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

Lesions of the brain stem and cerebellum due to toxic substances or nutritional deficiencies are mostly seen in chronic alcohol abuse and, more rarely, in severe malnutrition. Four clinical entities are described: Wernicke's encephalopathy is the most frequent of these, with progressive development of disorders of consciousness, oculomotor palsies and ataxia. The condition regresses under massive vitamin BA therapy, but it often leaves neuropsychological sequelae (e. g. Korsakoff's syndrome). The best treatment is prevention by giving thiamine to alcoholics and to malnourished subjects who receive glucose infusions. Cerebellar atrophy of alcoholic origin is responsible for subacute ataxia which, as a rule, is definitive. Central pontine myelinolysis is rare and presents as sudden quadriplegia with pseudobulbar palsy and sometimes "locked-in" syndrome. Beside alcohol, a frequent cause is major ionic disorders, such as hyponatraemia or its excessively rapid correction. Pellagra is a classical disease rarely seen in this country. It is due to nicotinamide (vitamin PP) deficiency.
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PMID:[Lesions of the brain stem and cerebellum of alcoholic and nutritional deficiency origin]. 234 51

The lethality of Poa huecu, a plant toxic to cattle and sheep, was followed by injection of chromatographic fractions in mice. The lethal aqueous extract was administered i.p. to Rockland mice of either sex and produced motor incoordination, transient ataxia, rough hair coat, tremors and muscle contractions and, occasionally, blindness. Doses greater than 1.5 g/kg mouse were always lethal. Fractionation of this lethal extract included dialysis, column chromatography on Sephadex G-25 and fractional precipitation with ethanol. Precipitates obtained with 70% and 85% ethanol were further purified on a DEAE-cellulose column. Eight fractions were obtained, each was injected into mice. Only fractions 3-6 were toxic. Fraction 3 produced slight hepatosis and hyperemia in the liver and gliosis in the brain. None of the other tissues exhibited histological lesions. Fractions 4 and 5 caused death of all animals within 30 min to 4 hr after injection. Polyacrylamide gel electrophoresis and acid hydrolysis showed that fractions 4 and 5 contained a glycoprotein of nearly the same mol. wt (67,000-94,000). Microscopic pathology in the mice treated with the lethal glycoprotein of fraction 4 included hyperemia in the kidneys, megakaryocytes in the spleen, slight hepatosis and focal coagulative necrosis with nuclear pyknosis and karyonexis in the liver, gliosis, intracellular brain edema with axon degeneration and swollen astrocytes in the brain. These brain injuries may relate to the motor incoordination of cattle that causes a delayed righting reflex. The major monosaccharides of the lethal glycoprotein are glucose and mannose, while rhamnose, arabinose, xylose and galactose are present in low percentages. Proline and the acidic amino acids (glutamic and aspartic acids) are the most abundant in the peptidic residue.
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PMID:The lethal principle of Poa huecu (coiron blanco): a plant indigenous to Argentina. 262 69

"Energy metabolism" is deranged in a wide variety of disorders of the nervous system. This term refers rather loosely to the pathways responsible for the utilization of the major substrates of brain. Primary disorders of energy metabolism are those in which the primary insult affects the cellular machinery required for energy metabolism. A typical example would be a defect in a gene coding for a mitochondrial protein. Biochemically, defects which appear to be hereditary and which lead to disease of the central nervous system have been described in each of the pathways of energy metabolism: glycogenolysis (the break-down of glycogen to glucose); glycolysis (the break down of glucose to pyruvate and lactate); the pyruvate dehydrogenase complex (which oxidizes pyruvate to enter the Krebs tricarboxylic acid cycle); the tricarboxylic acid cycle itself (which completes the oxidation of carbohydrates and other substrates to carbon dioxide); electron transport (which carries out their oxidation to water); the pentose phosphate pathway (an alternate pathway for glucose oxidation); and several "minor" mitochondrial pathways. Clinically, the spectrum of syndromes associated with primary disorders of energy metabolism is wide. Common manifestations include psychomotor retardation, with associated lactic acidosis and/or hypoglycemia. The laboratory abnormalities may be intermittent. Syndromes which have been culled out include congenital lactic acidosis, Leigh disease, intermittent ataxia, Kearns-Sayre-Shy syndrome (KSS), myoclonus epilepsy with ragged red fibers (MERRF), and mitochondrial myopathy-encephalopathy-lactic acidosis-stroke (MELAS). As with other families of inborn errors, both clinical and biochemical heterogeneity occur. Patients with apparently similar clinical syndromes can turn out to have different inborn errors, and patients with abnormalities of the same gene product can have clinically distinguishable syndromes. Secondary disorders are those in which the derangements of energy metabolism are presumably secondary to some other insult but may still be important for the cellular pathophysiology. These include the metabolic encephalopathies and probably a number of well-known neurodegenerative disorders. In the hereditary ataxias, abnormalities of mitochondrial markers are common but do not correlate consistently with the disorders as conventionally classified; a new classification into axonal ataxias, multiple system degenerations, and ataxic encephalopathies may be easier to relate to the pathophysiology.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Energy metabolism in disorders of the nervous system. 297 43

The effect of i.v. administration of ionophores on metabolism in ruminants was investigated in two experiments. In Exp. 1, four Angus heifers were assigned randomly to receive i.v. monensin (18 mg, n = 2) or vehicle (control, n = 2). Samples were collected from indwelling vena cava cannulas from -60 to 240 min. Concentrations of K, Mg (P less than .05) and P (P less than .10) were lower and glucose (GLU) and free fatty acids (FFA) were higher (P less than .05) in monensin-treated than in control heifers. Serum insulin (INS) initially declined and subsequently increased (P less than .05) following monensin administration. A second experiment was conducted to determine the effect of a higher dose of monensin and the effect of lasalocid on minerals and metabolites. Angus (n = 3) and Hereford (n = 3) steers were randomly assigned to treatments in two 3 x 3 latin square designs. Treatments were i.v. administration of monensin, lasalocid or vehicle (ethanol) administered on three consecutive days. Administration of monensin, but not vehicle or lasalocid, resulted in ataxia, hypernea, polyuria and anorexia for approximately 2 h. Plasma concentrations of K, P and Mg were suppressed (P less than .05) by monensin, but not by vehicle or lasalocid administration. The decrease in K was preceded by a transient increase in K 15 min after administering monensin. Concentrations of GLU and FFA increased (P less than .05) following monensin administration. Concentrations of INS were lower from 60 to 120 min and greater at 180 and 240 min compared with -60 to 0 min from monensin administration (P less than .05). These results provide first evidence of an effect of monensin on metabolism in ruminants independent of alterations in ruminal microbial metabolism.
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PMID:Intravenous administration of ionophores in ruminants: effects on metabolism independent of the rumen. 304 32

Clinical observations and results of investigations of pyruvic acid metabolism are reported in 4 children in whom subacute necrotizing encephalomyelopathy of Leigh was diagnosed intravitally. Attention is called to the similarity of the clinical manifestations with its onset in the first year of life, deficient body weight and growth, progressing neurological disturbances (weakening of muscle power, tremor, ataxia, nystagmus), course with periods of exacerbations, tachypnoea, skin changes (hirsutism, telangiectasia, perspiration), death at the age of 2-3 years. The biochemical changes in all children included raised serum levels of lactic acid, pyruvic acid and alanine, and acid-base equilibrium disturbances with metabolic acidosis (relatively balanced respiratory alkalosis). The results of the test of intravenous loading with glucose and alanine carried out in all children indicated indirectly reduced activity of pyruvate carboxylase. In one child histological examination of the brain carried out postmortem confirmed the diagnosis of Leigh's disease.
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PMID:[Suspected pyruvate carboxylase deficiency in 4 children with Leigh disease]. 309 72

Five Holstein cows developed a sudden clinical syndrome of ataxia, muscle tremors, recumbency, and bloody diarrhea. The pond where these cows obtained water contained a near pure culture of Microcystis aeruginosa, a toxic blue-green algae. All cows affected were treated with activated charcoal, procaine penicillin, glucose, and calcium and magnesium gluconate. All 5 cows were clinically normal ten days later. Many practicing veterinarians regard blue-green algae toxicosis as a rare syndrome that results in rapid death for consuming animals; however, this toxicosis may be common and not lethal. Because no diagnostic test is available for blue-green algae toxicosis, this condition rarely is diagnosed.
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PMID:Blue-green algae toxicosis in five dairy cows. 311 31

D-Lactic acid can accumulate in blood in some patients with intestinal failure, leading to a clinical syndrome of severe acidosis and encephalopathy. The possible impact of parenteral nutrition on its clinical course has not been established. One patient with a severe short-bowel syndrome supported by long-term parenteral nutrition who suffered repeated episodes of ataxia and disorientation associated with elevated serum levels of D-lactate was studied. Results demonstrated no impact of glucose- vs lipid-based parenteral nutrition formulations on total acid production or serum D-lactic acid levels, increased serum D-lactate levels during administration of neomycin, but prompt resolution of both acidosis and clinical symptoms with discontinuation of oral intake. This study confirms the findings of other investigators that D-lactic acidosis may be a significant, heretofore unappreciated complication in patients with severe short-bowel syndrome, and that prompt resolution may be effected with abrupt discontinuation of oral intake. Furthermore, the present study suggests neither a detrimental nor a beneficial effect of parenteral nutrition on this syndrome.
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PMID:Effect of parenteral nutrition and enteral feeding on D-lactic acidosis in a patient with short bowel. 312 29

We report two patients with chronic encephalomyelitis due to Borrelia burgdorferi in whom the definite diagnosis was delayed because of atypical clinical features. The first patient presented with chronic spastic paraparesis, slight ataxia and nystagmus of several years' duration. A tentative diagnosis of multiple sclerosis was made in spite of important abnormalities of the CSF biological characteristics. The second patient presented with an acute aphasia and a bilateral Babinski's sign. He was thought to suffer from benign herpetic meningoencephalitis. Several months later, as the patient experienced relapses with cerebellar and spinal cord involvement, falsely positive tests for syphilis were found and an antibiotic treatment was given. High protein content, low glucose levels, pleocytosis and oligoclonal bands were observed in all CSF samples, but the definite diagnosis was based on the detection of serum and CSF antibodies against B. burgdorferi.
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PMID:[Borrelia burgdorferi encephalomyelitis]. 318 97

The clinical course of herpes zoster associated encephalitis (HZAE) with special emphasis on the treatment with acyclovir is described from the experience in 14 own patients and 47 review cases. Immunosuppression and dissemination involved increased risk of HZAE, whereas cranial zoster implied no or only a slightly increased risk. The symptoms were mainly disturbances of mental function and ataxia. Nuchal rigidity was noted in approximately one third of cases. The median duration from dermatomal lesion to HZAE was 15 days in immunosuppressed patients versus 5 days in non-immunosuppressed patients. Abnormal spinal fluid findings included mononuclear pleocytosis, occasionally with low glucose concentration. Protein was elevated in half of the patients. Serum sodium levels were often low. Brain CAT scans were generally normal and EEGs always abnormal. Recurrence of HZAE was noted in 2 patients. Treatment with acyclovir seemed to have a beneficial effect. The results, however, need cautious interpretation due to the heterogenous patient material. Two patients developed signs of HZAE while on treatment with desciclovir but recovered during ongoing therapy.
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PMID:Herpes zoster associated encephalitis: clinical findings and acyclovir treatment. 322 75

Eleven patients with acquired cerebellar degeneration (10 of whom had paraneoplastic cerebellar degeneration [PCD]) were evaluated using neuropsychological tests and 18F-fluorodeoxyglucose/positron emission tomography to (1) quantify motor, cognitive, and metabolic abnormalities; (2) determine if characteristic alterations in the regional cerebral metabolic rate for glucose (rCMRGlc) are associated with PCD; and (3) correlate behavioral and metabolic measures of disease severity. Eighteen volunteer subjects served as normal controls. Although some PCD neuropsychological test scores were abnormal, these results could not, in general, be dissociated from the effects of dysarthria and ataxia. rCMRGlc was reduced in patients with PCD (versus normal control subjects) in all regions except the brainstem. Analysis of patient and control rCMRGlc data using a mathematical model of regional metabolic interactions revealed two metabolic pattern descriptors, SSF1 and SSF2, which distinguished patients with PCD from normal control subjects; SSF2, which described a metabolic coupling between cerebellum, cuneus, and posterior temporal, lateral frontal, and paracentral cortex, correlated with quantitative indices of cerebellar dysfunction. Our inability to document substantial intellectual impairment in 7 of 10 patients with PCD contrasts with the 50% incidence of dementia in PCD reported by previous investigators. Widespread reductions in PCD rCMRGlc may result from the loss of cerebellar efferents to thalamus and forebrain structures, a "reverse cerebellar diaschisis."
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PMID:The metabolic anatomy of paraneoplastic cerebellar degeneration. 326 71


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