Rumi Amano

Treatment of Intractable Childhood Epilepsy with High-Dose Valproate

Summary: Forty‐six children with refractory epilepsy (12 with symptomatic generalized epilepsy, 14 with symptomatic partial epilepsy, and 20 with undetermined epilepsy) were treated by high‐dose (serum level above 100 μg/ml) valproate (VPA) therapy. Monotherapy was used with 34 patients and two drugs with 12. Serum VPA concentrations ranged from 105.1 to 198.4 μg/ml. Assessment of initial response to treatment, after the serum level had reached the appropriate level, showed seizures to be completely controlled in 15 (32.6%) of 46 patients and improved in 12 (26.1%) (50% or more). Follow‐up of more than 6 months after the time of initial response showed control of seizures in 14 (30.4%) and improvement in 11 (23.9%). The initial effect on EEG was the disappearance of epileptic discharges in 3 (6.5%) of 46 patients and marked improvement in 15 (32.6%). Follow‐up revealed the disappearance of epileptic discharges in 7 (15.2%) and marked improvement in 9 patients (19.6%). High‐dose VPA therapy was especially effective for West syndrome and for epilepsy with continuous spike‐waves during slow‐wave sleep. Control of atypical absences and myoclonic seizures was relatively good. Hypofibrinogenemia and thrombocytopenia were sometimes encountered but these side effects were reversible with reduction of dosage.

Dipole tracing in childhood epilepsy with special reference to Rolandic epilepsy

To assess the clinical applicability of dipole tracing in childhood epilepsy, the location and stability of electric source generator of focal spikes seen in EEG of epileptic children were investigated using this method. The patients were divided into 3 groups; benign childhood epilepsy with centrotemporal spikes (BCECS, Group A, n=14), other types of epilepsy with centrotemporal spikes (Group B, n=15) and epilepsy with focal spikes in other areas (Group C, n=13). The spike dipole in each group was analyzed using dipole tracing method. The following results were obtained. (1) The spikes of BCECS were characterized by constantly stable dipoles, compared to those of the other types of childhood epilepsy. The spikes of epileptic children with mental retardation mostly lacked the stability of dipoles. These seemed to suggest that the stability of dipoles was closely related to the prognosis and pathophysiology of epilepsy. (2) The dipoles of BCECS were localized strictly in the Rolandic area. Dipole tracing from EEG spikes was considered to contribute to the elucidation of the pathophysiology of childhood epilepsy.

Myoclonus Epilepsy With Ragged-Red Fibers: A Clinical and Electrophysiologic Follow-up Study on Two Sibling Cases

We performed a 5-year clinical and electrophysiologic follow-up study on two sibling cases with myoclonus epilepsy with ragged-red fibers. Both had myoclonus, intention tremor, slight muscle weakness, slight mental disturbance, hearing impairment, and optic atrophy. Neither had epileptic attacks or truncal or gait ataxia. Biochemical activity of cytochrome c oxidase was at the lower limit of the normal range of values, and an adenine to guanine transition mutation at nucleotide 8344 in the transfer RNA specific for lysine of mitochondrial DNA was detected in both cases. The electroencephalograms showed slowing of basic patterns, diffuse spike-and-wave complexes, occipital dominant wave-and-spike phantoms, 6-and 14-Hz positive spikes, and photosensitivity. No definite deterioration of basic patterns was seen, and diffuse spike-and-wave complexes and photosensitivity gradually disappeared during the slowly progressive clinical course. P2 latencies of pattern-reversal visual evoked potentials throughout the clinical course and III through V interpeak latencies of auditory brainstem responses at follow-up were prolonged without giant sensory evoked potentials in both cases. (J Child Neurol 1993;8:366-372).

Monozygotic twin patients with congenital ichthyosis, microcephalus, spastic quadriplegia, myoclonus, and EEG abnormalities

A pair of monozygotic twins manifested widespread ichthyosis on the trunk and extremities, mainly on the extensor surface of the lower legs. Both patients had severe developmental retardation, microcephalus, spastic quadriplegia, myoclonus, and hearing impairment. Cranial computed tomography and magnetic resonance imaging revealed diffuse cortical atrophy. Electroencephalography documented diffuse slow wave dysrhythmia and marked epileptic discharges, namely bilateral occipital dominant, and low-voltage multifocal spikes which appeared asynchronously as a burst, mainly during sleep. Skin biopsy revealed hyperkeratosis with parakeratosis. Granular cell layer was not thickened and diminished. Papillomatosis was absent. Electron microscopy did not reveal any distinctive abnormalities. Sjögren-Larsson syndrome and other known ichthyosis syndromes were excluded because of the difference in localization of skin lesions, skin manifestations, lack of papillomatosis, the nature and severity of neurologic symptoms and marked electroencephalographic abnormalities.

Refractory Epilepsy in Infancy and Childhood–A Prospective Follow‐Up Study

Although therapy for childhood epilepsies has been dramatically improved in the past few years? not a negligible number of cases is still resistant to the rap^.^ To clarify the reality of refractory epilepsies in childhood, a comparative study was made between refractory cases and control cases with a favorable seizure prognosis. Finding a therapeutic clue to refractory epilepsies was expected in the analysis of factors responsible for intractability.

Lennox-Gastaut syndrome: a developmental study

The subjects were 84 patients (51 males and 33 females) all more than 10 years of age who were followed-up for over five years after having been first seen at Okayama University Hospital between 1968 and 1978. The ages at the time of follow-up ranged from 10 to 15 in 34 cases, 15 to 20 in 38 cases, and more than 20 up to 27 and 11 months in the remaining 12 cases. The periods of follow-ups ranged from five years and six months to 19 years and eight months. We investigated the long-term mental and seizure prognoses and clinico-electroencephalographic evolutional changes in these cases.

Rational Treatment of Refractory Epilepsy in Childhood

Abstract: A multifaceted study on childhood refractory epilepsy disclosed an insufficient classification of epilepsies and epileptic seizures, and inappropriate polypharmacy as the most important factors preventing appropriate therapy. By means of an adjustment of AEDs based on the accurate classification of epilepsies and epileptic seizures, the number of AEDs could be reduced in 37.5% and monotherapy was successful in 13.8% of refractory cases. Most of the latter were those of partial epilepsy and generalized epilepsy with the monoseizure type. The exacerbation of seizures due to AEDs was also mentioned as one of the important side effects of AEDs.