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Biomedical subjects

J Ono

Publications and source records attributed to J Ono.

At least 55 records · Page 3Linked to original sources
PubMed1999

Ictal video-EEG recording of three partial seizures in a patient with the benign infantile convulsions associated with mild gastroenteritis.

PURPOSE: In infants, benign convulsions can be triggered by febrile illness or mild diarrhea such as Rotavirus gastroenteritis. The triggering mechanism of these convulsions is still unknown. In spite of several reports concerning clinical features, the ictal EEG recordings were rarely analyzed by a video-EEG monitoring system. To reveal a clue for the triggering mechanism of these convulsions, we analyzed the correlation of clinical manifestations and the EEG discharges during the ictal events and compared with previous reports. METHODS: The ictal EEG of a cluster of three afebrile convulsions associated with mild gastroenteritis was recorded by an EEG closed-circuit TV (EEG-CCTV) monitoring system in a 6-month-old healthy female infant. RESULTS: All seizures began as complex partial seizures (CPSs), which exhibited a motionless stare with or without leftward deviation of both eyes, and evolved to secondarily generalized tonic-clonic seizures (SGTCSs) for approximately 90 s. Each of three ictal discharges began from the right occipital, right centroparietotemporal, and left occipital regions, respectively. CONCLUSIONS: Although initiating sites of ictal discharges of benign infantile convulsions associated with mild gastroenteritis (BICE) were previously reported to be variable among patients, these results indicated that those differ among seizures even in a same infant.

Cerebral Cortex↗
PubMed1999

Elevated plasma levels of glucagon-like peptide-1 after oral glucose ingestion in patients with pancreatic diabetes.

OBJECTIVE: The purpose of the present study was to evaluate plasma glucagon-like peptide-1 (GLP-1) responses after oral glucose ingestion in patients with chronic pancreatitis and to clarify how GLP-1 secretion relates to pancreatic diabetes. METHODS: An oral glucose tolerance test (OGTT) was performed in 17 patients with chronic pancreatitis. Plasma glucose, immunoreactive insulin (IRI), C-peptide, glucagon, and GLP-1 levels at each time point during OGTT were measured. The diagnosis of chronic pancreatitis was made by the findings of endoscopic retrograde pancreatography (ERP): evident dilation of the main pancreatic duct with or without pancreatolithiasis. RESULTS: The patients were divided into three groups according to the World Health Organization classification of diabetes based on plasma glucose levels after OGTT. The groups were: normal (three patients), impaired glucose tolerant (IGT) (six patients), and diabetic (DM) (eight patients). In the DM group, IRI and C-peptide response levels after oral glucose ingestion were significantly reduced as compared with those of the normal and IGT groups. No significant glucagon responses to oral glucose ingestion were found in the three groups. In contrast, plasma GLP-1 levels were significantly elevated after oral glucose ingestion in the DM groups as compared with normal and IGT groups. CONCLUSIONS: The present study affords evidence that plasma GLP-1 levels become elevated with development of pancreatic diabetes, although the precise mechanism of this elevation remains undetermined.

C-Peptide↗
PubMed1999

Interstitial deletion of 14q, 46, XY, del (14) (q24.3q32.1) associated with status nonepileptic myoclonia and delayed myelination.

A Japanese boy with interstitial deletion of the long arm of chromosome 14, including band 14q31, is described. The characteristic dysmorphic facial features, such as dolichocephaly, bushy eyebrows, horizontal narrow palpebral fissures, long philtrum, etc, and mental and motor developmental delay were observed. Other characteristic clinical manifestations were anuresis and status nonepileptic myoclonia The finding of delayed myelination of the cerebral white matter was observed on magnetic resonance examination, suggesting that an unknown factor related to myelination in the central nervous system might be localized in band 14q31.

Abnormalities, Multiple↗
PubMed1999

[Leiomyoma of the lung: a case report].

An asymptomatic 43-year-old female was admitted to the Hospital because of an abnormal nodule in the left lung field on screening chest X-ray. Chest CT showed a tumor shadow mass (4 x 3 cm) in the left lower lobe. Bronchofiberscopy revealed an endobronchial polypoid mass obstructing the lower lobe bronchus at the orifice of left B10. Lt lower lobectomy was performed. Histopathological examination of the specimen showed the tumor (4.5 x 3.6 x 3.6 cm) developing not only within the tracheal lumen, but also in the lung tissue. Because immunohistochemical staining for desmin and alpha-smooth muscle actin was positive, we diagnosed leiomyoma. As MRI revealed multiple uterine masses after operation, we discussed the diagnostic problems in the relation to metastasizing leiomyoma of the uterus.

Adult↗
PubMed1999

[Connatal type of Pelizaeus-Merzbacher disease: a case report].

Pelizaeus-Merzbacher disease (PMD) is a hereditary disorder with myelin dysplasia in the central nervous system. The connatal type is a more severe form compared to the classical type and shows developmental arrest or deterioration, nystagmus, spasticity, and/or convulsions in the neonatal period. A 1 1/4-year-old Japanese boy diagnosed as connatal type PMD is reported here. Soon after his birth, he demonstrated horizontal and rotatory nystagmus and opisthotonic posture. At the age of 10 months, he had difficulty in feeding. At the age of 1 year, he presented more severe opisthotonic posture and frequent vomiting. He showed deterioration in gross motor development. His chromosome analysis showed a normal male karyotype. Electroencephalogram did not show a sleep spindle. Auditory evoked brainstem responses (ABR) showed only wave I on both sides. Visual evoked potentials (VEP) showed prolongation of latencies. These results were compatible with PMD. Nuclear magnetic resonance imaging (MRI) demonstrated in the white matter of cerebrum and brainstem no high intensities on T1-weighted images and diffuse high intensities on T2-weighted images. Such absence of myelination including the brainstem was characteristic to the connatal type PMD. The diffuse disturbance of myelination appeared to correlate with the severity of clinical symptoms.

Brain↗
PubMed1999

Proteolipid protein gene duplications causing Pelizaeus-Merzbacher disease: molecular mechanism and phenotypic manifestations.

Pelizaeus-Merzbacher disease (PMD) is an X-linked disorder characterized by dysmyelination of the central nervous system (CNS) caused by mutations involving the proteolipid protein gene (PLP). In addition to point and frameshift mutations in the coding region, duplications involving the entire PLP have been recognized recently as a major genetic abnormality causing PMD. We devised an interphase fluorescence in situ hybridization (FISH) assay to establish an efficient screening test for PLP duplication. Thirteen patients from 11 Japanese PMD families were determined to have PLP duplications. This molecular diagnostic FISH test also readily detected female carriers. Molecular analysis revealed that the size of the duplication and location of the breakpoints showed striking variation. Fiber FISH demonstrated that the duplication is tandem in nature. Haplotype analysis indicated an intrachromosomal origin for the duplication. These results suggest that an unequal sister chromatid exchange in male meiosis is likely to be the major mechanism leading to the formation of the duplication. Patients with the duplication commonly present with a mild PMD phenotype. Two patients with an exceptionally severe clinical phenotype carried large duplications, suggesting that either the larger duplicated segment incorporates additional dosage-sensitive genes or that the location of the duplication junction may affect the phenotype.

Adolescent↗
PubMed1999

[Location of primary somatosensory area in cerebral arteriovenous malformation involving sensorimotor area].

The purpose of this study was to investigate the cortical reorganization associated with congenital brain lesion such as intracerebral arteriovenous malformation (AVM). Dipole source localization of somatosensory evoked potential (SEP) was performed in five patients with AVM encompassing sensorimotor cortex. Dipole tracing method combined with the scalp-skull-brain head model (Homma et al., 1994) was used to locate dipole source of an early cortical component of SEP elicited by median nerve electrical stimulation. The location of dipole source of SEP, which could be considered as the hand area of primary somatosensory area, was shown in the realistic section of the head and could be easily superimposed on the magnetic resonance imaging. SEP was recorded three times in each patients and the results were reproducible. In 2 patients whose postcentral gyrus was not involved in AVM, the dipole source of SEP was localized in the intact postcentral gyrus. The locations of dipole sources of SEP in the both hemisphere were symmetrical. In 3 patients whose postcentral gyrus was encompassed by AVM, the dipole source of SEP was localized in the surrounding intact gyrus which was distant from the usual region of postcentral gyrus. Somatotopy was different from the normal pattern. The hand area was located more medially than usual observed in normal postcentral gyrus. Despite the paucity of the number of patients and data obtained by dipole source localization, our findings support the existence of reorganization in the cerebral cortex with congenital lesion such as AVM. These findings of aberrant mapping of cortical function may be explained by the plasticity of brain function. The developing brain can inherit function that would normally have been performed by the region of brain involved in the lesion. We demonstrated that dipole tracing of SEP was a noninvasive method used to localize areas of eloquent cortex in patients harboring AVM. This method is of value in treatment planning.

Adolescent↗
PubMed1999

Proton MR spectroscopy of Sjögren-Larsson's syndrome.

We performed single-voxel proton MR spectroscopy (1H-MRS) in two children with Sjögren-Larsson's syndrome (SLS). Both patients showed two abnormal spectral peaks at 1.3 ppm and 0.9 ppm that were obtained with short echo times. These two abnormal spectral peaks were seen in high-intensity areas on T2-weighted images and also in basal ganglia of normal intensities. 1H-MRS may be useful for establishing the diagnosis and investigating the natural history of SLS, and for evaluating the efficacy of therapeutic approaches to SLS.

Aldehyde Oxidoreductases↗
PubMed1998

The effect of volatile anesthetics on light-induced phosphorylation in spinach chloroplasts.

(1) The light-induced phosphorylation in spinach chloroplast is coupled with proton transfer. (2) We investigated the effect of volatile anesthetics (halothane, enflurane, isoflurane) on the cyclic and/or non-cyclic light-induced phosphorylation. (3) We used potassium ferricyanide as a Hill oxidant for non-cyclic phosphorylation and phenazin-methosulfate as an oxidation-reduction indicator for cyclic phosphorylation. (4) These three anesthetics inhibit the Hill reaction in light induced phosphorylation. The inhibition rate of this reaction generates concave curves with minimum values at 303 K in each of the anesthetics.

Anesthetics, Inhalation↗
PubMed1998

Characteristic MR features of encephalitis caused by Epstein-Barr virus: a case report.

An 8-year-old girl showed symptoms of encephalitis during acute Epstein-Barr virus (EBV) infection. The diagnosis of EB virus infection was made by changes in the titres of EB virus-specific antibody. Cranial MRI demonstrated abnormal low and high signal intensities in the striatal body (putamen and caudate nucleus) on T1-weighted and T2-weighted images, respectively, during the acute phase. These abnormal findings had almost completely resolved 1 month later. EBV infection should be considered when lesions are localised to the basal ganglia.

Caudate Nucleus↗
PubMed1998

Molecular cloning of the chromosomal breakpoint in the LIS1 gene of a patient with isolated lissencephaly and balanced t(8;17).

Karyotypic analysis of a patient exhibiting a phenotype of isolated lissencephaly, and of her parents, revealed a de novo balanced translocation, t(8;17)(p11.2; p13.3). Since the lissencephaly (LIS1) gene was known to be located on 17p13.3, we investigated whether the translocation might involve this gene. We performed Southern analysis using cosmid clones that contained genomic sequences corresponding to LIS1, and found that the breakpoint was located within intron 1. As sequence analysis of the parental chromosomes in the vicinity of the breakpoint identified no additional putative transcripts, haploinsufficiency of the LIS1 gene is likely to be solely responsible for the patient's lissencephaly. Characterization of both breakpoints indicated a possible involvement of repetitive sequences in the recombigenic process that led to the translocation.

1-Alkyl-2-acetylglycerophosphocholine Esterase↗
PubMed1998

Alteration of the LIS1 gene in Japanese patients with isolated lissencephaly sequence or Miller-Dieker syndrome.

LIS1 is a genetic entity that is responsible for lissencephaly. Previously we have reported isolated lissencephaly sequence (ILS) in a Japanese patient carrying a balanced chromosomal translocation that disrupted the LIS1 gene. We examined mutations of LIS1 in 12 additional Japanese patients, 8 of them with ILS and 4 with Miller-Dieker syndrome (MDS). Fluorescence in situ hybridization (FISH) analysis disclosed deletions of part of the LIS1 gene or of the chromosomal region surrounding it in three of the ILS cases and in three of the MDS cases. In one of the remaining five ILS cases, SSCP analysis and subsequent sequence analysis identified a 1-bp deletion in exon IV, which can be expected to result in premature termination of the gene product. Our results indicate that in Japan, as elsewhere, abnormality of the LIS1 gene is a common cause of MDS/ILS.

1-Alkyl-2-acetylglycerophosphocholine Esterase↗
PubMed1998

Retinoic acids induce growth inhibition and apoptosis in adult T-cell leukemia (ATL) cell lines.

Adult T-cell leukemia (ATL) is a peripheral T-cell neoplasm caused by human T-cell leukemia virus type I (HTLV-I). Despite the administration of combined intensive chemotherapy, the reported survival time of patients with acute and lymphoma types of ATL is less than 10 months. We therefore examine the effects of all-trans-retinoic acid (ATRA), 9-cis-RA and 13-cis-RA and tried to elucidate the mechanisms of inducing growth inhibition and apoptosis by these RAs using four ATL cell lines established in our laboratory. All the investigated RAs inhibited cell growth and the cells were arrested at the G1 phase. Apoptosis was induced in three out of four cell lines. Among the growth regulatory proteins examined, the level of p21Waf1/Cip1 protein was found to increase after RA treatment, thus resulting in pRb hypophosphorylation which also induced the arrest of the cells at the G1 phase. In addition, the p53 level decreased at the same time. Fas-FasL system and the downregulation of CD25 (IL-2R/alpha) expression did not seem to be involved. Based on these findings, the ability of RAs to induce a remission of ATL is thus strongly suggested.

Adult↗
PubMed1998

Isolated lissencephaly sequence with balanced chromosome translocation involving 17p13.3.

We describe a patient with isolated lissencephaly sequence (ILS) who had a de novo balanced translocation with breakpoint at 8p11.23 and 17p13.3. She developed infantile spasms and had severe developmental delay. There was no apparent deletion of 17p13.3 on fluorescence in situ hybridization (FISH) analysis. The breakpoint was located centromeric to the Miller-Dieker syndrome (MDS) marker (D17S379), and telomeric to the marker D17S1566, which is located centromeric to the LIS1 gene. This is the second reported case of ILS with balanced translocation. It is suspected that the breakpoint of 17p13.3 in this patient is located in the responsible gene for ILS.

Brain↗
PubMed1998

Beta2-glycoprotein I-dependent and -independent anticardiolipin antibody in non-obese diabetic (NOD) mice.

In the present study we investigate whether or not anticardiolipin antibody (aCL) is produced in NOD mice, which is a representative animal model of insulin-dependent diabetes mellitus (IDDM). We found that IgG class aCL appeared in 6.9% of non-diabetic NOD mice at weeks 5-15. The rates increased with age to 31.6% at weeks 16-25 and 71.9% at weeks 26-35. In addition, the titre and incidence of aCL were higher in diabetic mice than in non-diabetic mice. It was also found that aCL in NOD mice involved beta2-glycoprotein I (beta2-GPI)-dependent and -independent aCL, when beta2-GPI was added to the aCL assay system. The IgG subclass of both beta2-GPI-dependent and -independent aCL belonged exclusively to IgG2a. In addition, immunohistochemical studies revealed the predominant accumulation of IgG2a- or IgG3-positive B lymphocytes within insulitis. These results suggest that the autoimmunity in NOD mice may thus be associated with Th1 predominant immunological response. In conclusion, aCL with multiple antigenic specificity were produced in NOD mice along with the development of insulitis and diabetes. NOD mice should thus be added to the list of animal models possessing antiphospholipid antibody.

Animals↗
PubMed1998

Enhanced response of growth hormone to growth hormone-releasing hormone and a decreased content of hypothalamic somatostatin in a stress-induced rat model of depression.

This study was designed to evaluate changes in the hypothalamic somatostatin-growth hormone axis (SRIF-GH axis) in a stress-induced rat model of depression. We exposed male Wistar rats to intermittent walking stress for two weeks, and then measured their spontaneous running activities for 12 days. We divided the rats into the depression-model group and the partial recovery group according to their spontaneous running activities after the termination of exposure to stress. We examined the secretion of GH from the anterior pituitary by injecting human GH-releasing hormone (hGHRH) with intracardiac cannulae or by applying hGHRH or SRIF to isolated anterior pituitaries using a perifusion system. We also determined SRIF content in the stalk-median eminence (SME) and the plasma concentration of GH. In the depression-model group, intracardiac administration of hGHRH caused the enhanced release of GH into plasma, while application of hGHRH or SRIF to the anterior pituitary in vitro had similar effects on GH release in the control and partial recovery groups. Furthermore, the SRIF content was decreased in the SME and the GH concentration was increased in plasma. The partial recovery group gave similar values to the control group. The enhanced response of GH to hGHRH in the depression-model group might have been caused by the reduced content of SRIF in the SME in view of the unchanged response of GH to the infusion of hGHRH or SRIF in the perifusion system.

Animals↗