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Biomedical subjects

I Goto

Publications and source records attributed to I Goto.

At least 127 records · Page 7Linked to original sources
PubMed1991

A multi-center, double-blind study on slow-release bromocriptine in the treatment of Parkinson's disease.

We report on the clinical efficacy of a slow-release formulation of bromocriptine studied in a multi-center, double-blind trial using standard bromocriptine as the control. We randomly allocated enrolled patients (N = 243) to either the slow-release or normal bromocriptine group. Sixty of them were de novo patients. The maintenance dose of slow-release bromocriptine was 14.2 +/- 0.7 mg/d and that of standard bromocriptine 13.5 +/- 0.7 mg/d (mean +/- SE). The slow-release formulation was taken twice and the standard three times a day. Forty-one percent of the patients treated with the slow-release bromocriptine and 32% of the patients treated with the standard bromocriptine showed moderate or marked improvement in the global improvement rating. There were no serious side effects, and the frequency of vomiting and epigastric discomfort was lower in the patients treated with the slow-release bromocriptine. Clinical efficacies for tremor, rigidity, akinesia, and gait disturbance were comparable between the two drugs tested. The slow-release bromocriptine seems to be a valuable drug for the treatment of Parkinson's disease with less severe side effects than regular bromocriptine.

Aged↗
PubMed1991

Cryoglobulinemia in a horse.

Cryoglobulin was isolated from a horse which had glomerulo-nephritis and a history of swelling and skin ulcers of the limbs in the winter. The isolated cryoglobulin showed a single peak on a gel permeation chromatography column with an apparent molecular mass (Mr) of 180,000 which could be divided into two gamma bands by cellulose acetate electrophoresis. Immunoelectrophoretic analysis revealed that the cryoglobulin formed two precipitation lines with anti-horse IgG. Spur formation was observed when the cryoglobulin and the IgG purified from a normal healthy horse were cross-reacted with anti-horse IgG on a double diffusion gel. In addition, sodium dodecyl sulfate-polyacrylamide gel electrophoresis under the reduced conditions showed that the isolated cryoglobulin consisted of two doublets of polypeptides with Mr values of 52,000 and 50,000, and 31,000, and 30,000, corresponding to the heavy chain and the light chain of the horse IgG molecules, respectively. These results suggest that the isolated cryoglobulin might consist of two different IgG molecules, and that the manifestations such as foot swelling with skin ulcers and renal failures were all induced by the cryoglobulin in the serum.

Animals↗
PubMed1991

[A case of adult-onset nemaline myopathy (adult-onset rod disease) with distal muscular hypertrophy].

We reported a 40-year-old male with adult-onset nemaline myopathy (adult-onset rod disease) showing muscular hypertrophy of distal limbs. At the age of 25, he noticed thinness of his thighs. Difficulty in climbing stairs slowly progressed from the age of 35. On admission neurological examination revealed muscular weakness and atrophy of proximal limbs and hypertrophy of distal flexors. Normal laboratory tests included serum creatine kinase, myoglobin, aldolase and pyruvate. Electromyography revealed severe neurogenic changes in the right biceps brachial muscle and the right quadriceps muscle, and moderate changes in the right gastrocnemius. Biopsy specimen of the deltoid muscle demonstrated type 1 fiber predominance and type 1 fiber atrophy, and there was small group atrophy and type grouping. Abundant nemaline rods were found mainly in type 1 fibers (81.5%). In order to evaluate hypertrophy of calf muscles, T1-weighted MRI of lower extremity was performed. While transaxial images through mid thigh showed moderate fatty replacement, increased volume and little fatty replacement were found in the mid calf. Therefore, hypertrophy of the calf muscle seemed to be compensative hypertrophy. But in this case neurogenic factors were indicated electromyographically and histologically. These findings may advocate the notion that neurogenic factors involved not only congenital but adult-onset rod disease.

Adult↗
PubMed1991

[A case of mixed connective tissue disease with subacute transverse myelopathy].

A rare case of mixed connective tissue disease (MCTD) with subacute transverse myelopathy and various neurological signs was reported. The patient, a 53 year-old woman, was admitted to our hospital with subacute progressive muscle weakness of left lower limb and sensory disturbance of bilateral lower extremities. At the age of 40, she suffered from sensory disturbance of her face, which improved in about three years. She had a high fever, Raynaud phenomenon, dyshydrosis on right side of her face at the age of 43. On the admission, physical examination revealed swollen fingers and telangiectasia of her face. Neurologically, she had transverse myelopathy at the level of Th6, bilateral trigeminal neuropathy, tonic, pupils, polyneuropathy and dyshydrosis. Laboratory examination showed positive antinuclear antibody, a high titer of antibody to RNase-sensitive components of extractable nuclear antigen, positive antinuclear RNP antibody and negative anti-Sm antibody. Her myelopathy improved with corticosteroid therapy, and ESR and the level of immunoglobulin were normalized. But, other neurological signs showed no improvement.

Cranial Nerve Diseases↗
PubMed1991

[Exercise-induced myalgia and high CKemia with a deletion in the dystrophin gene].

A 30-year-old man showed exercise-induced myalgia, calf muscle hypertrophy and high serum creatine kinase level, without muscular weakness. The symptoms began in childhood and did not progress. Electromyographic findings were consistent with myopathy, and the muscle histology showed nonspecific myopathic changes without evidence of storage of glycogen or lipid. Immunohistochemical staining with antibodies raised against three different dystrophin peptides revealed proper subcellular localization of dystrophin at the sarcolemma of all myofibers, but the intensity of the stain was decreased. Western blot analysis using the same antibodies revealed normal dystrophin in size, but showed the reduced amount of the protein. The DNA obtained from the patient's peripheral leukocyte showed a deletion of the dystrophin gene including exon 45 by using PCR technique. Though the detailed size and portion of the deleted gene are not ascertained, the deletion in this case may not severely affect the function of dystrophin, unlike cases of Duchenne or Becker muscular dystrophy. Dystrophin analysis is useful for the etiologic diagnosis in cases of myalgia and high CKemia.

Adult↗
PubMed1990

Increase in differentiated type of T lineage cells in the myasthenic thymus: two-color fluorocytometric analysis.

We made use of two-color flow cytometry to examine thymic lymphoid cells from patients with myasthenia gravis. The CD4+CD8- cells and the CD4-CD8+ cells were significantly increased in the thymus from the patients, compared with findings in the control samples. Conversely, the CD4+CD8+ cells were significantly decreased. Significant increases in CD1-CD3+ cells and significant decreases in CD1+CD3- cells were noted. The percentage of CD45R+ cells also increased. There were no increases in activated T lymphocytes, defined as CD4+DR+ cells, CD8+DR+ cells, or IL2R+ cells. We conclude that the proportion of T lineage lymphocytes that are differentiated is increased in the thymus of patients with myasthenia gravis, and could reflect accelerated differentiation.

Adolescent↗
PubMed1990

Nationwide survey of HTLV-I-associated myelopathy in Japan: association with blood transfusion.

To study the epidemiology of human T-cell lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in Japan, we conducted two nationwide surveys between October 1986 and March 1989. A total of 710 patients with HAM (definite HAM, 589; probable HAM, 121) were reported. Of the 589 patients with definite HAM, 69% were residents of the areas with the highest prevalence HTLV-I in Japan. To determine the importance of blood transfusion in the pathogenesis of HAM/TSP, we performed a case-control study in the Kagoshima district in southern Japan. Significantly more patients with HAM reported a history of blood transfusion (26/129, or 20%) than did subjects in a health survey of the general population (41/1,290, or 3%; odds ratio = 7.7, p less than 0.001) or than did hospitalized neurological patients (6/119, or 5%; odds ratio = 4.8, p less than 0.001). Furthermore, the cumulative percentages of the intervals between blood transfusion and the onset of the symptoms of HAM fit a lognormal curve, suggesting that transfusion was an important common exposure. Blood transfusion probably transmitted HTLV-I to the patients with transfusion-associated HAM because there was a significant decrease in the number of patients with the transfusion-associated HAM who received blood after implementation of nationwide screening of blood donors in 1986 (p = 0.004). In the first 2 years, screening the blood supply in Japan appears to have decreased the number of reported patients with HAM by 16%.

Humans↗
PubMed1990

Accumulation of galactosylsphingosine (psychosine) does not interfere with phosphorylation and methylation of myelin basic protein in the twitcher mouse.

In attempts to elucidate mechanisms of demyelination in the twitcher mouse (Twi), phosphorylation and methylation of myelin basic protein (MBP) were examined in the brainstem and spinal cord of this species. Phosphorylation of MBP in isolated myelin by an endogenous kinase and an exogenous [32P]ATP was not impaired and protein kinase C activity in the brain cytosol was not reduced. When the methylation of an arginine residue of MBP was examined in slices of the brainstem and spinal cord, using [3H]methionine as a donor of the methyl groups, no difference was found between Twi and the controls. Radioactivity of the [3H] methionine residue of MBP of Twi was also similar to that of the controls. Thus, accumulation of psychosine in Twi does not interfere with the activity of endogenous kinase, methylation of MBP, and the synthesis and transport of MBP into myelin membrane.

Adenosine Triphosphate↗
PubMed1990

Generation of CD4+ blastoid T cells in recipients of BP/IFA-sensitized spleen cells.

While sensitization of Lewis rats with BP/IFA does not induce EAE, recipients given BP/IFA-sensitized cells after culture with BP do develop EAE. To clarify the pathophysiology responsible for this discrepancy, cell dynamics were studied in the recipients. The CD4+ T cells from BP/IFA-sensitized donors showed no proliferative response to BP and no change in cell size or surface molecule expression, even in the presence of BP in culture. On the other hand, the recipient cells proliferated vigorously, regardless of the presence or absence of BP. In this case, a large number of CD4+ blastoid T cells was generated only in the presence of BP in culture. Such cells showed marked upregulation of CD4, CD2, class I and II MHC, and IL2 receptor molecules, a finding we also observed in the case of BP-cultured cells from BP/CFA-sensitized rats with severe EAE. The proportion of CD4+ blastoid T cells generated after culture depended on the number of cells transferred and on the presence of BP in culture, and closely correlated with the severity of EAE in the recipients. These data suggest that BP/IFA-sensitization can also induce BP-reactive cells capable of becoming CD4+ blastoid T cells with marked upregulation of CD4, CD2, class I and II MHC, and IL2 receptor molecules directly related to potent encephalitogenicity, in vivo.

Adjuvants, Immunologic↗
PubMed1990

Juvenile Sandhoff disease: a Japanese patient carrying a mutation identical to that found earlier in a Canadian patient.

A 35-year-old Japanese man with juvenile Sandhoff disease is described. He showed progressive neurogenic muscular atrophy, cerebellar ataxia and mental deterioration, beginning at age 10 years. The accumulation of GM2 ganglioside in the submucosal nerve cell was confirmed by positive immunostaining using anti-GM2 ganglioside antibody. Biochemical evaluation revealed nearly absent beta-hexosaminidase A and B activities in leukocytes and cultured fibroblasts. Hydrolysis of [3H]globoside I in the intact fibroblasts was apparently disturbed but the rate of hydrolysis was higher than those seen in cells from patients with infantile Sandhoff disease. Analysis of the beta-hexosaminidase beta-subunit gene of the patient disclosed a point mutation (a G-to-A transition) within intron 12. The mutation generates a new splice junction resulting in a 24-base insertion between exons 12 and 13 in the processed mRNA and consequently an 8-amino acid insertion in the translation product. This mutation is identical to that originally found in a Canadian patient with juvenile Sandhoff disease. A possible relationship with the clinical phenotype and the gene abnormality is discussed.

Adult↗
PubMed1990

Lysosulfatide (sulfogalactosylsphingosine) accumulation in tissues from patients with metachromatic leukodystrophy.

We describe here a sensitive assay method for lysosulfatide (sulfogalactosylsphingosine) in human tissues using HPLC. The method involves extraction of lipids, saponification, isolation using a C18 Sep-Pak column, derivatization with o-phthalaldehyde, and detection of the fluorescent lysosulfatide using HPLC. In control subjects, a small amount of lysosulfatide was detected in the cerebral white matter (9-35 pmol/mg of protein), spinal cord (35 pmol/mg of protein), sciatic nerve (14 pmol/mg of protein), and kidney (approximately 2 pmol/mg of protein) but not in the cerebral gray matter and liver. A marked accumulation of the lipid was noted in tissues from six patients with metachromatic leukodystrophy (MLD). The concentration of lysosulfatide was high in the cerebral white matter, spinal cord, and sciatic nerve (223-1,172 pmol/mg of protein). Even in the cerebral gray matter, kidney, and liver, where lysosulfatide was hardly detected in the control sample, a considerable amount (3-45 pmol/mg of protein) accumulated in MLD patients. The concentration and distribution pattern of lysosulfatide were similar to those of galactosylsphingosine (psychosine) accumulated in patients with Krabbe disease. Therefore, the accumulation of lysosulfatide may explain the demyelination in patients with MLD, as is the case with Krabbe disease.

Cerebroside-Sulfatase↗
PubMed1990

[An autopsied case of infective endocarditis with cardiac tamponade due to myocardial rupture].

Since it is very rare that cardiac tamponade due to myocardial rupture caused by infective endocarditis, occurs we are reporting this case. A 62 year old man, who had underlying diseases of pneumoconiosis and hypertensive heart disease, visited Chikuho Rosai Hospital complaining of chest oppression and general fatigue on Feb. 7, 1987. He was diagnosed as having ischemic heart disease by electrocardiogram. Two days later, he suddenly had chills and a fever, and the laboratory data showed leukocytosis and a positive C-reactive protein (CRP). The echo cardiogram showed mitral regurgitation (MR) and aortic regurgitation (AR), but neither vegetation nor pericardial effusion was observed. On Feb. 16, he was admitted with shock, and he died the next day. The blood cultures grew gram-positive cocci, respectively. From the clinical symptoms, chest roentgenogram and electrocardiogram, we suspected a cardiac tamponade. On autopsy findings, though coronary arteries were intact, the aortic valves had severe valvular adhesions, calcifications and hypertrophies. The rupture hole was observed in the left ventricles, which was just under the aortic valve through the pericardiac space. It seemed that he died of a cardiac tamponade due to the outflow of blood from this hole. On histopathologic findings of the cardiac wall, gram-positive cocci and many of neutrophils were observed.

Cardiac Tamponade↗
PubMed1990

Abnormality in the cavernous sinus in three patients with Tolosa-Hunt syndrome: MRI and CT findings.

Three patients with Tolosa-Hunt syndrome (THS) were examined using computed tomography (CT), orbital venography and magnetic resonance imaging (MRI). CT of the brain showed no definite abnormality in two patients, right optic nerve enlargement and an abnormal area around the orbital apex in one patient. Orbital venography showed an occlusion of the superior ophthalmic vein in one of three patients. MRI showed an abnormal soft tissue area in the cavernous sinus, with intermediate to high signal intensity on T1- or intermediate weighted images. There was clinical improvement following corticosteroid therapy and the abnormal soft tissue on MRI decreased in volume and in signal intensity, although this was equivocal in one patient. Thus MRI proved to be useful in demonstrating lesions in the cavernous sinus in patients with THS.

Adrenal Cortex Hormones↗
PubMed1990

Third interim report of the nationwide collaborative study on the long-term effects of bromocriptine in the treatment of parkinsonian patients.

The interim results obtained up to the end of the 3rd year of the multicenter nationwide cooperative study on the long-term effects of bromocriptine in parkinsonian patients are reported. Bromocriptine monotherapy could be continued in approximately 33% of the patients at the mean maintenance dose of 11.4 mg/day. The combined use of bromocriptine (11.1 mg/day) in parkinsonian patients already treated with levodopa had a favorable influence on the long-term side effects of levodopa such as the on-off phenomenon and dyskinesia. The beneficial effects of bromocriptine, levodopa and an early combination on parkinsonian symptoms such as rigidity and tremor remained at the end of the 3rd year. However, the effects of each mode of therapy on another parkinsonian symptom, akinesia, ceased by the end of the 3rd year.

Adult↗
PubMed1990

[Mitochondrial encephalomyopathy (focal cytochrome c oxidase deficiency) with transient episodes of muscle weakness and elevation of serum creatine kinase activity].

A 17-year-old boy who had mitochondrial encephalomyopathy with focal deficiency of cytochrome c oxidase (CCO) activity is described. He experienced 3 episodes of muscle weakness, fatigability, nausea, vomiting and concomitant increase of serum creatine kinase activity, at the age of 13, 15 and 17 years. During interval there was no muscle weakness and the serum creatine kinase activity was within normal range. Increased levels of lactic acid and pyruvic acid were observed in the blood and cerebrospinal fluid. After an aerobic exercise test, lactic acid and pyruvic acid in the blood increased to an abnormally high level, and the arterial blood became acidic (pH 7.297). On EEG, occasional intermittent irregular theta activities were observed in the anterior region, but there were no abnormalities on CT and MRI in the central nervous system. In the biopsied muscle, ragged-red fibers comprised 20% on modified Gomori-trichrome staining and a number of fibers with no CCO activity were scattered throughout. The CCO activity in the mitochondria isolated from the biopsied muscle was reduced to 49.2 nmol/min/mg protein (normal range 144.7-355.8), while other mitochondrial enzyme activities in the electron transport system were normal. From these data, the patient was considered to have a unique form of mitochondrial encephalomyopathy. By the administration of a large amount of coenzyme Q10, episodes of muscle weakness and nausea, and an increase of lactic acid and pyruvic acid in the blood after aerobic exercise test were no longer observed.

Adolescent↗