Search PubMed⌕ Search

Biomedical subjects

I Sobue

Publications and source records attributed to I Sobue.

At least 37 records · Page 2Linked to original sources
PubMed1986

Immunoglobulin abnormalities in patients with myotonic dystrophy.

In order to further investigate immunoglobulin (Ig) abnormalities in patients with myotonic dystrophy (MyD), we examined 65 patients with MyD for their serum concentration of IgG in relation to their immunological functions as well as other clinical and laboratory findings. Turnover of IgG was also examined. We found significant and selective reduction of serum IgG in patients with MyD. The serum concentration of IgG in patients with MyD had a significant negative correlation with duration of illness, which suggested that serum IgG levels in MyD decreased constantly throughout the disease course. Peripheral blood lymphocyte subpopulations, including functional T cell subsets, in vitro lymphocyte proliferation, and in vitro IgG production were normal. The plasma cell population in bone marrow was also normal. The IgG turnover study using 125I-labelled IgG as a tracer revealed an increased extravascular IgG pool, and an increased capillary permeability of intravascular IgG into the extravascular compartment, in patients with MyD. These observations suggested that the Ig abnormalities in patients with MyD were not due to a broader immunological derangements as previously postulated by other authors. The abnormal distribution of IgG is a possible factor leading to reduced serum concentration of IgG in patients with MyD.

Adolescent↗
PubMed1986

A family with beta-galactosidase deficiency: three adults with atypical clinical patterns.

Three adult patients in a single family showed severe myoclonus, ataxia, and pyramidal signs. Enzymatic analysis of lymphocytes, plasma, and cultured skin fibroblasts showed marked deficiency of beta-galactosidase activity, more profound with GM1 ganglioside than with another natural substrate, asialofetuin. Other lysosomal hydrolases were normal. Although the physical signs were similar to those of types 1 and 2 GM1 gangliosidosis, none had bony abnormalities.

Adult↗
PubMed1986

Evaluation of supra- and infratentorial brain atrophy by computerised tomography in spinocerebellar degeneration.

Measurement of various parameters of supra- and infratentorial brain atrophy in computerized tomographs of 142 cases of spinocerebellar degeneration (SCD) and 100 age & sex matched controls was carried out in order to investigate whether these parameters would correspond to the subtypes of this disease and differing grades of various clinical manifestations. One supra- and all infratentorial parameters of SCD showed statistically significant atrophy with a risk of P less than 0.005. Among the subtypes, OPCA had a more severely atrophied pons than LCCA (P less than 0.005), Menzel (P less than 0.05) and SSP (P less than 0.01). There was a correlation between the distribution of symptoms like gait, speech, ataxia of extremities and ocular movement disorders, and distribution and degree of infratentorial atrophy with statistical significance (P less than 0.05 approximately P less than 0.005). The degree of atrophy of the pons and the width of the IV ventricle were directly proportional to the duration of the illness in cases of less than 10 years, but not to those of over 10 years. Follow-up CT scan was done for 24 patients, 12 within 3 years, 12 after the lapse of 3 years. The latter group showed statistically significant atrophy between the 1st and 2nd scans in several parameters, but there was no significance between those of the former group.

Adolescent↗
PubMed1985

A microassay for acid beta-galactosidase activity toward asialofetuin.

To study the enzymatic properties of beta-galactosidase from the patients with a beta-galactosidase deficiency such as GM1 gangliosidosis, determination of enzymatic activity with naturally occurring substrates, asialofetuin in addition to another natural substrate, GM1 ganglioside, is essentially required. With a previously reported, simple and sensitive fluorometric assay for GM1 ganglioside beta-galactosidase using high performance liquid chromatography (HPLC), optimal reaction conditions were determined for the assay of acid beta-galactosidase activity toward asialofetuin in skin fibroblast homogenates. Under these conditions, reduced enzymatic activities could be detected in cultured skin fibroblasts from patients with type 1 and 3 GM1 gangliosidoses and mucopolysaccharidosis IV-B (Morquio B syndrome). This method was applicable to study of the enzymatic properties of the mutant beta-galactosidase and provided an alternative to assays employing radioactive or artificial substrates.

Asialoglycoproteins↗
PubMed1985

Concentrations of immunoreactive thyrotropin-releasing hormone in the brain of patients with olivoponto-cerebellar atrophy.

The concentrations of immunoreactive thyrotropin-releasing hormone (ir-TRH) in the brain of patients with olivoponto-cerebellar atrophy (OPCA) were studied. Three patients with OPCA and 8 non-central nervous system degenerative diseases were subjects in this study. Ir-TRH concentration in the brain was measured by radioimmunoassay. Ir-TRH was present in all parts of the dissected brain tissues (hypothalamus, frontal lobe, cerebellar cortex, olivary n., dentate n. and caudate n.) of patients with OPCA and non-central nervous system degenerative diseases. Ir-TRH concentration in the brain of case 1 and 2 was lower in the cerebellar cortex and olivary n., in contrast, ir-TRH concentration in case 3 was higher in the dentate n. The regions and the severity of pathological changes were different in each case of OPCA and changes in ir-TRH concentration in the brain did not always correlate with the severity of pathological changes. These findings suggest that changes in ir-TRH concentration in the brain of patients with OPCA may differ in each case, and may play some pathophysiological role in OPCA.

Adult↗
PubMed1985

Serum carbonic anhydrase III in progressive muscular dystrophy.

Serum carbonic anhydrase III (CA-III) levels were determined by means of an enzyme immunoassay method and compared with serum creatine kinase (CK) and muscle-specific enolase (MSE) levels in 143 patients with four types of progressive muscular dystrophy (PMD), namely, Duchenne muscular dystrophy (DMD), limb-girdle dystrophy, facioscapulohumeral dystrophy and congenital dystrophy. Serum CA-III levels were raised in the majority of patients, especially in those with DMD. In DMD patients, the gradual decline in the CA-III level was observed with age. High correlations were found between CA-III, CK and MSE levels. The frequency of cases with elevated CA-III levels was the same as or greater than that of elevated CK or MSE levels in four types of PMD. These results suggest that serum CA-III may be a useful marker of muscle disease.

Adolescent↗
PubMed1985

Effects of bromocriptine on parkinsonism. A nation-wide collaborative double-blind study.

The effects of bromocriptine in patients with Parkinson's disease manifesting various problems in levodopa therapy were tested in a double-blind manner with the collaboration of 59 institutions. The slow and low principle was in part adopted. Either bromocriptine or placebo was added to levodopa. Twenty-nine % of the bromocriptine-treated patients (n = 108), in contrast to 14.8% of the placebo-treated (n = 108), showed either marked or moderate improvement (P less than 0.05). Twenty to 37% improvement was noted in most of the symptoms studied in those treated with bromocriptine. The significant superiority of bromocriptine was also noted in the effects on wearing-off phenomena and frozen gait. No irreversible side effects were noted. It is concluded that bromocriptine is useful in patients who are manifesting various difficulties in levodopa therapy. Our results are comparable to those using higher maintenance doses. Dopamine antagonistic actions were not observed. This is unlike the case with experimental animals.

Bromocriptine↗
PubMed1985

Chronic experimental allergic neuritis (EAN) in juvenile guinea pigs: immunological comparison with acute EAN in adult guinea pigs.

In order to approach the mechanism of chronic or relapsing course in human chronic inflammatory demyelinating polyradiculoneuropathy, we established a chronic model of experimental allergic neuritis (EAN) in juvenile guinea pigs, and investigated the underlying cellular immune phenomenon in comparison with acute EAN in adult animals of the same strain. Two-week-old Hartley guinea pigs, sensitized with bovine peripheral nerve homogenate, developed chronic or relapsing EAN, whereas all adult animals developed acute monophasic EAN. Morphological examination of both the chronic and acute forms revealed scattered demyelination and mononuclear cell infiltrates which were essentially restricted to the peripheral nervous system, and indistinguishable from each other. Both the in vitro lymphocyte mitogenic response and in vivo skin testing revealed a significantly lower response to neuritogenic antigens (P2 protein and peripheral nerve myelin) in juvenile chronic EAN than in adult acute EAN throughout their respective courses. In addition, we showed, by means of assessing peripheral blood lymphocyte number and its subpopulations, that normal 2-week-old Hartley guinea pigs have not fully developed immunologically. These observations suggested that there was some immunological incompetence especially in cellular immunity in 2-week-old juvenile guinea pigs and that this might be one possible factor leading to chronic EAN.

Acute Disease↗