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T Westermarck

Publications and source records attributed to T Westermarck.

At least 37 records · Page 2Linked to original sources
PubMed1988

Experience over 17 years with antioxidant treatment in Spielmeyer-Sjögren disease.

During the last 17 yr, 74 patients with Spielmeyer-Sjögren disease were treated in Finland with antioxidant supplementation. Twenty-seven patients received a combination of vitamin E, vitamin C, methionine and BHT. As the disease began to progress, the treatment was changed to a combination of sodium selenite and vitamin E in 14 of the 27 patients. The same combination was also given to 47 children (During the last 5-6 yr, vitamins B2 and B6 were also added.) who had not received previous antioxidant supplementation. The latter combination (called the Westermarck formula) appeared to be helpful to some patients. Statistical correlations between various neurological items and relevant laboratory data were sought. In the older patients a significant correlation was found between neurological dysfunction and ceruloplasmin, and also between epilepsy and ceruloplasmin, while a negative correlation was noticed between neurological dysfunction and glutathione peroxidase. In the younger patients, a negative correlation was observed between superoxide dismutase and epilepsy. Serum apolipoprotein B levels were below the normal range in the 6 patients investigated. So far the Westermarck formula seems to have been the best treatment devised yet in Spielmeyer-Sjögren disease, but further studies are needed for a better understanding of the pathogenesis of neuronal ceroid-lipofuscinoses disorders.

Adolescent↗
PubMed1988

Vitamin B6 status of Finnish elderly. Comparison with Dutch younger adults and elderly. The effect of supplementation.

About 25% of Finnish and Dutch elderly appeared to be more or less deficient in vitamin B6 as compared to younger adults. Deficiency was observed at the cellular (PLP, EGOT and alpha-EGOT) as well as at the plasma level (PLP). The benefit of a one-year daily supplementation with 2 mg of pyridoxine-HCl was investigated at the biochemical and psychological level as compared to a placebo group. After one year, none of the supplemented elderly was deficient in biochemical terms. At the psychological level and at the level of general well-being, the elderly supplemented with vitamin B6 showed slight improvements. However, for the psychological variables significant correlations with the vitamin B6 parameters were not observed. Plasma fatty acids (e.g. gamma-linolenic acid) showed no correlation with the vitamin B6 status.

Aged↗
PubMed1986

Superoxide dismutase isoenzymes in cerebrospinal fluid and plasma from patients with neuronal ceroid-lipofuscinoses.

The neuronal ceroid-lipofuscinoses is a group of diseases characterized by a widespread accumulation in the body of pigments believed to be end-products of lipid-peroxidation damaged organelles. It was recently shown that cerebrospinal fluid from patients with infantile and juvenile neuronal ceroid-lipofuscinosis were less protective against superoxide radical-induced hydroxyl radical formation compared with controls. The content of superoxide dismutase isoenzymes in cerebrospinal fluid and in plasma from patients with different forms of neuronal ceroid-lipofuscinosis was analysed. No significant difference from controls could be demonstrated in samples from patients with juvenile neuronal ceroid-lipofuscinosis. The few samples from patients with infantile and late infantile neuronal ceroid-lipofuscinosis analysed all fell within the range defined by the controls.

Adult↗
PubMed1985

Antioxidant treatment in Spielmeyer-Sjögren's disease.

The data for 125 patients with Spielmeyer-Sjögren's disease is presented. Antioxidant therapy was given to 49. 27 received a combination of vitamin E, vitamin C, methionine and BHT. As the disease began to progress, the treatment was changed to a combination of sodium selenite and vitamin E in 14 of the 27 patients. The same therapy was also given to 22 children who had not received previous antioxidant supplementation. The number of positive and negative responses was nearly equal in the 2 treatment groups. However, the quality of the response was better in the selenite group and it has been possible in some cases to stop for several years, at least, the deterioration which began during the original therapy.

Adolescent↗
PubMed1983

Iron and oxygen radicals in tissue damage: implications for the neuronal ceroid lipofuscinoses.

The neuronal ceroid lipofuscinoses (NCL) are an important group of progressive encephalopathies characterized by accumulation of autofluorescent storage material, which probably arise during the destruction of cells by lipid peroxidation and the action of oxygen radicals such as superoxide (O.2-) and the hydroxyl radical (OH.). The rates of lipid peroxidation and of superoxide-dependent hydroxyl radical formation would be greatly accelerated by the presence of non-protein-bound iron salts. Cerebrospinal fluid from patients with different types of NCL has a higher level of non-protein-bound iron and lower antioxidant activity than that of controls. The raised iron content and decreased antioxidant protection found in cerebrospinal fluids may be symptomatic of a more general abnormality in iron metabolism and protection against its damaging effects.

Antioxidants↗
PubMed1983

Occurrence of caries and periodontal disease in selenium-treated patients with Spielmeyer-Sjögren's disease.

The possible influence of continuous exposure to selenium on dental caries and gingivitis was examined during a clinical trial of selenium administration to patients suffering from a progressive encephalopathy, Spielmeyer-Sjögren's disease. The study group consisted of eight children (mean age 12.5 years) and a control group of the same age and size from the same institution. DMFS- and Gingival Bleeding Indices were recorded at the onset of the selenium medication and thereafter once a year. No difference in the caries incidence or in the prevalence of gingivitis between the two groups could be shown during the follow-up period.

Adolescent↗
PubMed1981

Superoxide dismutase, catalase and glutathione peroxidase in infantile, late infantile and juvenile neuronal ceroid-lipofuscinosis.

The neuronal ceroid-lipofuscinoses are characterized by a widespread deposit in the body of pigments believed to be end products of lipid peroxidation damaged organelles. CuZn superoxide dismutase, Mn superoxide dismutase, catalase and glutathione peroxidase, enzymes which conceivably might protect against lipid peroxidation, were investigated in blood cells from patients afflicted with infantile, late infantile and juvenile neuronal ceroid-lipofuscinosis. In some cases the enzymic activities were slightly lower than the activities of the controls, but no deficiencies which might be of etiolgical importance were revealed.

Adolescent↗
PubMed1980

Nature of lowered 5-hydroxytryptamine uptake by blood platelets of patients with Down's syndrome.

Disturbance of central serotoninergic system has been suggested in Down's syndrome (DS). In this syndrome the 5-HT concentration in blood platelets is below normal, but the mechanism behind this has been controversial. Recently, evidence has accumulated indicating a decreased active transport of 5-HT possibly due to lowered activity of Mg++-dependent Na+-K+-stimulated adenosine triphosphatase. In the present study the kinetic analysis of 5-HT uptake by blood platelets from DS patients revealed a decreased Vmax, suggesting decreased transport function but an unchanged affinity to the uptake receptors, as indicated by normal Km values. The controls were mentally retarded patients from the same institute. The uptake continued in a linear fashion up to 20 min., suggesting an unchanged storage of 5-HT. Also the effect of zinc on the 5-HT uptake was studied; plasma zinc levels have been noted to be lowered in DS infants. In vitro zinc caused a dose-dependent inhibition of uptake at 10(-5) and 10(-4) M. In vivo, after 2 weeks treatment 135 mg/day orally no significant effect was noted.

Adolescent↗