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Biomedical subjects

H Oguni

Publications and source records attributed to H Oguni.

46 records · Page 3Linked to original sources
PubMed1983

[Two-dimensional echocardiographic diagnosis of tetralogy of Fallot with a total conus defect].

Tetralogy of Fallot (TOF) with a total conus defect has some characteristics different from classical TOF in respect to its morphogenesis and clinical features. This anomaly, although rare in Europe, constitutes approximately 10% of all types of TOF in Japan. In this report, we studied 30 cases of TOF with angiographic or surgical documentation in order to investigate two-dimensional echocardiography as a possible mean of detecting a conus defect in TOF. We classified the entire group into three types according to the location of a ventricular septal defect (VSD): subpulmonic VSD (six cases, I), infundibular muscular VSD (11 cases, II) and perimembranous VSD (13 cases, III). A long-axis view along the right ventricular outflow tract rather than a short-axis view at the level of the aortic root was adopted as a useful examination for viewing VSDs. Using the former image, we could observe precisely a VSD just below the pulmonary valve in all six cases of Type I and three of Type II, but none of Type III. Three cases of Type II which had a short infundibular septum of several millimeters in length with surgical confirmation were very similar to cases of Type I. The discontinuity between the interventricular septum and the pulmonary valve was confirmed to be identical with subpulmonic VSD by angiographic and pathological studies. In conclusion, we could differentiate TOF with a total conus defect from the other types of TOF by detecting the echo defect in the septum just below the pulmonary valve with the aid of a long-axis view of the right ventricular outflow defect.

Adolescent↗
PubMedDate not supplied

Electroencephalographic features of epileptic drop attacks and absence seizures: a case study.

A study of epileptic drop attacks (EDA) by simultaneous video-polygraphic recordings was carried out in one epileptic patient with myoclonic astatic seizures (Doose syndrome). EDA was shown to correspond to a burst of generalized bilaterally synchronous spike and wave complexes (GBSSW) at 3 Hz. Absence seizures were also observed with a burst of GBSSW with similar characteristics. The amplitudes of the corresponding slow wave component of GBSSW among the three intensities of atonia, i.e. complete atonia, minor atonia and no discernible atonia (control), was compared. A high amplitude was demonstrated to correspond with more pronounced atonia and a lower amplitude with reduced or absent atonia. These findings suggest that EDA corresponding to GBSSW have a neurophysiological mechanism in common with absence seizures, and that if the GBSSW is intense, it may be sufficient to cause immediate loss of global muscle tone.

Child, Preschool↗
PubMedDate not supplied

Video-EEG analysis of drop seizures in myoclonic astatic epilepsy of early childhood (Doose syndrome).

We studied 36 drop seizures in 5 patients with myoclonic astatic epilepsy of early childhood (MAEE) with simultaneous split-screen video recording and polygraph. Sixteen were falling attacks and 20 were either less severe attacks exhibiting only deep head nodding or seizures equivalent to drop attacks in terms of ictal pattern but recorded in the supine position. All seizures except those that occurred in patients in the supine position showed sudden momentary head dropping or collapse of the whole body downward. Recovery to the preictal position was observed in 0.3-1 s. As a result of carefully repeated observations, the 36 seizures were classified as myoclonic flexor type in 9, myoclonic atonic type in 2, and atonic type, with and without transient preceding symptoms in the remaining 25. The MF seizure was characterized by sudden forward flexion of the head and trunk as well as both arms, which caused the patient to fall. In the myoclonic atonic seizure, patients showed brief myoclonic flexor spasms, immediately followed by atonic falling. The AT seizure showed abrupt atonic falling, with and without transient preceding facial expression change and/or twitching of extremities. The ictal EEGs of all 36 seizures exhibited generalized bilaterally synchronous single or multiple spike(s) and wave discharges. Atonic drop attacks appear to be a common cause of ictal epileptic falling in MAEE.

Child, Preschool↗
PubMedDate not supplied

A study of unilateral brief focal atonia in childhood partial epilepsy.

We studied unilateral brief focal atonia (BFA) in seven patients with childhood partial epilepsy. BFA was observed as a transient dropping of one arm lasting from 100 to 150 ms when patients were asked to keep both arms outstretched in front of the body. Close examination using simultaneous video-polygraphic recordings showed dropping of the arm to correspond exactly with a single sharp and slow wave complex arising from the contralateral centrotemporoparietal region. The BFA occasionally would progress to atonic seizures or atonic absence seizures, when the localized epileptic discharge evolved into generalized discharges. In one patient we found a positive correlation between the intensity of BFA and the amplitude of the contralateral epileptic discharges. A higher amplitude corresponded to more pronounced BFA and a lower amplitude to less pronounced BFA. These results led us to conclude that the apparently interictal single sharp and slow wave complex in the rolandic region may inhibit contralateral motor control, thus producing BFA that corresponds with the spike amplitude.

Brain↗
PubMedDate not supplied

Treatment of severe myoclonic epilepsy in infants with bromide and its borderline variant.

We studied the efficacy of bromides (BR) as add-on therapy in 11 patients with severe myoclonic epilepsy in infants (SME) and in another 11 with the borderline variant of SME (BVSME). Study subjects were aged 8.5-183 months (mean 64.4 months). Longest duration of BR treatment was 37 months (range 4-37 months; mean 19.7 months). Eight of 22 (36%) of patients with generalized tonic-clonic seizures (GTCS) had an excellent effect (> 75% reduction in total seizure frequency or duration) and 9 (41%) had a moderate effect (50-75% reduction) 3 months after introduction of BR. Twelve months after initiation of BR. 5 of the patients with significant improvement were no longer responsive; ultimately, therefore, 6 had an excellent effect and 2 had a moderate effect. Of those with partial seizures (n = 5) and myoclonic/absence seizures (n = 5), only 1 patient in each group showed a moderate effect at the 12-month time-point. Dosages and serum concentrations of BR ranged from 30 to 100 mg/kg (mean 58 mg/kg) and from 64 to 159 mg/dl (mean 101 mg/dl), respectively. Of the 12 patients experiencing side effects, including drowsiness, appetite loss, and skin rash, 1 required a reduction in BR dosage because of an extensive acneiform rash on the face. The results show that BR treatment holds promise for patients with SME and BVSME and should therefore be investigated further.

Adolescent↗
PubMedDate not supplied

Chronic localized encephalitis (Rasmussen's syndrome) preceded by ipsilateral uveitis: a case report.

Chronic localized encephalitis (Rasmussen's syndrome) developed in an 11-year-old girl 1 month after operation for residual cataract due to chronic uveitis that had appeared at age 9 years ipsilateral to the affected cerebral hemisphere. Although the pathogenesis of Rasmussen's syndrome remains unknown, our case provides additional evidence indicating that a viral ocular infection is a possible cause or part of the disorder.

Adult↗
PubMedDate not supplied

Video-polygraphic analysis of myoclonic seizures in juvenile myoclonic epilepsy.

We studied myoclonic seizures (MS) in 5 patients with juvenile myoclonic epilepsy (JME) using video polygraphic recordings to investigate the clinical characteristics of MS in this epileptic syndrome. The total number of MS analyzed was 302 (range 27-125, mean 60) seizures per patient. MS occurred either singly or repetitively (37 vs. 63%) and corresponded to generalized bilaterally synchronous single or multispike-and-wave complexes at 3-5 Hz. Video analysis of the myoclonic jerks demonstrated that either distal or proximal muscle involvement predominated. In the former, there was mild bilateral flexion and some external rotation of the forearms. In the latter, flexion of both arms at the elbow, flexion and abduction of the thighs, and extension of the back was observed. Asymmetry of MS was noted in 4 of 5 patients. Facial involvement of MS occurred infrequently in 2 patients. When the patients kept both arms outstretched, the arms dropped or there was sudden interruption of ongoing electromyographic (EMG) potentials immediately after myoclonic jerks (postmyoclonic inhibition) in all patients. One should inquire about these clinical characteristics of MS in JME when taking a thorough history in patients with primary generalized tonic-clonic seizures (GTC).

Adolescent↗
PubMedDate not supplied

Effect of anterior callosotomy on bilaterally synchronous spike and wave and other EEG discharges.

We analyzed pre- and postoperative interictal and ictal EEG patterns in relation to seizure outcome in 29 patients with intractable epilepsy who had undergone anterior callosotomy. Twenty-two patients had generalized bilaterally synchronous sharp or spike and slow wave discharges (GSW) and 7 did not: Postoperatively, patients with preoperative GSW showed disruption of synchrony, increased amplitude asymmetry, and a decreased amount of GSW. No significant decrease was noted in the amount of total epileptic discharge after operation. There was a significant relation between seizure outcome and decrease in amount of GSW. The degree of disruption of synchrony, total amount of epileptic discharge, and pre- and postoperative EEG patterns were not good indicators of seizure outcome. Postoperative changes in lateralization of epileptic foci consisted either of increased lateralization, less lateralization but increased independent discharges in the previously nonpredominant hemisphere, or appearance of some lateralization. There appears to be a spectrum of GSW: Both secondary bilateral synchrony and secondarily generalized corticoreticular epileptic discharges are distributed along this spectrum.

Adult↗