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Biomedical subjects

J Palo

Publications and source records attributed to J Palo.

At least 145 records · Page 8Linked to original sources
PubMed1976

Selenium, vitamin E and copper in multiple sclerosis.

There has been accumulation of the nutritional muscular dystrophy of the cattle in a certain western district of Finland where the prevalence of multiple sclerosis (MS) is also highest. This animal disease is due to lack of selenium (Se) and vitamin E. The Se content of whole blood was low (52.6 +/- 11.3 ng/ml) in MS patients from this high-risk area compared to the controls (68.8 +/- 11.0). The data for serum failed to confirm this tendency. All Se values appeared to be lower than international values suggested. The values for both vitamin E and copper were within the international normal range.

Copper↗
PubMed1976

Recent findings on some "new" neurometabolic diseases.

The most common inborn error of glycoprotein catabolism appears to be aspartylglycosaminuria (AGU). It is characterized by deepening mental retardation, progressive lesions of connective tissue, and increased urinary excretion of aspartyglycosylamine. The first symptoms usually appear after 3 years of age and closely resemble those of Hurler's disease. The condition is a hereditary lysosomal storage disease due to a defective enzyme. The main clinical findings in a infantile type of neuronal ceroid-lipofuscinosis (INCL) are psychomotor retardation, visual failure, and a virtually isoelectric E.E.G. at the final stage of the disease. The symptoms of this hereditary disorder first appear between 8 and 18 months of age and the mean age at death is 6.5 years. Striking cerebral and cerebellar atrophy, together with neuronal loss and accumulation of lipofuscin-like material, can be observed at neuropathological examination.

Amidohydrolases↗
PubMed1975

Aspartylglycosaminuria: a generalized storage disease. Morphological and histochemical studies.

Aspartylglycosaminuria (AGU) is a hereditary metabolic disorder characterized by slowly progressive mental deterioration from infancy, urinary excretion of large amounts of aspartylglycosamine, and decreased activity of the lysosomal enzyme aspartylglcosamine amido hydrolase in various body tissues and fluids. The nature and distribution of the morphological and histochemical alterations in AUG are described in the light of the first AGU patient investigated post mortem and brain and liver. Most nerve cells and hepatocytes contained large vacuoles without any histochemically demonstrable lipid or carbohydrate material. Ultrastructural studies revealed numerous electron-lucent vaculoles, limited by a single, membrane, in the cytoplasm of these cells. In addition to evenly disperesed finely granular or reticular material the vacuoles contained small electron-opaque "lipid" droplets and polymorphic membraneous or granular aggregates. Similar vacuoles were also seen in a number of other cell types, particularly in the kupffer cells and brain macrophages, as well as in the capillary pericytes. Biochemical studies suggest that the principal storage material consists of aspartylglycosamine itself; glycoasparagines of higher molecular weight are present as only minor components. Correlated morphological and biochemical studies thus definitely establish that AGU is a generalized storage disorder. The condition is apparently due to decreased activity of aspartylglycosamine amido hydrolase, with accumulation of products of flycoprotein carabolism in cytoplasmic vacuoles in both epithelial and mesenchymal cells.

Adult↗
PubMed1975

Studies on the clustering of multiple sclerosis in Finland I: Comparison between the domiciles and places of birth in selected subpopulations.

Further studies pm yjr rofr,op;phu pg ,i;yo;r dv;rtpdod (MS) IN Finland were carried out in three different types of geographical unit: in counties, combined clerical districts, and single clerical district. For longitudinal studies, the prevalence of MS by present domicile was compared to that by place of birth, and also to the number of MS cases in relation to the number of births. The analysis was based on 1,866 living MS patients. The highest prevalence by present domicile was recorded in the southwestern county of Turku and Pori (52.3 cases per 100,000 inhabitants). It was 39.6 for the whole country. Two separate clusters were found at the level of combined and single clerical districts: one in the western county of Vaasa, and another in the southwestern county of Turku and Pori. The highest prevalences by present comicile were found in two clerical districts of Vaasa (83.5 and 79.7). They also had very high prevalences by place of birth. They lie close to each other, but are not immediate neighbours. Another high-risk focus was revealed in an area of the neighbouring districts of Turku and Pori. In fact, the two highest prevalences by place of birth were found in this region (123.1 and 95.0). The focus extended from the coastal area to the more central region of the county, and further to the western districts of the otherwise medium-risk county of Hme. Thus, two separate foci were found in Finland: one in the western and another in the southwestern part of the country. The clustering became even more pronounced when the prevalences by place of birth were analysed. Considering that the Finnish population still largely originates from genetic isolates of varying degrees, gene enrichment explains the clustering at least partly.

Adult↗
PubMed1975

Cerebral aspergillosis with special reference to cerebrospinal fluid findings.

A case of cerebral aspergillosis in a 69-year-old diabetic man is reported. The disease, initially presenting as headache and vertigo, was caused by Aspergillus fumigatus. Seven CSF specimens were examined. The main findings included decreased glucose and increased protein concentrations, leukocyte reaction and one positive culture for A. fumigatus. Neuropathological examination revealed granulomatous leptomeningitis and typical fungal hyphae. The process probably lasted for more than 1 year and was an important contributing factor to two brain stem infarcts.

Aged↗
PubMed1974

Aspartylglycosaminuria: a gargoyle-like syndrome with autosomal recessive inheritance.

Aspartylglycosaminuria is an autosomal recessive disorder of glycoprotein catabolism, characterized by presence of aspartyglycosamine in the urine, progressive mental retardation, coarse face, impaired speech and motor functions, and signs of involvement of connective tissue and skeleton. In infancy, clinical symptoms are mild or absent. Vacuolized lymphocytes are often found in the blood and bone marrow. The disease appears unusually common in Finland.

Adolescent↗