged exposure to nitrous oxide. 8 36

Studies on acute toxicities of pepleomycin sulfate were carried out in both sexes of mice and rats, comparing with bleomycin, and male dogs. Pepleomycin was administered subcutaneously, intravenously and intraperitoneally in both sexes of mice and rats, and intravenously in male dogs respectively. Mice and rats, and intravenously in male dogs respectively. Mice and rats were observed respectively for 10 and 14 days after the administration. LD50 values were calculated by the method of Litchifield & Wilcoxon. LD50 values of pepleomycin were 4 approximately 6 times smaller than those of bleomycin in all routes of mice, but difference between them was not significant in all routes of rats. Additionally sex-difference of LD50 values was scacely recognized in all routes of both species. Toxicological findings observed in common to all routes of both species were ataxia, depression, tremor and epiphora, and only in all routes of mice, head-twitch, running-round and rolling were especially recognized as toxic behavior, which were not observed in bleomycin. Hepatic and renal lesions were recognized in biochemically and histopathologically in the survived rats. The dogs treated with pepleomycin 50 and 30 mg/kg had the decrease in food intake and the loss of body weight. They became moribund in 9 approximately 36 days after administration. In these dogs the lesions of liver and kidney were severely recognized in biochemical and histopathological findings. One of them which received 50 mg/kg recovered biochemically and histopathologically in 209 days after administration by the supplemental nutrition in early stage.
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PMID:[Toxicological studies on pepleomycin sulfate (NK 631). I. Acute toxicity of pepleomycin in mice, rats and dogs (author's transl)]. 8 4

A virus isolated from the CSF of a patient who had amyotrophic lateral sclerosis for 7 years, and prolonged pleocytosis in the CSF, was adapted to suckling mouse brain by subsequent serial blind passages. This Schu virus belongs to the tick-borne encephalitis complex of the genus Flavivirus (Togaviridae). Suckling mouse brain homogenate of the 13th passage was used for transmission experiments in various species of laboratory animals. Golden hamsters infected subcutaneously fell ill after a number of months, lost weight, and had paresis of the legs. Histologically they had petechial hemorrhages in different parts of the CNS and inflammatory changes in the gray substance of the spinal cord. Pilot studies with repeated inoculations of small doses of different flavivirus strains suggest a course of the disease in experimental animals which resembles slow-virus infections insofar as no encephalitis is produced and degenerative changes of the anterior horn cells prevail over inflammatory signs in the spinal cord. After intracerebral application of Schu virus, cynomolgus monkeys developed the typical lesions of togavirus panencephalitis with epileptic seizures, ataxia, and paresis. After subcutaneous application, the virus seems to spread along peripheral nerves to anterior spinal roots and spinal cord, where mainly motor neurons of the anterior horn are damaged, and from there to the brain. The histological findings are such that one may assume the disease of the patient was due to the infection with the virus isolated from his CSF. Therefore, the hypothesis may be advanced that at least some of the cases diagnosed as amyotrophic lateral sclerosis are due to a togavirus infection.
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PMID:Attempts to reproduce amyotrophic lateral sclerosis in laboratory animals by inoculation of Schu virus isolated from a patient with apparent amyotrophic lateral sclerosis. 8 63

Head movement-dependent oscillopsia (HMDO) with peripheral vestibular, brainstem and cerebellar lesions is reviewed. The differentiation of this kind of oscillopsia is based mainly on clinical grounds. HMDO with bilateral abolition of caloric responses, and in the absence of disease of the central nervous system, is due to bilateral vestibular disease. HMDO in patients with internuclear ophthalmoplegia and other brainstem signs is probably due to a lesion of VOR pathways in or near the medial longitudinal fasciculus. The occurrence of HMDO with ataxia of gait and cerebellar eye movement disorders (rebound nystagmus, flutter-like oscillations), in the absence of brainstem lesions (medial longitudinal fasciculus), is clinical evidence for HMDO due to a cerebellar lesion. An attempt is made to associate the different kinds of oscillopsia with current knowledge of the vestibulo-ocular reflexes.
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PMID:Clinical and theoretical aspects of head movement dependent oscillopsia (HMDO). A review. 8 58

Three siblings presented in early childhood with central-nervous-system (CNS) dysfunction, candida dermatitis, keratoconjunctivitis, and alopecia. Two were studied immunologically and had absent delayed-hypersensitivity skin-test responses and absent in-vitro lymphocyte responses to candida antigen. One of them had selective IgA deficiency and no antibody response to pneumococcal polysaccharide immunisation, and the other had a subnormal percentage of T lymphocytes in peripheral blood. The first two siblings died with progressive CNS deterioration and overwhelming infection. The third child, who presented with a periorificial candida dermatitis, alopecia, keratoconjunctivitis, and intermittent ataxia at eighteen months of age, had intermittent lactic acidosis and raised excretion of beta-hydroxyproprionate, methylcitrate, beta-methylcrotonylglycine, and beta-hydroxyisovalerate in urine. After four days of oral biotin, 10 mg/per day, the metabolites in her urine were significantly reduced, suggesting a biotin-responsive multiple carboxylase deficiency. These findings, taken with previous reports of immune defects in patients with disorders of branched-chain aminoacid catabolism, suggest a new biochemical basis for primary immunodeficiency disease.
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PMID:Multiple biotin-dependent carboxylase deficiencies associated with defects in T-cell and B-cell immunity. 8 54

Leptophos (O-[4-bromo-2,5 dichlorophenyl] O-methyl phenylphosphonothioate) (PhosvelR) was administered orally to chickens and rats in doses of 0.5 and 5.0 mg/kg/day for 26 weeks. Hens fed 5.0 mg/kg, except one, showed ataxia and became paralysed in the legs at varying times from 8 to 19 weeks. A fifth hen showed ataxia early in the experiment but recovered fully for the remainder of the experiment. Rats fed both doses and chickens fed 0.5 mg/kg showed no signs of delayed neurotoxicity. All hens fed 5.0 mg/kg stopped laying by about the third week. Animals of both species fed 5.0 mg/kg either lost weight (chickens) or gained less weight (rats) than the others. Erythrocyte acetylcholinesterase (AChE) of the chickens given both doses was significantly depressed at first, then increased, and later dropped to control levels. AChE of rats fed 0.5 mg/kg was significantly inhibited but soon recovered to within control levels. On the other hand, the AChE of rats fed 5.0 mg/kg was inhibited throughout the experiment. Plasma cholinesterase (ChE) of both species was first inhibited and then recovered erratically for both insecticide concentrations. Histological alterations in the spinal cord of paralysed hens included axon and myelin degeneration in the ventral, lateral and posterior columns. In the paralysed hens, 79% of the neurotoxic esterase in the brain were inhibited, whereas in the non-paralysed hens (including the one non-paralysed hen receiving 5.0 mg/kg/day) and all rats only about half as much was inhibited.
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PMID:Neurotoxic effects of leptophos (PhosvelR) in chickens and rats following chronic low-level feeding. 8 38


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