A video-EEG study of astatic seizures observed in patients with myoclonic astatic epilepsy of early childhood (Doose syndrome).
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Biomedical subjects
Publications and source records attributed to Y Fukuyama.
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The existence of temporal clustering of suicides was analyzed by comparing the distribution of weekly numbers of suicides under age 20 in Japan (1982-1987) with a Poisson distribution. The linkage between newspaper reports about teenage suicides and these clusters of suicides was examined in an ecologic study. The following results were obtained: 1) The distribution of weekly numbers of suicides under age 20 was not consistent with a Poisson distribution. The observed numbers for weeks with clustering was higher than the expected numbers. Therefore it is suspected that teenage suicides was not an independent occurrence but clustered in specific weeks with clustering observed in April and between the 15th and 25th weeks in 1986. 2) The age and sex characteristics of those who committed suicide during the observed clusters every April did not differ from that of suicide victims for the rest of the year. The incidence ratio for suicide between 15th and 25th weeks in 1986 compared to the same period for other years was higher in girls than in boys, and higher in younger teenagers than older teenagers. Analysis by prefecture of suicides which occurred between the 15th and 25th weeks in 1986 showed high incidences for Hokkaido, Tohoku, Chugoku, and Shikoku blocks, and low incidences for the Kanto and Kinki blocks. 3) Twenty news articles reporting teenage suicides with headings much larger than four columns appeared in the Asahi Newspaper in 1982-87. Significant linkage between newspaper reports about teenage suicides and trend of weekly numbers of suicides in 1985 and 1986 was not observed. 4) Weekly numbers of suicides significantly increased following newspaper reports on January 23rd 1985 and on April 8th 1986, describing a suicide caused by battering, and a jumping suicide of a young singer who was popular among teenagers, respectively. However no increase in the number of suicides was observed following other newspaper reports.
A case of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes, in which a pituitary growth hormone (GH) secretion deficiency of hypothalamic origin was revealed through neuro-endocrinological examinations, was described. The case was a 10-year-old girl, who had been suffering from generalized tonic seizures since age 5, four episodes of alternating hemiplegia since age 6, stunted growth since age 7, and simple partial motor seizures as well as gelastic seizures since age 8. Marked elevation of lactate and pyruvate in both serum and CSF, abundant ragged red fibers in biopsied muscle, and low density areas in the left occipital lobe and bilateral globus pallidus in addition to diffuse brain atrophy on CT scan and MRI of the head were demonstrated, although the activities of muscle enzymes complex I-IV were within normal ranges. Pituitary GH secretion was deficient under the loadings with insulin, L-DOPA, sleep, and a single growth hormone releasing factor (GRF) administration, but normal GH response was registered under the repetitive stimulation with GRF. Activities of other hormonal axes were normal. It is likely that short stature commonly observed in MELAS patients is due to hypothalamic dysfunction, which might be brought out by chronic ischemia and energy deficiency of the diencephalon based upon mitochondrial abnormality of that region. It is likely that gelastic seizure in this case is due to hypothalamic dysfunction.
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We report a 3-year-11-month-old boy who manifested action myoclonus only. Histochemical analysis of the quadriceps muscle revealed subsarcolemmal hyperactivity. The administration of 5-hydroxytryptophan and carbidopa dramatically improved the action myoclonus and reduced an amplitude of giant somatosensory evoked potentials. A nosological relation of this case with "essential myoclonus" and mitochondrial encephalomyopathy was discussed.
Phlorofucofuroeckol A (1), a novel phlorotannin with both dibenzo-1,4-dioxin and dibenzofuran elements, has been isolated from the brown alga Ecklonia kurome OKAMURA as a potent anti-plasmin inhibitor. Its structure has been elucidated to be 1,11-di(3,5-dihydroxyphenoxy)- benzofuro[3,2-a]dibenzo[b,e][1,4]dioxin-2,4,8,10,14-pentaol on the basis of the spectral data, in particular, by means of negative nuclear Overhauser effect (NOE) and long-range carbon-proton couplings (2JCH and 3JCH). Phlorofucofuroeckol A inhibited the action of alpha 2-macroglobulin (IC50 = 1.0 micrograms/ml) and alpha 2-plasmin inhibitor (IC50 = 0.3 micrograms/ml), the main plasmin inhibitors in plasma.
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We studied the 3rd lumbar vertebral trabecular bone mineral density in 59 cross-sectional pictures of quantitative computed tomography (QCT) with CaCO3 phantom for 28 hospital control children and 30 cases of suspected bone metabolic disorders. The QCT value of bone mineral density of control children showed neither age dependency nor sexual difference before puberty: for males was 221.8 +/- 30.2 mg CaCO3/cm3 (Mean +/- SD) under 4 years, 218.1 +/- 39.7 at 5-9 years and 217.2 +/- 30.9 at 10-15 years; and for females 220.9 +/- 18.3 under 4 years and 240.0 +/- 29.4 at 5-9 years. The QCT values of bone mineral density in bed-ridden patients, children receiving glucocorticoids and children receiving anticonvulsants were significantly lower than that in control children (p less than 0.005). The QCT value of bone mineral density of bed-ridden patients was significantly lower than that of children receiving glucocorticoids and of children receiving anticonvulsants (p less than 0.05, p less than 0.005 respectively). Our study confirmed that single energy quantitative CT was very useful in pediatric clinical application.
We report a 3-month-old female with infantile spasms that responded transiently to pyridoxine and permanently to oral L-dopa. Initial CSF levels of homovanillic acid were low, suggesting disturbed turnover of dopamine. These findings suggest that disturbed brain monoamine metabolism may be causally related to infantile spasms.
1. A phase I study of buspirone was conducted in 7 healthy male volunteers. 2. Diazepam was selected as the control drug and administered in equipotent doses to buspirone. Dosage was initiated at 2.5mg and doubled until a maximum dosage of 20mg was attained. Subsequently, 10mg was administered once a day for three consecutive days. 3. Clinico-pharmacologically both drugs produced sleepiness/drowsiness, but dizziness, light-headed feeling and feeling of drunkenness were marked only in the diazepam group. 4. No drug-related abnormalities were observed in clinical laboratory test values, endocrinological tests and ECG. 5. On the Uchida-Kraepelin test, no change with the control values was observed under buspirone but subjects administered diazepam exhibited marked deterioration during the latter half of the test. Moreover, in the tapping test, significant impairment was observed in the diazepam group whereas buspirone had no effect. 6. On the EEG some fast waves were observed with diazepam whereas buspirone exhibited slow waves.
The long-term electroencephalographical observations were documented during the course of the Rett syndrome in 8 patients. The abnormal EEG findings changed with age and the clinical stage. In the premonitory stage (birth-1.5 years of age), EEG seemed to be normal. In the acute exacerbation stage (1.5-5 years old), occipital dominant alpha wave-like activity was characteristic and persisted for a few years, but it disappeared afterwards along with the seizure activity. At around 4 years of age, when the whole spectrum of symptoms manifested, the EEG background activity in the waking stage showed higher amplitude, lower frequency and more irregular than normal. Sleep spindles were hardly observed, while frequent seizure activity was found on sleep EEG. Before the onset of epileptic attacks, paroxysmal discharges, such as diffuse spike-and-wave complexes, were noted during sleep recordings. In the chronic stage (after age 6), a monotonous theta rhythm (MTR), which was not influenced by either opening or closing of the eyes but attenuated only by a big noise or strong pain stimuli, characteristically dominated the waking tracing. The MTR was generalized over both hemispheres. After age 20, the MTR tended to be more localized to the centro-parietal area. This suggests that a reduction in responsiveness to visual and other stimuli occurs in this disease, which may be attributable to a dysfunction of the reticular activating system in the brainstem.
In Fukuyama type congenital muscular dystrophy (FCMD), congenital muscular dystrophy and anomalies of the central nervous system are regarded as the major features, but the existence of ocular lesions has hardly been recognized as being important. In the present study, close ophthalmologic examinations were performed on 11 patients with FCMD, and we found myopia, weakness of the orbicularis oculi, congenital nystagmus, cortical blindness, optic atrophy, chorioretinal degeneration, etc. In particular, the chorioretinal degeneration observed in the ocular fundus was considered to be specific to FCMD. It is thought that these ocular lesions or changes are caused by the same mechanism as that involved in the central nervous system anomalies.
The Japanese Committee for the National Registry of Subacute Sclerosing Panencephalitis (SSPE) confirmed that 215 cases of SSPE occurred in the 20 years from 1966 to 1985, as discovered in the 10-year surveillance from April 1976 through March 1986. The annual incidence in recent years has been between 10 and 23 cases. Among cases with a certain history of measles illness or measles vaccination, 184 (90.2%) had a history of measles illness without receiving measles vaccine. There were 11 probable measles vaccine-associated cases (5.4%), three (1.5%) being vaccinated with a combined use of killed and live vaccine and eight (3.9%) with further attenuated live vaccine. There were nine cases (4.4%) without a history of either measles illness or measles vaccination. Intervals between measles illness and the onset of SSPE varied from 1 to 16 years (mean, 7.0 years). The periods following measles vaccination with further attenuated live vaccine were 2 to 11 years (mean, 4.6 years). Annual incidence rates of SSPE per million cases of measles ranged between 6.1 and 40.9 (mean, 16.1) in the 10 measles epidemic years 1968-1977, and those following vaccination with further attenuated live vaccine were zero in most years and at the highest 3.08 (mean, 0.9) per million doses of distributed vaccine.
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6,6'-Bieckol (1), 2-O-(2,4,6-trihydroxyphenyl)-6,6'-bieckol (4), and 8,8'-bieckol (2), bispolyphenols with a dibenzo-1,4-dioxin skeleton, have been isolated as potent anti-plasmin inhibitors from the brown alga Ecklonia kurome OKAMURA. Their structures have been determined to be dimers of eckol linked at the C-6 or C-8 positions, through an aryl-aryl bond on the basis of spectral data. Their inhibitory actions on anti-plasmins (alpha 2-macroglobulin and alpha 2-plasmin inhibitor) and some proteases have been examined.
Eckol (1), a novel phlorotannin with a dibenzo-1,4-dioxin skeleton, has been isolated from the brown alga Ecklonia kurome Okamura as a potent and specific anti-plasmin inhibitor. Its structure has been elucidated based on the spectral data, in particular, by means of negative nuclear Overhauser effect (NOE), and finally established as 1-(3,5-dihydroxyphenoxy)-2,4,7,9-tetrahydroxydibenzo-1,4-dio xin by X-ray analysis. Some partially methoxylated derivatives of eckol were prepared by methylation with diazomethane and also by selective dimethylation of eckol permethylate (1b) to establish the structural requirements for inhibitory activities on alpha 2-macroglobulin and alpha 2-plasmin inhibitor, the main plasmin inhibitors in plasma.