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Biomedical subjects

T Negoro

Publications and source records attributed to T Negoro.

At least 55 records · Page 3Linked to original sources
PubMed1994

Evolutional changes of cortical hypometabolism in West's syndrome.

The pathophysiological basis of the epileptic encephalopathy West's syndrome remains unknown. We have done serial positron emission tomography (PET) with fluorine-18-labelled 2-deoxy-3-fluoro-D-glucose (FDG) in twelve patients with newly diagnosed West's syndrome. Throughout follow-up, PET revealed diffuse or focal cortical hypometabolism in eleven patients, whereas magnetic resonance imaging (MRI) showed morphological abnormalities in only five. At disease onset, PET showed cortical hypometabolism in eight patients (diffuse in three, focal in five). The second PET showed normal metabolism in six of these patients but focal abnormalities in three of the four with normal results on first PET. In all seven patients with normal findings on the second PET, tonic spasms ceased after initial treatment and no epileptic seizure occurred thereafter. In the five patients with cortical hypometabolism on the second PET, tonic spasms persisted or recurred, or partial seizures appeared. However, in two patients PET abnormalities disappeared in accordance with the later resolution of epileptic seizures. All patients with normal MRI and second PET results had normal psychomotor development. Diffuse or focal cortical hypometabolism that cannot be detected by MRI or computed tomography is common in patients with West's syndrome. However, this anomaly is not permanent and changes with clinical symptoms. These functional abnormalities in the cerebral cortex may be associated with the development of West's syndrome.

Cerebral Cortex↗
PubMed1994

Focal spasms in clusters, focal delayed myelination, and hypsarrhythmia: unusual variant of West syndrome.

We report a patient who began to have clusters of seizures characterized by brief elevation of the right arm at 6 months of age. An interictal electroencephalogram (EEG) at 7 months revealed hypsarrhythmia without definite asymmetry. Simultaneous EEG and video recording disclosed that these focal spasms were associated with fast wave bursts superimposed on slow waves most markedly in the left centro-midtemporal region. The patient became seizure-free after synthetic ACTH therapy. The patient is developmentally normal at 3 years 5 months, but magnetic resonance imaging studies revealed findings suggestive of delayed myelination in the left frontal region. This patient is considered to have had an unusual variant of West syndrome associated with focal delayed myelination.

Adolescent↗
PubMed1994

Photosensitive epilepsy in children.

We performed a retrospective analysis of 17 children with photosensitive seizures (PSS) who had been followed for more than 3 years (mean: 9 years). PSS were verified in all patients by simultaneous video-EEG monitoring. The seizures were precipitated by flickering stroboscopes (14 patients) or were induced by patients themselves (3) with head-nodding in front of illumination, blinking at television or close viewing of striped patterns. PSS consisted of myoclonic seizures (eight patients), generalized tonic-clonic convulsions (5), partial seizures (3) or atypical absence (1). According to the International Classification of Epileptic Syndrome, three patients were classified as having severe myoclonic epilepsy in infancy and five as having juvenile myoclonic epilepsy. The remaining nine could not be categorized as any specific epileptic syndrome. Children with age of the onset of epilepsy at 7 years or younger tended to suffer intellectual deficit in addition to intractable seizures.

Adolescent↗
PubMed1994

[Studies on auditory brainstem responses showing absence or significantly poor formation of waves II-V in children].

We performed a retrospective study on 7 children in whom the auditory brainstem responses (ABR) showed absence or significantly poor formation of waves II-V. Two patients had degenerative diseases that affected the brains diffusely. In the remaining 5 patients, the symptoms consisted of action tremor (3 patients) or pyramidal signs (2 patients). There were neither clinical signs nor neuroimaging findings suggesting brainstem involvements. The evolutional changes of the ABRs had a good correlation with the clinical course. Absence or significantly poor formation of waves II-V of ABR occurs in various diseases and may not necessarily reflect severe organic brain lesions.

Brain Diseases↗
PubMed1994

[Clinico-epidemiologic study of infantile hydrocephalus in Japan].

A nation-wide survey on congenital hydrocephalus, including secondary hydrocephalus occurring within one year after birth, was carried out in 1988. The number of patients with congenital hydrocephalus treated during the previous one year was estimated to be 3,200-3,500 throughout Japan. The number of new cases of this disease which occurred during the year was calculated to be 800. This indicates a rate of 0.58 per 1,000 live births. Clinico-epidemiologic features were compared with regard to three subjects listed below. (1) The difference between the epidemiologic profile of term and preterm infants with hydrocephalus was investigated. As for etiology, the incidences of primary hydrocephalus and hydrocephalus complicated by Arnold-Chiari malformation type II and meningocele were significantly higher in term infants than in preterm infants. It was estimated that about 30% of the hydrocephalus in preterm infants and only 7.7% in term infants occurred as a consequence of intracranial hemorrhage. The degree of neurological impairment and the mortality rate were higher in preterm infants than term infants. (2) The changing panorama of infantile hydrocephalus in Japan was investigated comparing the epidemiologic data of four groups according to the birth year. As for pregnancy period, preterm infants with hydrocephalus were gradually increasing recently. Hydrocephalus secondary to intracranial hemorrhage was increasing and that secondary to intracranial infection was decreasing. The rate of shunt malfunction was decreasing significantly. (3) Clinico-epidemiologic features of intractable hydrocephalus based on the diagnostic criteria of the research committee was investigated comparing intractable with non-intractable groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Humans↗
PubMed1993

Benign epilepsy of children with complex partial seizures following febrile convulsions.

Among the partial epilepsies with complex partial seizures (CPS) following febrile convulsions (FC), there is an idiopathic epilepsy with extremely benign outcome, characterized by: 1) no past history suggesting brain insult, no underlying brain lesions, no neurological abnormalities, no mental retardation; 2) a high incidence of a positive family history of FC or benign epilepsy; 3) no past history of prolonged febrile convulsions; 4) EEG spike foci other than anterior temporal ones; 5) CPS easily controlled with full recovery.

Adolescent↗
PubMed1993

[A study on ACTH dosage for treatment of West syndrome].

We conducted a retrospective study of 50 patients with West syndrome who had been treated with ACTH. The patients were classified into two groups; group I, receiving standard dose of ACTH (0.025 mg/kg/day); and group II, receiving low dose of ACTH (0.015 mg/kg/day). The short-term effect of treatment was evaluated. Each group was further divided into two subgroups; cryptogenic (I-C, II-C) and symptomatic (I-S, II-S). There was no significant difference in the control rate of clinical seizures, between the two dosage groups of cryptogenic etiology. In the symptomatic cases, a greater effectiveness was achieved in group II-S than I-S. None of the patients with cryptogenic etiology experienced any relapse of infantile spasms. Patients with symptomatic etiology had a higher incidence of relapse. The rate of clinical and EEG seizure control was lower, and the incidence of side effects was higher in group I-S patients. The poor short-term effect of treatment in group I-S patients may be due to a higher incidence of prenatal etiology.

Adolescent↗
PubMed1993

[Atypical early infantile epileptic encephalopathy with suppression-burst].

We report an 8-month-old infant with tonic spasms that started at the age of 38 days and occurred in clusters. He had no myoclonic seizures, or erratic myoclonus. Repeated simultaneous video-EEG monitoring revealed abrupt flexion of the neck and extremities associated with high voltage slow waves preceded by multiple spikes and/or sharps, which resembled spasms seen in West syndrome. Interictal EEGs, before and after the onset of epilepsy, showed suppression-burst. The bursts consisted of irregular high voltage slow waves mixed with poorly developed spike before the onset of epilepsy. On the other hand, duration of suppression phase ranged from 1 to 27 seconds, and burst-burst interval from 2 to 30 seconds after the onset of epilepsy. He was considered as having an atypical early infantile epileptic encephalopathy with suppression burst.

Diagnosis, Differential↗
PubMed1992

Islet amyloid polypeptide (IAPP/amylin) causes insulin resistance in perfused rat hindlimb muscle.

It has been reported that islet amyloid polypeptide (IAPP) has insulin antagonistic effects in vivo and in vitro. To determine whether IAPP affects glucose metabolism in skeletal muscle, we performed in situ rat hindlimb perfusion which is a near-physiological system. Forty min after the beginning of insulin infusion at 1000 microU/ml, the synthesized rat amide form of IAPP was infused at 1 nM or 10 nM for 50 min and glucose concentration in the effluent was measured to calculate glucose uptake (GU). The GU did not change during the 1 nM IAPP infusion, but significantly decreased during 10 nM IAPP infusion (554 +/- 24 to 445 +/- 29 nmol/g/min, P less than 0.01). Rat calcitonin gene-related peptide (CGRP), which has sequence homology with IAPP and has been reported to inhibit insulin action, was also administered. Similar to the effect of IAPP, the GU did not change during 1 nM CGRP infusion but significantly decreased during 10 nM CGRP infusion (507 +/- 7 to 323 +/- 15 nmol/g/min, P less than 0.01). In the experiments without insulin infusion, the GU was not changed even by 10 nM IAPP infusion. Therefore, IAPP directly reduced only the insulin-mediated GU in the skeletal muscle, and this effect of IAPP occurred at the same dose as that of CGRP. These data suggest that both IAPP and CGRP may cause insulin resistance in skeletal muscle not through a CGRP receptor but a yet unknown receptor, which has similar binding affinity for both IAPP and CGRP.

Amyloid↗
PubMed1992

Usefulness of PET scan in a child with mesial frontal lobe epilepsy.

Positron emission tomography (PET) scan with 18F-fluorodeoxyglucose (18F-FDG) was performed in a 14-year-old boy who had seizures suspected to have originated in mesial frontal lobe. The seizures occurred in clusters and were characterised by a change in the facial expression at seizure onset and complex motor manifestations consisting of kicking, swaying and screaming. Ictal EEG showed rhythmic alpha-waves in the left frontal area association with the ictus. Cerebral CT, MRI and SPECT revealed nothing of significance, but the PET brain scans showed frontal and parietal hypometabolism, which was most prominent in the left mesial frontal lobe. The present case suggests that FDG-PET scanning may be useful for the diagnosis of the mesial frontal epilepsy, when other imaging studies fail to show abnormalities.

Adolescent↗