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Biomedical subjects

T Kunieda

Publications and source records attributed to T Kunieda.

At least 91 records · Page 5Linked to original sources

Scalp-recorded, ictal focal DC shift in a patient with tonic seizure.

PURPOSE: We recorded focal ictal DC shifts from scalp electrodes in a 9-year-old boy with intractable, clinically generalized tonic seizures. The patient had a high intensity signal abnormality of the left temporal cortex with thickening of the gyri on T2-weighted MRI. METHODS: Scalp digital EEGs were recorded using electrodes made of silver/silver chloride. The low frequency filter (LFF) was set at 0.016 Hz. Recorded seizures were subsequently analyzed with LFF settings of 1.0, 0.016 and 0.03 Hz. RESULTS: All recorded seizures initially showed diffuse, low voltage, high frequency activity (electrodecremental pattern) followed 10-20 s later by quasirhythmic activity over the left frontotemporal region. In two seizures, LFF of 0.016-0.03 Hz revealed a slow negative shift over the left frontotemporal area simultaneously with onset of the bilateral electrodecremental pattern. However, in the other seizures, this initial slow negative shift was obscured by artifacts. Subsequent electrocorticography (ECoG) delineated frequent epileptiform discharges in the left temporal as well as frontal cortex. CONCLUSIONS: Scalp-recorded ictal DC shifts may help identify focal epileptogenic brain area in patients with clinically generalized seizures although the technique is vulnerable to artifact.

Child↗

Subdural recording of Bereitschaftspotential is useful for functional mapping of the epileptogenic motor area: a case report.

A 26-year-old man with intractable focal motor seizure beginning with tonic contraction of the left orbicularis oculi muscle had prolonged EEG monitoring with subdural grid electrodes placed over the right perirolandic cortex. Electrical stimulation of the cortex with implanted subdural electrodes showed a relatively low threshold for afterdischarges (ADs) but could not disclose the motor area for the left upper face where or near where the epileptogenic area was expected to be present. Bereitschaftspotential recorded from the subdural electrodes in association with self-paced voluntary blink (eyelid closing) disclosed the motor area specifically related to voluntary movements of the left upper face, which was most likely buried in the sulcus. This observation suggests that recording of Bereitschaftspotential from subdural electrodes is useful for mapping the motor cortex, especially in patients with focal motor seizure with low threshold for ADs to electric stimuli.

Adult↗

Human second somatosensory area: subdural and magnetoencephalographic recording of somatosensory evoked responses.

OBJECTIVE: To investigate somesthetic functions of the perisylvian cortex. METHODS: Somatosensory evoked magnetic fields (SEFs) and somatosensory evoked potentials (SEPs) of the perisylvian cortex were recorded directly from subdural electrodes in a patient with a left frontal brain tumour. RESULTS: The most prominent SEP components after electrical stimulation of the right and left hands and the right foot were double peaked negativity recorded just above the sylvian fissure (latency 80 to 150 ms), respectively (N1a and N1b). Generator sources for the magnetoencephalographic counterparts of those peaks (N1a(m) and N1b(m)) were both localised at the upper bank of the sylvian fissure, and those of N1a(m) were more anteromedially located than those of N1b(m). CONCLUSIONS: These findings suggest the existence of at least two separate somatosensory areas within the human perisylvian cortex.

Adult↗

Crescentic glomerulonephritis and elevated antimyeloperoxidase antibody in a patient with Churg-Strauss syndrome.

A 45-year-old man with a long history of bronchial asthma was admitted to the National Cardiovascular Center with complaints of severe pain, swelling, and ecchymoses of the lower extremities. A diagnosis of Churg-Strauss syndrome was made because of marked eosinophilia, vasculitis, and a history of asthma. Urinalysis revealed severe proteinuria and microscopic hematuria. A renal biopsy demonstrated extensively crescentic glomerulonephritis. The antineutrophil cytoplasmic antibody against myeloperoxidase (MPO-ANCA) level, determined by enzyme-linked immunosorbent assay (ELISA), was 494 ELISA units. Under treatment with prednisolone, the symptoms and eosinophilia improved within 3 days. The MPO-ANCA level decreased to its lowest value, and renal function was ameliorated within 3 months. The patient has been followed regularly on an ambulatory basis with a regimen of prednisolone.

Anti-Inflammatory Agents↗

Long-term continuous intravenous infusion of prostacyclin for severe primary pulmonary hypertension.

A patient suffering from severe symptomatic primary pulmonary hypertension (PPH) underwent long-term intravenous prostacyclin therapy; the first time for such treatment in Japan. A 26-year-old male had experienced gradually progressive dyspnea for about one year. Despite conventional therapy he suffered repeated syncopal attacks. However, after receiving a permanent central venous access device and a portable infusion pump, he recovered fully and was discharged. This remedy seems to be promising for PPH as has already been proven in Europe and North Americas, although in Japan it is not as yet commercially available and some problems still need to be resolved.

Adult↗

[Pulmonary hemodynamics and long-term outcome in patients with chronic pulmonary thromboembolism and pulmonary hypertension].

We examined pulmonary hemodynamics and long-term outcome in 78 consecutive patients with chronic pulmonary thromboembolism and pulmonary hypertension. These patient's ages ranged from 19 to 75 years; the mean was 51.0 +/- 13.8 years and there was no difference in sex distribution. Mean pulmonary-artery pressure (PAm) was 44 +/- 14 mmHg and total pulmonary resistance (TPR) was 1138 +/- 634 dyne.sec.cm-5. Overall five-year survival was 58.4%, but in patients whose PAm was below 30 mmHg, the outcomes were better: all of these patients survived for at least 5 years. In patients whose PAm was above 30 mmHg, no correlation was found between the outcome and the magnitude of PAm. We divided the patients into four subgroups according to their TPR: 500 dyne.sec.cm-5 (TPR, 500 > or = TPR < 1000 dyne.sec.cm-5, 1000 < or = TPR < 1500 dyne.sec.cm-5, and 1500 dyne.sec.cm-5 < or = TPR. The five-year survivals in these groups were 100%, 88.9%, 52.4%, and 40.0%, respectively. Outcome correlated with the magnitude of TPR. TPR was useful for predicting the outcome of chronic pulmonary thromboembolism.

Adult↗

[Use of jerk-locked back averaging for detecting the epileptiform discharge in a patient with supplementary motor seizure].

A 23-year-old, right handed women has suffered from supplementary motor seizures manifesting tonic followed by clonic contractions of the left foot, occasionally spreading to the left hand and trunk, since the age of 7 years. The mean frequency of seizures on admission was about 10 times per day. A brain MRI showed an abnormal intensity area about 3 cm in diameter in the right mesial frontal lobe involving the superior frontal and cingulate gyri. Six habitual seizures were recorded during long-term monitoring with digital EEG segments time-locked to the clonic contractions of the left foot, a negative spike was detected about 50 ms before the EMG onset at the midline fronto-central area slightly lateralized to the left. In the focal motor seizure arising from the mesial frontal lobe like in the present case, jerk-locked back averaging helps detecting the epileptiform activity which is otherwise undetectable.

Adult↗

Lack of endothelin ETB receptor binding and function in the rat with a mutant ETB receptor gene.

Congenital aganglionosis rat is a mutant with an autosomal recessive gene (sl). Recent studies have revealed that the endothelin ETB receptor gene of sl/sl rat has a deletion of 301-bp region spanning exon 1 and intron 1 corresponding to the first and the second transmembrane domains of the receptor. In the present experiments, we examined the functions of ETB receptors in the sl/sl rats. In the membranes of cerebellum, heart, and lung of control (+/+ and sl/+) rats, ET-1 induced a monophasic, competitive displacement of [125I]ET-1 binding, whereas ET-3, IRL 1620, and BQ-123 showed biphasic displacement. In the membranes of sl/sl rats, in contrast, ET-1, BQ-123, ET-3, and IRL 1620 showed only monophasic displacement. Scatchard analysis revealed a single [125I]ET-3 binding site in the membrane of control heart but not in the sl/sl rat heart, and the specific binding sites for [125I]ET-1 in both control and sl/sl rat hearts. In the control rat aorta but not in the sl/sl rat aorta, ET-3 induced endothelium-dependent relaxation. These results suggest that sl/sl rats do not have functional ETB receptors.

Animals↗

A mutation in endothelin-B receptor gene causes myenteric aganglionosis and coat color spotting in rats.

Congenital aganglionosis rat (AR) is a mutant with an autosomal recessive gene (sl), which shows megacolon caused by the absence of myenteric ganglion cells and white coat-color with a small pigmented spot on the head. Recently, targeted disruption of the endothelin-B (ETB) receptor gene (EDNRB) in the mouse has been reported to cause aganglionic megacolon and coat color spotting resembling the phenotypes of the sl/sl rats. To identify the mutation responsible for the phenotypes of the sl/sl rats, we determined the nucleotide sequences of the EDNRB genes of the sl/sl rats and found that a 301-bp region intervening between direct repeat sequences was deleted in the EDNRB gene, and the deletion produces various transcripts due to aberrant splicing.

Animals↗

Typing of X chromosomes bearing Tabby allele in mouse preimplantation embryos by detection of a microsatellite marker.

Tabby (Ta) is a semidominat allele of the locus on the mouse X chromosome, which causes a characteristic coat pattern and developmental defects in endocrinic glands. To establish a method for identifying the X chromosome bearing the Ta allele in early preimplantation embryos, we performed PCR amplification of an X chromosomal microsatellite marker locus from preimplantation embryos obtained from mating between XO female and XY male mice. The microsatellite marker locus was shown to be polymorphic between X chromosomes bearing the Ta and wild-type alleles. The amplification of the marker locus from early preimplantation embryos demonstrated that the Ta locus can be correctly typed by this method from embryos as little as 2 cells.

Animals↗

Mucopolysaccharidosis type VI in rats: isolation of cDNAs encoding arylsulfatase B, chromosomal localization of the gene, and identification of the mutation.

Mucopolysaccharidosis (MPS) type VI, the lysosomal storage disorder caused by the deficiency of arylsulfatase B (ARSB) activity, occurs in humans, cats, and rats. To characterize the molecular lesion(s) causing MPS VI in rats, cDNAs encoding rat ARSB were isolated from a rat liver cDNA library. The nucleotide and deduced amino acid sequences of rat ARSB had approximately 80 and 85% identity with the human ARSB sequences, respectively. The chromosomal location of the rat ARSB gene was determined by PCR analysis of rat-mouse somatic cell hybrid panel. The ARSB gene was assigned to rat chromosome 2, where the locus for the MPS VI phenotype in rats has been localized by linkage analysis. To identify the mutation(s) within the ARSB gene causing MPS VI in rats, the ARSB sequence were amplified from affected animals and completely sequenced. Notably, a homoallelic one-base insertion at nucleotide 507 (507insC) was identified, resulting in a frame shift mutation and premature termination at codon 258. The presence of the insertion completely correlated with the occurrence of the MPS VI phenotype among 66 members of the MPR rat colony. Thus, we conclude that 507insC is the causative mutation in these animals and that the MPS VI rats are an authentic model of human MPS VI.

Amino Acid Sequence↗

Chromosomal localization of a strain-specific p53-related sequence in rat (Rattus norvegicus).

Strain-specific restriction fragments hybridizing to human p53 gene cDNA, which was detected in only particular strains of inbred rat were revealed by Southern blot hybridization of DNAs of various inbred rat strains. Chromosomal location of the strain-specific fragments was determined on rat chromosome 1 between Kal and Pkc loci by linkage analysis using microsatellite marker loci. The fragments were concluded to be a novel p53-related sequence which is present in particular rat strains and absent from the remaining strains.

Animals↗