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Biomedical subjects

H Oguni

Publications and source records attributed to H Oguni.

At least 37 records · Page 2Linked to original sources
PubMed1998

Dramatic effect of ethosuximide on epileptic negative myoclonus: implications for the neurophysiological mechanism.

Epileptic negative myoclonus (ENM) is a recently defined epileptic seizure type seen in various epileptic syndromes. Although the long-term prognosis appears to be favorable, the treatment of localization-related epilepsy (LRE) with ENM in childhood is sometimes difficult due to the apparently pharmaco-resistant nature of ENM. We evaluated the effects of antiepileptic drugs (AEDs) in 10 patients with ENM. Carbamazepine was administered to eight patients, none of whom improved. Responses to clonazepam and valproic acid were unpredictable, whereas ethosuximide (ESM) achieved complete control of ENM in all six cases treated with this drug as adjunctive therapy. The pharmacological responses of ENM to CBZ and ESM were quite similar to those of absence seizures. According to the SPECT and ictal EEG findings in addition to the pharmacological responses from this study, we favor to postulate that ENM is produced by a direct inhibitory action on the motor cortex resulting in the interruption of voluntary muscle contraction as generated by sharp-slow wave complexes, compatible with the mechanism considered to underlie absence seizures. ENM are refractory to treatment and persisting if the wrong AEDs, such as PHT or CBZ, are selected at the diagnosis of LRE. We recommended a trial of ESM when ENM develops during the clinical course of LRE regardless of etiology.

Adolescent↗
PubMed1998

Startle epilepsy with infantile hemiplegia: report of two cases improved by surgery.

PURPOSE: To study the effectiveness of surgical therapy on 2 patients with startle epilepsy with infantile brain damage (SEIBD), a rare but distinctive epileptic syndrome characterized by motor seizures resistant to antiepileptic drugs (AEDs). METHODS: The patients with SEIBD both had hemiplegia, due to gross contralateral hemispheric lesions, and suffered from tonic postural seizures, frequently provoked by sudden unexpected somatosensory stimuli on the paretic side of the body. These attacks occasionally caused the patients to drop to the floor, and mild-to-moderate injuries had been sustained; they were resistant to all currently available AEDs. Consequently, these daily drop attacks severely restricted the patients' social lives and school participation. Both patients underwent corpus callosotomy and resection of epileptogenic premotor and supplementary motor lesions. One patient also underwent additional multiple subpial transections of the primary sensorimotor area. RESULTS: Seizures, as well as quality of life, improved dramatically in both patients after surgery. CONCLUSIONS: Startle epilepsy with infantile hemiplegia is a distinct epileptic syndrome characterized by structural brain damage restricted primarily to one hemisphere, large ipsilateral epileptogenic lesions involving the perisensorimotor area, refractory startle-provoked drop attacks, and a good response to epilepsy surgery.

Adolescent↗
PubMed1997

Electrophysiological study of myoclonic seizures in children.

To investigate the neurophysiological mechanisms underlying myoclonic seizures in childhood, we measured the latency between the onset of electromyogram (EMG) potentials and that of corresponding spikes obtained by the back-averaging method. The subjects were nine patients, with various epileptic syndromes, ranging in age from 2 months to 15 years, 11 months. The numbers of seizure events obtained by averaging ranged from five to 51 with a mean of 25. The latencies between averaged spike-and-wave complexes and deltoid EMG potentials ranged from 21 to 80, with a mean of 38 ms. Comparing these results with reported age-matched physiological conduction times, these latencies appeared to be slightly prolonged in all of our patients. Our results support the hypothesis that myoclonic seizures are produced through either a cortical or a subcortical generator, via a polysynaptic mechanism acting on muscles, rather than a monosynaptic corticospinal pathway.

Adolescent↗
PubMed1997

Atonic epileptic drop attacks associated with generalized spike-and-slow wave complexes: video-polygraphic study in two patients.

PURPOSE: We studied falling manifestations of atonic epileptic drop attacks (AEDA) in 2 patients with cryptogenic epilepsy with myoclonic-astatic seizures. METHODS: Using video-polygraphic examinations. For analysis, we recorded 51 seizures in 1 patient and 18 seizures in the other. The extent of AEDA ranged from collapsing and landing on the buttocks to head nodding only without falling. RESULTS: Detailed video analysis of the drop attacks, with the patient in standing position, demonstrated the first manifestations to be flexion at the waist and knees, followed by further knee flexion, leading to falling straight down and landing on the buttocks. Ictal polygraphs demonstrated diffuse interruptions of ongoing EMG discharges corresponding to the falls, indicating that these attacks are true atonic seizures. CONCLUSIONS: The falling manifestations of AEDA appeared to be characteristic and different from those of tonic drop seizures, which have been described as falling forward with tonic flexion of the hips, upper trunk, and head, as well as abduction or elevation of the arms.

Cerebral Cortex↗
PubMed1996

Long-term prognosis of Lennox-Gastaut syndrome.

We investigated the long-term prognosis of Lennox-Gastaut syndrome (LGS) in 72 patients followed up for > 10 years. Long-term seizure and intellectual outcomes were poor, as previously reported. The diagnosis of LGS was first made in the age range from 2 to 15 years with peak occurrence at 5 years. Progressive IQ score deterioration with age was apparent. At the last examination, 33% of patients with cryptogenic and 55% with symptomatic LGS had lost the characteristics of LGS, and their seizure disorders were classifiable as symptomatic generalized epilepsies, severe epilepsy with multiple independent spike foci, or localization-related epilepsies. Disabling drop attacks appeared in 46% of patients and tended to occur at older than 10 years. Gait deterioration was recognized in 12 patients and seemed to be due largely to progression of the epileptic encephalopathy. The gait disturbances, as well as increased frequency of violent drop attacks, were disabling in daily life and resulted in some patients being wheelchair bound.

Activities of Daily Living↗
PubMed1993

[Chronic hypothermia and a disordered thermoregulation in a child with severe brain damage and malnutrition].

Patients with brain damage, especially under malnutrition, sometimes show a disordered thermoregulation. Our case was an 11 year-3-month-old boy with severe brain damage probably of prenatal origin, who was physically extremely small in size for age and showed difficulty of taking food. Low body temperature ranging from 34.0 degrees C to 36.0 degrees C became outstanding since 9 years of age. Continuous rectum temperature monitoring was starged, when he was admitted in our department of pediatrics in Tokyo Women's Medical College Hospital for the therapy of acute infection. Before hospitalization, he took orally only 150 to 200 kcal/day. After the recovery from the infection, a higher calorie diet appropriate for his body weight was given through a nasal tube. Dramatic improvement was observed in terms of sleep pattern, body temperature and thermoregulation after the administration of higher calorie nutrients. It is suggested that the circadian rhythm of the body temperature would be disturbed by chronic malnutrition in children with brain damage.

Body Temperature Regulation↗
PubMed1992

A case with nocturnal paroxysmal unilateral dystonia and interictal right frontal epileptic EEG focus: a lateralized variant of nocturnal paroxysmal dystonia?

We report here a patient with frequent nocturnal attacks, characterized by paroxysmal dystonic posturing of the left arm and contraction of the lower face, lasting 20-30 seconds. The attacks occurred in clusters of up to 20 spasms during the NREM stage of sleep. Interictal EEG showed clear-cut epileptic discharges in the right frontal region. Repeated ictal video-EEG recordings revealed only arousal pattern immediately before and during seizures except in those which evolved to left sided clonic hemiconvulsions. The dystonic attacks here described share many features of nocturnal paroxysmal dystonia (NPD) of Lugaresi and Cirignotta in common, including nocturnal periodic occurrence, absence of ictal EEG changes during attacks, and favorable response to CBZ, except for their unilateral nature. It was assumed that our case may be a lateralized variant of NPD and would further support the hypothesis that NPD is one particular form of frontal lobe epilepsy.

Child↗
PubMed1991

Anterior callosotomy in the treatment of medically intractable epilepsies: a study of 43 patients with a mean follow-up of 39 months.

We studied the effectiveness of anterior callosotomy in 43 patients whose follow-up period averaged 39 months. These patients had intractable generalized seizures, characterized by a combination of seizure patterns, most frequently drop attacks with generalized tonic-clonic, generalized tonic, and absence seizures. Overall, drop attacks were the most frequent (31/43 or 72%) and the most disabling seizure pattern (27/43 or 63%); they were also the most likely to benefit from anterior callosotomy (70%). Patients with lateralized changes tended to have a better result than did those without lateralization, but patients with synchronous and symmetrical spike and wave discharges also benefited. The preoperative intelligence quotient and the etiological factors were not predictors of outcome. There was a correlation between the extent of section and the results; patients with section of the anterior two thirds had a better result, compared with those who had section of the anterior half.

Adolescent↗
PubMed1985

A case of absent right superior vena cava with persistent left superior vena cava: cross-sectional echocardiographic diagnosis.

We report a case of absence of the right superior vena cava. Hitherto, the findings in this condition have only been obtained using invasive methods; this report is the first in which the diagnosis was made by means of echocardiography. The findings include a markedly enlarged coronary sinus draining into the right atrium and a specific finding of contrast echocardiography in which contrast material injected into the right antecubital vein can be seen in the enlarged coronary sinus. The contrast echo flows into the right atrium from the coronary sinus only. The size of the coronary sinus was far beyond the range (91 +/- 38 mm2/m2) in patients with persistent left superior vena cava with the presence of the right superior vena cava.

Adolescent↗