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Biomedical subjects

M Osame

Publications and source records attributed to M Osame.

At least 379 records · Page 21Linked to original sources
PubMed1991

Vacuolar myositis with expression of both MHC class I and class II antigens on skeletal muscle fibers.

We describe here a 10-year-old patient with high levels of serum IgE and inflammatory myopathy whose muscle fibers exhibit excessive autophagy. Previous studies have demonstrated surface expression of class I MHC antigens on muscle fibers from patients with inflammatory myopathy. The muscle fibers of this patients showed marked expression of both class I and class II MHC antigens. The reaction products were demonstrated not only on sarcolemma but also in and around some vacuoles. Both CD4-positive and CD8-positive T-lymphocytes were noted in inflammatory exudates surrounding these fibers but B-lymphocytes were rare. We hypothesize that myocyte expression of both class I and class II antigens may play a role in the pathogenesis of this new type of inflammatory myopathy.

Autoimmune Diseases↗
PubMed1991

Identification of (23S)-5 alpha-cholestane-3 alpha,7 alpha,12 alpha,23,25-pentol in urine of patients with cerebrotendinous xanthomatosis.

This paper describes the identification of a new bile alcohol possessing the 5 alpha-cholestane structure that was found in the urine of patients with cerebrotendinous xanthomatosis. The urine samples were extracted with reversed-phase resin, treated with beta-glucuronidase, and separated on silica gel and reversed-phase column chromatography. The new bile alcohol isolated was the second component of the urinary bile alcohols and was identified as (23S)-5 alpha-cholestane-3 alpha,7 alpha,12 alpha,23,25-pentol by means of gas-liquid chromatography/mass spectrometry and nuclear magnetic resonance spectroscopic studies.

Brain Diseases↗
PubMed1991

Cytotoxic and suppressor activities in patients with HTLV-I-associated myelopathy.

Since there are several immune abnormalities and autoimmune-like features in human T-cell lymphotropic virus type I (HTLV-I)-associated myelopathy/HTLV-I-associated tropical spastic paraparesis (HAM/TSP), we examined cytotoxic and suppressor cell functions in HAM/TSP. In this study, we assayed cell-mediated cytotoxicity of CD8+ T-cell lines established from cerebrospinal fluid lymphocytes of two patients with HAM and concanavalin A-induced suppressor cell activities of peripheral blood lymphocytes from five patients with HAM. Our study revealed that three of four CD8+ T-cell lines showed cytotoxic activities against autologous CD4+ T-cell lines infected with HTLV-I, and two of the three lines showed major histocompatibility complex class I-restricted cytotoxicity. We also demonstrated that the concanavalin A-induced suppressor function was not defective in HAM patients. Therefore, the immune abnormalities and autoimmune-like features observed in HAM/TSP may not result from defective cytotoxic or suppressor cell activities.

Adult↗
PubMed1991

Clinical manifestation of human T-cell lymphotropic virus type-I-associated myelopathy and vesicopathy.

Patients with human T-cell lymphotropic virus type I (HTLV-I)-associated myelopathy (HAM) sometimes have accompanying voiding disturbances. We performed clinical surveys and urodynamic examinations on 25 untreated patients with HAM. Although 4 cases (16%) were entirely aware of urinary symptoms, the onset of urinary symptoms preceded other pyramidal symptoms in 6 cases (24%). All cases suffered from dysuria. The cause of dysuria was thought mainly to be detrusor external sphincter dyssynergia, but in some cases an underactive detrusor and poor opening of the bladder neck at voiding were also the causes of dysuria. There was a tendency for urinary dysfunction to become worse as the primary disease progresses. Patients with HAM must be carefully followed up by urologists in order to prevent deterioration of the urinary tract.

Adult↗
PubMed1991

Detection of human T cell lymphotropic virus type I proviral DNA and its gene expression in synovial cells in chronic inflammatory arthropathy.

To investigate the pathogenesis of human T cell lymphotropic virus type I (HTLV-I)-associated chronic inflammatory arthropathy (HAAP), we sought to detect proviral DNA in the articular lesions. For the detection of proviral DNA, we used the polymerase chain reaction (PCR). Proviral DNA was detected not only in the peripheral blood mononuclear cells (PBMCs) and synovial fluid cells (SFCs), but also in the T lymphocyte-depleted cultured synovial cells (CSCs). These findings suggest that the infection by HTLV-I might occur in vivo in non-T cells. Furthermore, we detected HTLV-I tax1/rex1 messenger RNA in fresh synovial tissues and CSCs but not in fresh PBMCs and fresh SFCs using reverse transcription and PCR. Immunohistochemically, the CSCs from HAAP patients were also shown to express the HTLV-I antigens. These data indicate that HTLV-I in the non-T synovial cells can be transcribed and expressed. Moreover, the sequences of pXII regions in the CSCs demonstrated 97.5-99.4% homology to that in MT-2 cells, HTLV-I-infected cell line. This confirmed that the PCR-amplified bands reflect HTLV-I itself. These results suggest that this organ-specific inflammation can be attributed to non-T cell virus infection in articular lesions.

Arthritis↗
PubMed1991

Angiographic morphology of the posterior communicating artery and basilar in patients with ICA-PComA aneurysm.

The relationships between the angiographic morphology of the posterior communicating artery (PComA) and the basilar artery (BA) and saccular aneurysms at the internal carotid artery (ICA)-PComA junction were evaluated in 23 patients with ICA-PComA aneurysm and 46 controls. No significant differences were found in the height of the basilar top, the dislocation and inner diameter of the BA, and the distance between the basilar top and the ICA-PComA junction. However, the angle between the PComA and C2 portion of the ICA was larger and the PComA straighter in ICA-PComA aneurysm patients. Tension in the PComA and mechanical damage to the divergent angle of the PComA are probably important factors in the development of ICA-PComA aneurysms.

Basilar Artery↗
PubMed1991

A case of HTLV-I associated myelopathy(HAM) in Korea.

We report the first Korean case of HTLV-I associated myelopathy (HAM), which was confirmed by Western blot assay of serum and cerebrospinal fluid. Interestingly, the proband's wife was a Japanese Korean who had lived in the endemic area of HAM, Kyushu. Japan, Investigations revealed significantly elevated anti HTLV-I antibody titers in the serum, but not in the CSF, suggesting that she was a carrier of HTLV-I. Considering that the patient had not had a previous blood transfusion, the most common route of HTLV-I, it is likely that the patient was infected by his wife through sexual intercourse. Although previous reports suggest that the transmission of HTLV-I is essentially from man to man or man to woman, our case suggests that woman to man transmission also occurs.

Adult↗
PubMed1991

[Advanced stage of giant axonal neuropathy].

A woman has appeared normal during her neonatal and childhood period except for a short stature. Her parents were healthy and non-consanguineous. At the age of 8, she noticed difficulty in climbing stairs and had tendency to fall. In her 13th year, she developed marked scoliosis and genu valgum. Physical examination at 14 years of age revealed a gentle and shy child of short stature with brown-black kinky hair. Neurological examinations revealed progressive mental retardation, optic nerve atrophy, moderate and coarse nystagmus on lateral and vertical gaze, atrophic tongue with fasciculations, slow and scanning speech, distal muscular weaknesses with diffuse atrophies in the four extremities and sensory deficiencies in all modalities with a glove-stocking type distribution. At the age of 15, she was unable to walk without a wheelchair. During the course she showed slowly progressive muscular weakness, ataxia and decreasing sensation especially in the lower extremities. She died of infection of the respiratory and urinary tracts at the age of 25. Pathologically the abnormalities in the biopsied and autopsied sural nerve were characterized by an advanced stage of nerve fiber degeneration without giant axons. The phrenic nerve obtained at autopsy at 1 to 10 cm from axon terminal revealed the presence of several large focal axonal swelling of 15-20 microns in diameter. On the other hand, sections of the phrenic nerve at 15 cm from axon terminal displayed a mild to moderate reduction in the number of myelinated fibers without giant axons. The difference of pathological findings among these specimens seems to depend on the time as well as the site of the examination.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

[Symptomatic adrenoleukodystrophy heterozygote with fluctuated neurological symptoms--a case report].

We report a patient with symptomatic adrenoleukodystrophy (ALD) heterozygote, a 57-year-old female, who manifested fluctuated neurological symptoms. She is a mother of the patient with adrenoleukomyeloneuropathy associated with hypoparathyroidism and cerebral calcification. The heterozygote of ALD was diagnosed by elevated levels of very long chain fatty acids in her plasma and erythrocyte membranes. At age 51, she had a disturbance of consciousness, which subsided two months later. Neurologically, bilateral pyramidal tract signs were noted. CT revealed low-density areas in the white matter neighboring the bilateral anterior horns of lateral ventricles and small calcification in the basal ganglia. Four years later, her neurological manifestations including gait disturbance and mental deterioration subacutely progressed. MRI (T2-weighted image) showed diffuse high intensity areas in the cerebral white matter. Adrenal insufficiency, hypoparathyroidism and peripheral neuropathy were not detected. It has been reported that some ALD heterozygotes developed neurological symptoms, which resembled those of the patients with adrenomyeloneuropathy. The presented patient manifested neurological symptoms with fluctuation, which was not common clinical course in the heterozygotes of ALD. On the differential diagnosis of neurological diseases such as multiple sclerosis, cerebrovascular disease or other cerebral white matter diseases, it is important to consider the possibility of ALD heterozygotes.

Adrenoleukodystrophy↗
PubMed1991

[Chronic progressive external ophthalmoplegia (CPEO); mitochondrial DNA deletion, brain MRI and electrophysiological studies].

To evaluate the correlation of clinical symptoms and deletion of mitochondrial DNA (mtDNA) in CPEO, we examined brain MRI, lower limb SSEP and mtDNA in 19 patients (nine men, ten women) with CPEO averaging 44.9 years of age. Of these patients, three had typical Kearns-Sayre syndrome (KSS) as defined by the presence of the invariable triad of CPEO, retinitis pigmentosa and an onset before the age of 20, as well as at least one of the followings: heart conduction block, cerebellar ataxia and elevated CSF protein. One patient was diagnosed as having probable KSS because the symptoms had begun at the age of 33. All patients with typical and probable KSS had one or more of the following common manifestations: mental retardation or dementia, hearing loss, short stature, and endocrinological disorder. All other 15 patients had ocular myopathy with limb muscle weakness. Total DNA was isolated from 19 biopsied muscles, and analyzed by the methods of Southern blot hybridization and PCR. Thirteen patients has heteroplasmy with the deleted and normal mtDNA, and six patients who had ocular myopathy did not have mtDNA deletion. The age of onset in the patients with mtDNA deletion averaged 23.0 years of age, while that without mtDNA deletion averaged 39 years of age. All KSS and two ocular myopathy patients shared the common site in mtDNA deletion, while nine with ocular myopathy showed the different sizes of deletion ranging from 2.3 to 9.5 kb in the different sites. Brain MRI was obtained from 12 of the 19 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

[Juvenile parkinsonism in monozygotic twins].

A pair of monozygotic twins concordant for juvenile Parkinsonism are described. These twin sisters have lived together until 18 years old. Twin A noted tremor in the right hand and right-sided stiffness and slowness at the age 20. Initially, a marked improvement was shown with L-dopa treatment. However, one year after the beginning of treatment, dopa-induced dyskinesia appeared. Twin B noted tremor and right-sided stiffness and basal ganglia calcification in both patients. These twin patients suggest that genetic factors may play an important role in the cause of juvenile Parkinsonism.

Adult↗
PubMed1991

[Hypothyroidism with increased serum levels of cholestanol and bile alcohol--analogous symptoms to cerebrotendinous xanthomatosis].

We reported a 53-year-old woman with hypothyroidism due to ectopic thyroid gland. She showed intellectual impairment, bilateral pyramidal tract sings, slight cerebellar signs, and degenerative changes of brain white matter on CT and MRI, which were similar to symptoms and signs in cerebrotendinous xanthomatosis (CTX). We found increases of serum cholestanol in the patient and additional 3 patients with hypothyroidism. Total bile alcohol was also increased in the serum of the patients. We speculate that hypothyroidism and CTX might have a similar pathophysiological background on the development of neurological complications and atherosclerosis.

Alcohols↗
PubMed1991

[A case of scapuloperoneal atrophy with rigid spine having lobulated fibers in muscle biopsy].

The patient, a 52-year-old male, noticed abnormalities on walking at about 20 years of age, followed by slowly progressive muscle weakness of arms and neck. The family history was negative. He showed muscular atrophy and weakness with a preferential involvement of the scapular, arms and peroneal muscles. Deep tendon reflexes were absent. He had a limited range of motion in the spine, but the onset was unclear. Creatine kinase (CK) was elevated (324 IU/L) and the EMG study showed myogenic pattern. Muscle biopsy was obtained from the biceps brachii muscle; on NADH dehydrogenase stain, there was subsarcolemmal increase in the oxidative enzyme activity showing "lobulated fiber" mostly seen in type 1 fibers. On electron-microscopy, the sub-sarcoplasmic areas which had high NADH activity, contained many mitochondria and glycogen particles. However, iodine-glycogen complex spectrum analysis pattern and debranching enzyme activity were normal. CT scan revealed low density in the paravertebral muscles, suggesting degeneration. This is a rare type of scapuloperoneal atrophy different from Emery-Dreifuss syndrome, rigid spine syndrome and FSH type muscular dystrophy.

Adult↗
PubMed1991

[Nationwide survey of necrotizing myelopathy associated with malignancy in Japan].

Twenty-two cases of transverse myelopathy associated with malignancy were collected in nationwide survey in Japan. Paraneoplastic necrotizing myelopathy (PNM) was suspected in 9 of them and 8 cases were diagnosed as radiation myelopathy (RM), clinically and pathologically. Other 5 cases had different causes of transverse myelopathy. In comparison with PNM and RM, malignant lymphoma, flaccid paraplegia and sphincter dysfunction were highly associated in the patients with PNM. On the other hand, lung cancer, spastic paraplegia, abnormality in spinal MRI and dysesthesia in legs as an initial symptom were prevalent in the cases of RM. Six cases of them were compared pathologically. Although all cases had no metastasis of malignant cells, five cases of PMN showed acute and chronic necrosis and rarefaction of spinal cord with or without perivascular cuffing. One case of RM had focal spinal cord atrophy and no lymphocytes infiltration. Immunohistochemically, herpes simplex virus type 2 (HSV2) infection in spinal cord was shown in the 2 cases of the necrotizing myelopathy. Virus infection such as HSV2 could be one of causes of PNM and virological study must be done in the cases of necrotizing myelopathy associated with malignancy.

Adult↗
PubMed1991

[A study on the frequency of von Willebrand factor deficiency state].

In order to elucidate the prevalence of von Willebrand factor (vWF) deficiency state, we investigated plasma vWF levels among 343 patients with bleeding tendency, 465 patients without bleeding tendency and 704 random unrelated normals living in Kagoshima prefecture. The prevalence of symptomatic vWF deficiency state was at least 2.3 per 100,000 of the population, and that of asymptomatic one in healthy individuals was 1.3%. These results indicate that the overall incidence of vWF deficient state in general population would be considerably higher than that previously reported.

Adolescent↗
PubMed1991

[Plasma concentrations of plasminogen activator inhibitor 2 in patients with hematological malignancies and their clinical significance].

We investigated plasma levels of plasminogen activator inhibitor 2 (PAI-2) in 40 patients with hematological malignancies including acute leukemia, chronic leukemia, hemophagocytic histiocytosis (HH) and histiocytic sarcoma (HS). The plasma PAI-2 levels in the patients with mononuclear phagocyte system (MPS) proliferative disorders (M4, M5, and CMMoL on FAB classification, HH and HS) were all above the cut-off value in healthy subjects. In a patient of reactive HH, there was a close correlation between Plasma PAI-2 and serum ferritin levels during the clinical course. These results indicate that the increase of plasma PAI-2 would be a diagnostic indicator of MPS proliferative disorders.

Adult↗